Paediatrics

Angular Deformity

Normal development

 

1 year:  Bow legs / 15° varus

2 year:  Neutral 

3 year:  Knock knees / 10° valgus

 

3 year old knock knees

 

6 year:  Physiological valgus / 6° valgus

 

Normal valgus six year old

 

Note range ~ 15° either way at each age

- persistence of physiologic variations may occur

- especially in some families & racial groups

 

Salenius and Vankka

- chart of normal tibio-femoral variations by age

 

History

 

FHx

 

Examination

 

Unilateral / bilateral

- angular profile

- femorotibial angle

- inter-malleolar / intercondylar distance (quantify)

 

LLD / rotational profile / joint laxity

 

Height vs Age

 

X-ray

 

Erect AP Standing Long Leg view

- patella directed forward

- femur & tibia on same Xray

 

Indications

- if pathological form suspected

- asymmetry

- < 5th percentile

- severe deformity

- positive FHx

 

- other musculoskeletal abnormality

 

Causes of both Varus and Valgus

 

Trauma: Malunion / Partial physeal arrest

Rickets / Renal disease

JRA

Osteopaenia

OI 

 

NHx

 

Avoid dogmatic predictions

- clinical course variable

- not all cases resolve

 

Shoe wedges & other bracing ineffective

 

Prognosis

 

Uncertain

- genu valgum may cause CMP / PF Dislocations

- genu varum may cause OA Knee

 

Varus deformities

 

Physiologic Bow legs

 

1. Lateral tibial bowing

- occurs in first year of life

- nearly always resolves

 

2. Common Bowing

- involving the femur & tibia

- seen in second year

- prior to age 2 years, development of MFC lags behind lateral

- resolution occurs in most children

 

Management Physiological  

 

Bracing doesn't affect the NHx

- exclude pathological causes of deformity & reassure parents

 

Surgery 

- corrective osteotomy / epiphysiodesis / guided growth with 8 plates

- recommended for those children with persistence or worsening of physiologic varus

 

Pathological

 

Blount's

Rickets

Trauma - malunion / epiphyseal arrest

Infection - physeal damage

Skeletal dysplasia - achondroplasia / OI / enchondromatosis / metaphyseal chondrodysplasia

Anterolateral bowing - pseudoarthrosis

Fibrous dysplasia

JRA

 

Valgus Deformities

 

Physiological Valgus

 

NHx

- knock knees very common age 2-6

- starts at 2 years

- maximum 3-4 years

- usually resolves by 7

- may not always resolve

- minimal correction occurs > 8 yo

 

X-ray if

- asymmetrical / Unilateral

- progressive

- LLD

- intermalleolar distance > 10 cm

- outside normal parameters i.e. 15° either side normal for age

 

Bracing

- no evidence of efficacy

 

Surgical Indications

- > 15° valgus

- cosmesis

- poor gait / function

- avoided < 12 years

 

Options

- osteotomy / hemiepiphyseodesis / Guided Growth with 8 plates

 

Pathological Valgus

 

Traumatic

 

1.  Distal femoral physeal injury

- Usually SH I or II 

 

2.  Proximal tibial metaphyseal fracture / Cozen's

- tendency for valgus deformity

- reason why is uncertain

 

Theories

- rotation on xray hides valgus

- ST interposition

- overgrowth

 

Manage

- careful moulding cast into varus

- usually resolves over time

 

Infection - physeal injury

Congenital Posteromedial Bowing

Anteromedial Bowing - fibular hemimelia

Skeletal dysplasia - MED / pseudoachrondroplasia / Kneist

NMD - cerebral palsy / spina bifida

Lateral Condylar Hypoplasia

 

OI

 

Rickets

 

JRA

 

Blount's Disease

DefinitionBlounts

 

Progressive varus deformity of knees

- secondary to abnormality of medial upper tibial physis

- localised varus & internal rotation deformity

 

Infantile form 

- onset 1-3 years / bilateral

 

Adolescent form 

- onset > 6 years / unilateral

- 5 times less common

- M:F

- presents 8 - 14 years

 

Epidemiology

 

Africans / African Americans / West Indians

 

Associations

 

Female

Obesity

FHx

Early walking

 

Aetiology

 

Unknown / Multifactorial

 

Familial

- no consistent inheritance pattern

-  ? related to tendency to obesity 

 

Mechanical

- most likely due to abnormal compression on medial side of proximal tibial physis

- causes retardation of growth

-  ? traumatic role

 

Pathology

 

Posteromedial disordered endochondral ossification 

- dense islands of hypertrophied chondrocytes

- acellular areas of dense fibrocartilage in resting zone

- abnormal groups of capillaries

 

Fragmented physis

 

Clinical

 

Infantile presents at 12-36/12 

 

Bilateral & symmetrical

- bowing noted when commence walking

- associated internal tibial torsion due to fibular tethering normal ER of tibia with growth

- continuum between physiological vara & Blount's (infantile may be severe physiological vara) 

- varus should resolve by the age of 2

 

Examination

 

Milestones / height & weight percentiles

 

Knee ROM 

 

Measure of Genu Varum & Tibial torsion

 

Ligamentous Laxity

 

X-ray

 

Indications

- severe genu varum

- rapidly worsening

- height < 25th percentile

- marked asymmetry

- FHx

 

Findings

- localised deformity at proximal tibia

 

Metaphyseal - Diaphyseal Angle

 

Blounts Metaphyseal Diaphyseal Angle

 

Technique

- line drawn perpendicular to axis of tibia 

- line drawn through medial & lateral beaks of metaphysis

- Blount's > 11°

- physiologic bow legs < 11°

 

Measurements

- 11° is arbitrary cut off where Blount's is more likely

- 16o definite

 

Medial Physeal Slope

 

Blounts Medial Physeal Angle

 

Technique

- line through medial physis & line through lateral physis

 

Measurements

- significant if > 60°

- prognostic of progression

 

CT

 

Used to identify presense of physeal bar

 

Langenskiold Classification

 

Stage I: Beak 2-3 years

- irregular metaphyseal ossification

- medial metaphyseal beaking

 

Stage II: Saucer 2-4 years

- saucer shaped defect in medial metaphyseal beak

- medial epiphyseal wedging

 

Stage III:  Step 4-6 years

- saucer deepens into step 

- medial epiphysis irregular

 

Stage IV: Bent plate 5-10 years

- growth plate inclined distally at medial side

- i.e. epiphysis extends down over meta beak

 

Stage V: Double epiphysis 9-11 years

- Xray appearance of severe posteromedial depression

 

Stage VI: Medial physis ossified 10-13 year

- medial physeal closure

 

Blounts Langenskiold Type VI

 

DDx

 

Physiological varus

Metaphyseal dysplasia / achondroplasia

Ricket's

Trauma / Tumour / Infection

OI

JRA

 

Physiological Varus

- symmetrical involvement

- normal growth plate

- medial bowing of proximal tibia & distal femur

- metaphyseal-diaphyseal angle < 11°

 

Rickets

- short stature, osteopaenic

- widened physes / cupped metaphyses 

- distal Femur Flared too

- may have coxa vara

- hypophosphataemic most common

 

Renal Osteodystrophy

 

Metaphyseal Chondrodysplasia

- widened metaphysis, cupped physis

- similar to rickets

- mild short stature

- may have coxa vara as well

 

Focal fibrocartilagenous dysplasia 

- generalised abnormality or focal deformity in tibia

 

OI

 

Trauma

Infection

Tumor

 

NHx

 

Progresses to severe OA by early adulthood

- metaphyseal-diaphyseal angle >11° --> likely to progress

- medial-physeal slope >60° likely to progress

- Philadelphia sign: lateral subluxation of tibial epiphysis 

- if restore normal valgus should have good outcome

 

Need to manage child before they develop a bar (i.e. end stage of growth plate injury)

 

Management Infantile Type

 

Algorithm

 

Depends on

- age of child

- stage of disease

 

1.  <2 years 

 

Observe

 

2.  2 - 3 years & Medial Physeal Angle < 60°

 

KAFO Single Medial upright 

- free ankle with no knee hinge

- flexion limited

- knee cuff pulls it into valgus

 

Full-time bracing successful > 50%

 

3. Age > 3 years / Progression in Brace / Medial Physeal Angle > 60° 

 

Aim

- correct varus and internal rotation deformity

 

Options

 

A.  Lagenskiold I - IV

- osteotomy

- guide growith

 

B.  Lagneskiold V / VI

- take down bar and osteotomy or

- epiphysiolysis + medial metaphseal osteotomy

 

Langenskiold Stages I-IV Surgical Management

 

1.  Osteotomy

 

Aim

- restore alignment

- deformity reversible

- if restore physiological valgus (7o) then resolution is usual for I & II / possible for III & IV 

 

Type of osteotomy

 

A.  Opening / closing wedge

B.  "Smiley" upside down dome

C.  Oblique osteotomy

- Rab biplanar oblique osteotomy

- fix with single screw

 

Osteotomy Technique

 

Performed distal to TT

- closing wedge simplest but upside down dome has least shortening

- must osteotomise fibula

- usually want to correct IR deformity at same time

- must release anterior compartment to prevent compartment syndrome

- desired valgus & ER achieved

- fixation with K wires or screw

- POP post operatively

 

Recurrence after osteotomy

 

1. Obese

2. > Stage III

3. Medial physeal slope > 60°

4. Age

- > 5 y = 76%  

- < 5 y = 31%

 

2.  Guided growth / 8 plate

 

Now common mechanism of treating condition

 

3.  Osteotomy and external fixation

 

Langenskiold Stages V & VI 

 

Issue

 

Irreversible

- need to address physis as well as osteotomy

- usually total physiodesis

- overcorrection 10°

 

Surgery 

- must do fibula osteotomy as well

- usually perform total physeodesis of ipsilateral side

- always perform fasciotomy

- may need to realign epiphysis in severe forms with large medial-physeal slope

- consider epiphysiodesis of other side to address LLD

 

Options

 

1.  Medial Metaphyseal Elevation Osteotomy

 

Indications

- Grade V

 

Blounts Elevation

 

2.  Physeal Bridge Resection (physeolysis) + Osteotomy

 

Indications

- Grade VI

- bridge < 30% of physis

 

Technique

- excise bar where CT shows a bridge

- Insert fat into defect

 

3.  Lateral Hemi-epiphysiodesis + osteotomy

 

Indications

- grade VI

- bridge > 30%

 

Technique

 

All need fibula osteotomy

All need prophylactic compartment release

 

Complications

 

Compartment syndrome - must prophylactic release

Recurrence of varus - usually secondary to physeal bar

LLD

OA

 

Adolescent Type

 

Management

 

Wait till skeletal maturity, then HTO

 

Cerebral Palsy

Background

Definition

 

CP is a permanent disorder of movement and posture 

- non-progressive 

- brain injury before the age of two years

 

Strict definition excludes familial & progressive congenital problems & those acquired in childhood as a result of head injuries

 

Incidence

 

2-3 per 1000 live births

 

Higher in 

- premature and low birth weight children

- low socio-economic group have higher incidence

- more common in advanced countries

(possible increase due to improved survival of premature and low birth weight infants)

 

Quadriplegia has diminished

 

Athetosis has been the most dramatic decrease due to

- Rh immunisation

- the drop in erythroblastosis fetalis

- better treatment of jaundiced infants

 

Most common

- spastic diplegia or hemiplegia

- 65%

 

Natural History

 

About 50% have normal intelligence

- 25% end up independent as adults

 

Walking

- hemiplegics 100% walk

- diplegics 75% walk

- quadriplegics 25% walk 

 

Likely to walk

- sit by 2 years 

- stand by 4 years

 

Aetiology

 

Prenatal (44%)

 

Maternal infection (TORCHS)

- Toxoplasmosis

- Rubella

- Cytomegalovirus

- Herpes

- Syphilis

 

Maternal exposure

- Alcohol

- Drugs

 

Perinatal (27%)

 

Hypoxia (accounts for <10% of CP)

Prematurity

Low birth weight

 

Postnatal (5%)

 

Meningitis

Head injury

Immersion

 

Other (24%)

 

Common Causes

 

Hemiplegia - obstetric

Diplegic - prematurity

Quadriplegia - anoxia

Athetoid - kernicterus

 

Prematurity Risk

 

2500 gms = 0.34% 

1500 gms = 14%

<1500 gms = 90%

 

Pathology

 

The lesion that is seen in common is "Periventricular Leukomalacia "

 

Classification

 

Anatomic and Pathophysiologic classification

 

It takes at least 2 years to reach a static state

 

Pathophysiology Classification

 

Spastic (60%) 

- hyperactive reflexes

- develops contractures, bony deformity & ultimately joint dislocation 

- benefits from orthotics and surgery

 

Athetoid (10 - 20%)  

- major problems with control of limb position and limb balance

- succession of slow writhing movements

- rarely benefit from orthotics & surgery

- benefit greatly from therapists in terms of self care & occupation programs

 

Hypotonia (2-5%)

- common in infancy

- most become hypertonic with time

 

Ataxic (2-5%)

- uncoordinated, unbalanced, wide based gait

 

Mixed 

- important to identify these children as the motion disorder affects the outcome of surgery

- typically spasticity & athetosis with total body involvement

 

Spastic - motor cortex

Athetoid - basal ganglia

Ataxic - cerebellar

Mixed

 

Anatomic

 

(True monoplegia or triplegia probably don't occur)

 

Diplegia 

- lower limbs affected much more than upper limbs

- 75% eventually walk

- IQ may be normal

- strabismus is common

 

Quadriplegia

- extensive involvement

- low IQ, high mortality

- only 25% able to walk

 

Hemiplegia 

- usually with spasticity

- develop early handedness

- 100% walk regardless of treatment

 

Mercer Rang's Stages of CP

 

Stage 1:  Dynamic contracture

- secondary to spasticity

- physiotherapy / orthotics / botox

 

Stage 2:  Fixed musculotendinous contractures

- tendon lengthening / transfer

 

Stage 3:  Bone & joint involvement

- osteotomy / arthrodesis + tendon lengthening

 

Pathogenesis

 

1.  Weakness   

 

UMN lesion / loss of voluntary movement

 

2.  Spasticity

 

Feature of all pyramidal lesions

- excessive activity of disinhibited neurones / overactive stretch reflex

- hypertonicity with increased DTR

- clonus

 

3.  Contracture

 

Shortening of the muscle-tendon unit due to failure to keep pace with bony growth

- normally muscles lengthen as bone grows

- as bone grows this worsens spasticity and shortens muscle

- as the muscles don't lengthen get contractures

 

The sarcomeres add to the muscle at the musculotendinous junction in response to stretch

 

4.  Deformity

 

Secondary to unopposed muscle contracture

- worse in all cases where muscles cross 2 joints

- psoas, RF, hamstrings, T Achilles

 

Hip dislocation

- persistent adduction leads to valgus neck

- persistent flexion leads to anteverted neck

- acetabular dysplasia (deficient posteriorly), hip subluxation and dislocation can follow

 

It is actually uncertain how increased / decreased tibial torsion and increased femoral anteversion appear

 

Cascade

 

CNS lesion 

Abnormal neurological activity 

Spasticity 

Reduced muscle excursion and growth 

Contractures 

Joint instability 

Subluxation / Dislocation 

Degenerative arthritis 

 

Clinical Features

 

Spastic Quadriplegia

 

Usually due to cerebral anoxia

 

Presentation

- floppy child that won't feed

- choking during feeds - bulbar palsy

- failure to thrive

 

The primitive reflexes (Babinski, Moro, Parachute) are preserved

 

Intelligence / vision / hearing affected

- usually mentally impaired

- may have epilepsy

 

Only 10-20% will walk 

- can be delayed up to 7 years

- hip dislocation and scoliosis will develop in non ambulators

 

Spastic Diplegia

 

Usually due to prematurity

 

Lower limb affected more than upper

- all milestones delayed

- most walk by 4 years of age

 

Can vary from mild toe walker to non-ambulators 

 

Try to improve efficiency of gait 

 

Spastic Hemiplegia

 

Usually due to obstetric complications

 

Usually noticed at walking age

- don't swing one arm

- limp with one - handedness

 

Right-sided form may have speech delay

 

Seizures common

 

All walk independently 

- aim to improve walking appearance or posture of upper limb 

 

GMFCS 

 

Gross motor function classification system

 

I  Essentially normal, decreased speed and balance

- walk stairs without aids

- some difficulty on uneven ground

 

II Hand rail up stairs

 

III Walker inside, wheelchair outside

 

IV Wheelchair inside and out

 

V Nil head support

 

Orthopaedic Management

 

Goals

 

1.  ADL's

 

Upper limb / spine

 

2.  Mobility

 

Walking / transferring

- keeping a GMFCS III walking is crucial 

- keeps them out of nursing Home

 

Ability

- 25% TBI (Total body involvement)

- 100% hemiplegics

- 75% diplegics

 

Prognostic signs

- > 2 primitive reflexes over 1 year poor sign

- should sit at 2 years, walk at 4 years

- TBI v hemi v diplegic

 

Guidelines

 

Expert field

 

Should never decide on treatment at one visit 

- variable nature

 

Gait analysis lab 

- can help decide best options 

 

Single Event Multi Level surgery (SEML)

- avoid birthday syndrome (where children are operated on every year)

- has a psychological impact

 

Surgery between 4-8 years 

- after 8 years tend to develop fixed deformity

 

Non-operative Management

 

Physiotherapy

 

No scientific evidence that it alters the outcome

- very important nontheless

- high contact with parents  / children

- keeps joints as supple as possible

 

Cast correction

 

Young and mild cases

 

Bracing 

 

Useful particularly in the equinus ankle where it can be maintained plantigrade in AFO

 

Botox

 

Botulinium toxin Type A

- blocks acetylcholine release at NMJ

- 50-60% patients respond well 

- for dynamic deformity UL or LL (R1 v R2)

 

Use

- every 6/12

- peaks at 4/52

- lasts 3-4/12

- at MT junction

- are maps of injection for every muscle in body

 

Areas

- useful in T Achilles

- useful in hamstrings

- overused in psoas

- very useful in UL

 

Problems

A.  Will only fix dynamic spasticity not fixed contracture of muscle

B.  Expense - $500 per IU

 

Examination

 

R1 v R2

- R1 is passive range of motion performed slowly

- R2 is passive range of motion performed quickly

- a difference between the two indicates dynamic component

 

Valium

 

Potentiates GABA

 

Intrathecal Baclofen

 

GABA analogue

 

Results

 

Gilmartin J Paed Neurology 2000

- significant improvement over placebo 

- indicated for spastic quads / TBI

- pump under abdo wall with reservoir

- into spinal cord

 

Selective Dorsal Rhizotomy 

 

Best for Spastic Diplegia < 9 without contractures

- need strong legs (as lose some strength with loss of spasticity)

- able to cooperate in rehab

- UL improvement also

 

Technique

 

Laminectomy

- stimulate rootlets to find which mediate spinal reflex

- if only these cut, sensation unchanged

- 30% of dorsal rootlets cut

- decreases feedback from stretch receptors

 

Complications

 

Spinal deformity

Weakness / parasthesia / bladder problems

 

Operative Management

 

Indications

 

1.  Inability to control spastic deformity by non operative measures

 

2.  Fixed deformity that interferes with function

 

3.  Joint instability

 

Strategies

 

Stage 1

- physio to help prevent contractures

- botulinum

- splints

- selective posterior rhizotomy

 

Stage 2 

- tight muscles released / lengthened

- weak muscles augmented by tendon transfers

 

Stage 3 

- fixed deformities corrected by osteotomy / arthrodesis

 

 

 

Diplegia

Benefit most from Treatment

 

Problems

 

1. Tight psoas / Adductors / Hamstrings / T Achilles

2. Increased Femoral Anteversion

3. External Tibial Torsion

4. Valgus Foot

 

Principles

 

Surgery once walking but before school (3-4)

SEML surgery

Gait analysis lab

 

Gait

 

True equinus

Apparent equinus (FFD knee and hip)

Jump

Crouch

 

Hip

 

A.  FFD

 

Contracture of iliopsoas is cause

- preserve iliacus to maintain strength of hip flexors

- lengthen psoas at pelvic brim / Sutherland release not divide at LT

- Sutherland release at brim

 

B.  Adduction Contracture

 

C.  Internal Rotation Deformity

 

Causes intoeing / scissoring gait

- cause unclear

- children with tight hamstring can only sit in W position

- ? causes femoral anteversion

 

Early release of hamstring can decrease intoeing

 

DRVO proximal femur

 

Knee

 

A.  FFD 

- due to hamstring contracture

- often unrecognised co-contracture of quads / rectus femoris

- if hamstrings alone lengthened, stiff flexed knee gait becomes stiff extended knee gait

 

B.  Spastic Crouch Correction

- gait with flexed knees & hips

- release psoas / hamstrings / rectus

 

Ankle & Foot

 

A.  Ankle Equinus

 

True equinus

- tight T Achilles

 

Apparent equinus

- tight hamstrings

- patients use equinus to extend knee

- if release T achilles in this situation will cause crouch gait

 

B.  Ankle Equinovalgus

 

3 Solutions

- Evans Procedure / Lateral Column Lengthening

- STJ fusion & T Achilles lengthening

- Triple Arthrodesis

Examination

Examination

 

Aids 

 

AFO / KAFO

- ankle foot orthosis

- knee ankle foot orthosis

 

GRAFO

- ground reaction AFO

 

Kaye walker 

- seat on it

- co-ordinates walking

 

Reciprocal Gait Orthoses

 

Sitting

 

Short adducted leg - dislocated hip

Scoliosis

Kyphosis - query secondary tight hamstrings

 

Walking

 

Decreased velocity 

 

Coronal Plane

- scissoring / tight adductors)

- asymmetrical arm swing / hemiplegia

- LLD / hip dislocation

 

Sagittal

- equinus / jump / crouch 

 

A.  Equinus

- ankle in equinus

- knee straight or in recurvatum

- hip extends full

 

B.  Jump

- equinus of ankle

- flexion of knees and hips, never extend fully

 

C.  Crouch

- ankle in dorsiflexion

- over lengthening of T Achilles

- have to flex knees and hips to regain centre of balance 

 

Lower Limb

 

R1 - do slowly

R2 - do quickly

 

Looking for a difference between the R1 and R2

- if reduced ROM on R2, have spasticity / dynamic element

- amenable to botox

 

Supine

 

1. Psoas

- FFD / Thomas test

- must test knee first

- do over edge of bed if FFD knee

 

2. Adductors

- scissored gait if bilateral

- apparent leg length inequality if unilateral

- Trendelenburg gait 

- decreased hip abduction

 

3. Hamstrings

- FFD at knee

- knee flexed at start of stance phase

 

Popliteal angle (hip flexed at 90°)

- straight is 0˚

 

Unable to sit up with legs straight

- decreased  SLR

- can't touch toes

 

4. Triceps Surae

- ankle equinus

- tiptoe gait

 

Silverskiold test 

- distinguish between the gastrocnemius and soleus

- test ankle DF range with knee flexed and extended

- if gastrocnemius tight, reduced DF with knee extended

 

On side

 

5. Iliotibial Tract

 

Obers' test 

- patient on side and flex knee with hip in neutral abduction then as flex knee further hip abducts 

 

Prone

 

6. Quadriceps

- stiff leg gait

- inability to flex knee with hip extended suggests tight rectus

 

Ely test (RF)

- child prone 

- when the knee is flexed the hip flexes suggesting tight RF

 

7.  Rotational profile

 

Tone

 

Increased / clonus / clasp knife

 

Reflexes

 

Increased

 

Primitive Reflexes

 

Moro  

- child supine in arms, allow head to drop back 

- arms & legs stick out in extension

- disappears by 4 months

 

Parachute

- arms and legs extend when child held prone

- appears at 5 months

 

Labyrinthine

- tone reduced & arms/legs flex when prone but increased tone & extended arms & legs with supine position

 

Upper Limbs 

 

General

- resting position

- contractures

- joint stability

 

Hand placement

- ability to place hand in space

- < 10 seconds

 

Stereognosis

- ability to identify ojects in hand without looking

Hemiplegia

Problems

 

1.  Lower limb

 

All walk

- Ankle > knee > hip

 

Ankle

- most require operations for ankles

 

LLD

- unilateral underdevelopment

- LLD 0-5cm (average 2cm)

 

2.  Lower limb

- one handedness

- decrease movement in swing

- astereogenesis

- usual upper limb contractures

 

Ankle

 

4 problems

 

1. Equinus 

2. Weak Dorsiflexion

3. STJ Varus

4. Valgus

 

Equinus

 

Non operative

 

Up to age 4

- AFO

- Botox

- corrective casts for fixed equinus

 

Operative

 

Indications

- > age 4

- nil heel strike

- recurvatum with fixed equinus 

 

Options

- Bauman

- Strayer

 

Remember "Little equinus better than calcaneus"

-  use Gait analysis 

 

Foot Drop after T Achilles Release

 

Non-operative 

 

Leaf spring AFO / Articulated AFO

 

Operative

 

Tendong transfer

- tendon active in swing phase to foot dorsum

- FDL is the best option

- dynamic EMG to decide

 

Dynamic Equinovarus

 

Non-operative

 

Passively correctable

- Botox

- AFO

 

Operative

 

Gait analysis important

- caused by T anterior: varus in swing

- caused by T posterior: varus in stance and swing

 

T posterior

- split transfer to PB

- + T achilles lengthening

 

T anterior

- SPLATT + T achilles lengthening

 

Bony changes

 

Triple arthrodesis

 

Valgus Deformity

 

Less Common

 

Aetiology

- often due to tight T achilles

- attempt DF by escape into valgus

 

Management

 

Respond to T achilles lengthening

 

If severe need Triple arthrodesis

 

Upper limb

 

4 problems

 

1.  Thumb in palm

2.  Flexed wrist / fingers

3.  Elbow contracture

4.  Shoulder IR and Adducted

 

Concepts

 

If astereognosis, correcting the hand posture is unlikely to give functional gains 

 

Need voluntary control & desire to manipulate environment

 

Thumb in Palm Deformity

 

Common but difficult to treat

 

Options

 

A.  Adductor Pollicis & Short flexors released

- may need release web space contractures

- FPL lengthened

 

B. EPB ± EPL reinforced with PL, FCR or BR

 

C. MCPJ stabilized by capsulodesis or arthrodesis

 

Wrist & Finger Deformity

 

Findings

 

Flexed and pronated wrist

Flexed fingers + Swan Neck

 

Pathology

 

Shortening of FCU / FDP / FDS / Pronator

Weakness of wrist extension

 

Management

 

Wrist deformity

- FCU to ECRL / EDC

- wrist arthrodesis salvage

 

Finger Flexion

- lengthening at musculotendinous junction

 

Swan-Neck Deformity

- FDS Slip tenodesis PIPJ

 

Elbow Flexion Contracture

 

Indications

- >45°

 

Management

 

Botox

 

Z-Lengthen of Biceps insertion / release of lacertus fibrosis / Release of brachialis

 

Distal release of PT for pronation contracture

 

4.  Shoulder

 

Usually IR & Add

 

Management

 

Release of SSC & T major  ± P major

 

 

Hip

Aims

 

Prevent contractures

Prevent dislocations

Improve walking 

Provide stable and painless sitting

Allow perineal care

 

Issues

 

Hip Dislocation

Adductor contractures

Flexion contractures

In-toeing

Windswept hips

 

Hip Dislocation

 

Natural History

 

Accepted that a dislocated hip in CP is painful

- unilateral dislocated hips should be reduced unless deformity of femoral head has developed

- less certain of management of bilateral hip dislocation

- importance of vigilant screening

 

Pathology

 

Excessive femoral anteversion

- ? due to tight hip flexors

 

Excessive neck valgus

- ? due to tight adductors

 

High Risk

 

GMFCS 3 / 4 / 5

Spastic quadriplegia

Those wheelchair bound at high risk

 

Rates Hip Dislocation associated with GMFCS

 

I    0%

II   15% - adductor surgery

III  41% VDRO

IV  70% VDRO

V   90% VDRO

 

Screening

 

Non-ambulators annual X-ray essential

- treat tight adduction early / <5 years

- minimum 40° abduction with knees flexed

 

X-ray

 

Reimer's Migration Percentage

- % of epiphysis lateral to acetabulum

- > 30% high risk & requires intervention

 

Guidelines

 

Early ST release +/- bony reconstruction 

 

> 8 years require pelvic procedure (minimal remodelling)

 

Severely deformed hip - don't reduce

 

Severe pelvic obliquity / scoliosis - address first

 

Algorithm

 

1.  < 5 years old + MP > 30%

- soft tissue procedure

- adductor +/- psoas if tight

- preventative measures

- can use botox

 

2.  > 5 years old + MP > 30%

- likely to progress

- adductor release + VDRO / varising derotation oseotomy

 

3.  > 8 years old

- must address acetabulum / add pelvic operation

- CP acetabulum is deficient posteriorly / DDH deficient anteriorly

- Salter worsens posterior deficiency

- Periacetabular osteotomy / Dega

 

4.  Deformed femoral head / salvage

- Schanz osteotomy / pelvic support osteotomy

- excision deformed femoral head

- valgising osteotomy

- suture ligamentum teres to psoas tendon remnant

 

Adduction Contractures

 

Indication

 

Adduction < 30o

 

Treatment

 

Tenotomy adductor longus at groin

- open or percutaneous

 

Obturator Neurectomy 

- may lead to abducted position and affect gait in ambulators

- not recommended

- it denervates adductor brevis which is an important antigravity muscle

- wide base gait

- no improvement with regard to hip stability with neurectomy over simple adductor tenotomy

 

Flexion Contracture

 

Indication

 

FFD > 20o

- lengthening psoas over pelvic brim

- must not do tenotomy if patient walks

- psoas is the main power driver for walking in these children

- may render them unable to walk

 

Options

 

Sutherland technique

- find and preserve femoral nerve

- leave iliacus to preserve hip flexor strength

 

Intoeing

 

Aetiology

 

Increased PFA

 

Management

 

Subtrochanteric FDRO

 

Windswept Hips

 

Definition

 

Pelvic obliquity

- elevated hip adducted and internally rotated 

- lower hip is abducted and externally rotated

 

Soft tissue releases

 

Adducted Hip

- psoas / adductors / hamstrings 

 

Abducted Hip

-  ITB / abductors

 

Bony

- VRDO both sides

 

 

Knee & Foot

Principles

 

Foot

 

A little equinus is better than calcaneus

A little valgus is better than varus

A little varus is better than severe valgus

TA lengthening is most over used operation in CP

 

Knee

 

A little knee flexion is better than recurvatum

 

Knees

 

Problems

 

Tight hamstrings 

- prevents knee extension with hip flexion

 

Often have co-spasticity with tightness of quads 

- unmasked when lengthen hamstrings

- limit the amount of knee flexion in gait 

- result in a stiff knee gait which is very energy inefficient

- i.e. must check Ely's / may need release of RF

 

Jump Gait 

 

Issue

 

Knee flexed / equinus ankle

 

Non-operative Management

 

Botox / physio

 

Operative Management

 

Lengthen hamstrings at the musculotendinous junction

- release the Semitendinosis with tenotomy

- intramuscular recession of Semimembranosus 

- usually leave biceps femoris

- aim for Popliteal angle of 70o

 

Stiff - Knee Gait

 

Spastic rectus femoris can cause limited flexion of knee during swing phase of gait 

- particularly after hamstring lengthening

 

Operative

- Transfer of this to sartorius / SemiT

- release of rectus is just as good

- find plane of tissue under vastus intermedius

 

Tibia

 

Problem

 

Externally rotated tibia

 

Management

 

TDRO / tibial derotation osteotomy

- supramalleolar

- anteromedial approach

- DCP

 

Foot and ankle

 

Problems

 

1.  Equinus

2.  Equinovarus

3.  Valgus

4.  Hallux Valgus

 

Equinus

 

T Achilles lengthening may be the most overused operation in CP

- over-lengthening will lead to slumping in the patient with spastic hamstrings and hip flexors

- crouched posture / debilitating calcaneus

 

Operative Options

 

1.  Formal Z lengthening / Zone 3 / tendon

- > 2cm lose up to 80% of power

- soleus important contributer to power

- soleus might not be tight

- Silverskiold test determines gastrocnemius tightness

 

2. Hoke triple percutaneous slide technique

- proximal and distal cuts medial 

- middle cut lateral

- similar problems to above

 

3. Musculotendinous release (Zone 1)

- best procedures

- Baumann - multiple perpendicular divisions of aponeurosis of gastrocnemius +/- soleus

- Strayer - gastrocnemius recession / single transverse division of aponeurosis of gastrocneumius

 

Equinovarus

 

Cause

 

Tibialis posterior and T Achilles both tight

- most common in spastic hemiplegia 

 

Options

 

1. Perform intramuscular recession

- never extramuscular procedure for fear of reverse deformity

 

2. Tibialis posterior split transfer to Peroneus brevis 

- very effective and preserves plantar flexion

 

3.  Hindfoot deformity fixed 

- Dwyer lateral closing wedge osteotomy 

- lateral calcaneal slide

 

Valgus Defomity

 

Preoperative

 

Always standing Xray of the ankle to ensure no ankle valgus as well

- Coby x-ray

 

Operative Options

 

1.  Grice extra-articular subtalar arthrodesis

 

Results of the Grice are not very good 

 

Scott etal JPO 1988

- 62 feet - 30% fail, 60% poor

- best in child 4-12 years 

 

2.  Evans lateral calcaneal lengthening + first MT plantarflexion osteotomy + T Achilles lengthening

- need first MT plantarflexion osteotomy to enable MT to touch ground post lateral calcaneal lengthening

- may need FDL to T Post transfer also

 

3.  STJ Fusion

 

Hallux Valgus

 

Should always fuse

- high failure with anything else 

 

 

Scoliosis

Epidemiology

 

Scoliosis 10 to 15 x more common in patients with spastic quadriplegia than spastic diplegia

 

Typically

- spastic quadriplegic patient

- long C shaped curve

- lumbar apex

- progressive past maturity

- pelvic obliquity / dislocated or subluxed hips

- can extend into cervical spine

- high risk of respiratory compromise

 

Issues

 

Sitting in wheelchair

Hygiene

Respiratory problems

Feeding difficulties (can lose peg in abdominal folds)

 

Non operative Management

 

Options

 

Brace

- difficult to use

 

Modified chairs with supports

 

Operative Management

 

Indications 

 

Curves > 45º

Documented progression of >10º

Deterioration in function especially respiratory

 

Aims

 

Sitting balance

- level pelvis and shoulders

 

Surgery

 

Typically long instrumented posterior fusion

- proximal TP and pedicle hooks

- distal pedicle screws

- sublaminar wires to aid correction

 

Complications

 

Post operative pneumonia very high

- need to admit to ICU

 

Post operative ileus common

 

Infection rates high /  poor nutrition

 

 

Upper Limb

Indications for Surgery

 

Upper limb surgery is mainly in spastic hemiplegia

- many of the CP' s have sensory neglect for affected limbs   

- won't use limb post surgery anyway

 

Surgical indications

- a reasonable level of IQ (>70)

- spastic not athetoid 

- voluntary grasp and release

- intact sensation / stereogenesis

- good motivation

- hygiene

 

Options

 

1.  Tendon lengthening / division

2.  Tendon transfer

3.  Tenodesis / arthrodesis

 

Principles

 

1. Lengthening is more predictable than transfer

2. Tendon transfers alone can never overcome rigid osseous deformity

3. Joints which are not under voluntary control should be tenodesed or arthrodesed before tendon transfer

4. Agonist-antagonist tenodesis (spastic coupling) is a good approach because it is symmetrical & balanced

 

Typical Posture

 

Shoulder - adducted and internally rotated

Elbow - flexed + pronated

Wrist - flexed + pronated

Fingers - swan-neck +/- flexed

Thumb - in palm

 

Shoulder

 

Soft tissue

- lengthening / release of P major and subscapularis

 

Bony

- external rotation osteotomy humerus

 

Elbow

 

Indication

- contracture > 45o

 

Options

 

Mild

- lengthen biceps / lacertus fibrosis / brachialis

 

Severe 

- release CFO (Steindler) 

- distal release of brachioradialis and pronator teres + anterior capsulotomy

 

Pronation

- release of pronator teres +/- transfer to radius (makes it a supinator of the forearm)

- if severe osteotomy of radius putting it in neutral rotation

 

Wrist

 

Flexion deformity 

 

Class 1 (mild) 

- fingers can be extended with only 20o or less of wrist flexion 

- Release FCU or CFO slide (Steindler)

 

Class 2 (moderate)

- full flexion only possible with > 20o wrist flexion 

- A: extensor power present

- B: no extensor power

- CFO release

- transfer FCU to ECRB if no extensor power

- +/- FDS to ECRB transfer

 

Class 3 (severe) 

- great wrist & finger flexion deformity without extensor motors

- no functional gain is expected 

- surgery here is to improve cosmetic appearance only

- multiple releases +/- wrist arthrodesis

 

Fingers

 

Swan neck deformity

 

Aetiology

- over pull of extrinsic extensors / central slip shortening / intrinsic spasticity

- final common pathway is volar plate incompetence with hyperextension at the PIPJ

 

Management

- FDS tenodesis through a volar Brunner incision

 

Thumb

 

Most crippling upper extremity deformity

- can be a significant hygiene problem in severely affected 

- may need surgery despite not fitting criteria set out above

- in practice surgery is directed at what the pathology is 

 

Type 1 

- weak EPL

- reroute EPL + reinforce with PL or FCR or brachioradialis

 

Type 2 

- intrinsic contracture & first dorsal interosseous tightness

- release webspace +/- Z plasty

 

Type 3 

- weak APL & EPB

- APL tenodesis reinforced with PL, FCR or brachioradialis

 

Type 4 

- spasticity of  FPL

- Z lengthening of FPL

 

 

Foot

Arthrodesis

Types

 

1.  Grice

2.  Lambrinudi

3.  Dunn

 

Grice

 

Definition

 

Extra-articular STJ fusion

- lateral bony block in sinus tarsi

- prevents valgus deformity

- allows undisturbed foot growth

 

Indication

 

Flexible valgus hindfoot in children 4 - 12

Polio

 

Contra-indications

 

Fixed hindfoot

CP - high failure rate

Varus - high failure rate

 

Technique

 

Ollier's incision

- oblique lateral incision

- tip of fibula to base 4th MT

 

Superficial dissection

- between peroneal brevis and tertius

- elevate EDB

- expose sinus tarsi

- remove all ST from talus and calcaneus

- grooves in calcaneum and talus

- no articular surface is exposed

 

Bone graft

- reduce hindfoot

- cancellous iliac (no structural)

- tibial wedge (structural)

- may supplement with metalwork / K wires

 

Post operative

- cast for 6/52

 

Dennyson and Fulford Modification of Grice

 

Cannulated screw

- across talus and bone block and into calcaneum

 

Complications

 

Pseudoarthrosis

Graft slippage / residual deformity

Overcorrection into varus

 

Lambrinudi

 

Concept

 

Plantar flexion of the talus

- is eventually limited by abutment of the posterior process of the talus

- against the lower end of the tibia

 

Plantigrade forefoot is fused to the maximally flexed talus

 

Indications

 

Isolated fixed equinus deformity in patient older than 10 

- tight triceps surae / weak dorsiflexors

- polio most common cause in past

 

Argument exists that the procedure is not suitable for flail footdrop 

- recurrence of deformity due to stretching of the dorsal and anterior soft tissues

- need strong dorsal capsule +/- dorsal tendon transfers

 

Contraindications

 

Ankle joint instability - will be worsened by the procedure as narrow posterior part of talus is in the mortise

Painful pre-existing tibiotalar osteoarthritis

Severe knee or hip instability such that a brace must be worn

Age < 11 years

 

Operative Technique

 

Preoperative planning is essential

- lateral X-Ray taken with foot in extreme equinus

- tracing made and sectioned along lines of subtalar and midtarsal joints and size of wedges calculated

- wedges calculated so that forefoot is plantigrade or up to 10o of equinus in relation to the tibia

- hindfoot in neutral or up to 5o valgus

- greater equinus if need to compensate for short limb

 

Ollier incision

- expose sinus tarsi by elevate EDB

- Z sectioning of peroneal tendons

- CFL divided

 

Talar osteotomy done parallel to transverse axis of ankle joint with talus in extreme equinus

- microsagittal saw

- correct any hindfoot deformity by resecting appropriate calcaneal wedge 

 

V shaped trough fashioned in lower part of proximal navicular

- denude calcaneocuboid joint of cartilage

- sharp distal margin of remaining talus wedged into trough in navicular

 

K wire CCJ and TNJ 

- talus is locked in complete equinus such that no more plantar flexion can occur

 

N.B.  talonavicular pseudarthrosis is a common cause of failure

- ensure talus well medial in trough

- adequate width and depth of trough to allow sufficient bone contact

 

Postoperative management

- long leg POP 6 weeks (NWB)

- short leg POP 6 weeks (NWB)

- if united (attention to talonavicular joint) then remove cast and weight bear

- if not united then walking cast until united

 

Complications

 

Instability

 

Deformity

- positioning of talus medially in navicular trough crucial 

- loss of correction of up to 20o in 10-20% of cases

- post-operative supination deformity

 

Painful ankle OA - will develop with instability or talar AVN / often asymptomatic

 

Talar AVN - avoided by leaving anterior ankle capsule intact

 

Pseudarthrosis

- vast majority talonavicular

- risk factors: early weight bearing, no K-wire fixation

 

Dunn Arthrodesis

 

Concept

 

Variant of triple arthrodesis

- adapted to improve muscle balance in paralytic conditions with a predominant weakness of the triceps surae (calcaneus)

 

Technique

 

Navicular and variable portion of talar head and neck resected

- foot is displaced posteriorly on remaining talus

- lever arm of triceps surae improved

- talus fused to cuneiforms

 

 

 

 

 

 

 

CTEV

Background

Definition

 

Congenital Talipes Equinovarus

 

Congenital abnormality of the foot characterised by 

- hindfoot equinus & varus 

- forefoot Adduction

- midfoot Supination

 

Latin: talus - ankle / pes - foot / equinus - horse like

 

Clinical

 

Foot

- pipe stem calf

- short wide foot

- small heel

- curved lateral border

- short first ray ray

 

Short T Achilles

 

LLD

 

ER torsion tibia

 

Epidemiology

 

1:1000 live births

- 1/1000 Caucasian

- 5/1000 Polynesians

- 0.5/1000 Asian

 

Boys 2:1

 

Bilateral 30-50%

 

Genetics

 

Twin studies

- 33% risk in monozygotic twins

- 3% dizygotic twins

- i.e. is not just the placental environment

 

Sibling or parent

- 3% chance

- 30x increased risk

 

Sibling and parent

- 10 - 20% risk

 

Deletion on chromosome 2

 

Associations

 

Spina bifida & arthrogryposis (teratogenic)

 

Plus

- cerebral palsy

- muscular dystrophies

- spinal muscular atrophy

 

Theories of pathogenesis

 

1.  Fetal development arrest in fibular phase

- during first and second months leg and foot coplanar

- by beginning of third month foot is in 90o of equinus, has supinated & adducted with plane at right angles to leg

- by the middle of the 3rd month the equinus has corrected

- by the end of the third month the supination and adduction has corrected to the adult position

- Bohm postulated arrest in the first phase in week 5

 

2.  Retracting fibrosis (Ippolito and Ponseti)

- decrease in size & number of muscle fibres in posteromedial muscles

- ? fibrous tissue in muscles, tendon sheaths & surrounding fascia

- marked shortening and thickening of the tibionavicular and plantar calcaneonavicular ligaments   

 

3.  Neurogenic theory

- minor innervation changes in posterior muscles

- subsequent fibrosis and shortening

 

4.  Myogenic theory

- myofibroblasts in medial fascia

 

5.  Vascular theory

- anterior tibial artery / dorsalis pedis frequently missing

 

Pathology

 

All tissues in foot abnormal

- bony / muscle / ST / vascular

 

Bony deformities

 

Talus 

- primary deformity

- equinus

- medial deviation of head and neck of talus

- external rotation of body of talus in mortise

- narrow neck & hypoplastic head due to lack of development of articular cartilage on lateral surface of head

 

Os Calcis 

- equinus

- medial rotation 

- long axes of talus & os calcis are parallel in sagittal & transverse planes

 

Navicular 

- subluxed medially against medial malleolus

 

Cuboid 

- medial column subluxed medially and adducted

- lateral & medial columns conjoined therefore cuboid subluxed medially also

 

Metatarsals 

- adductus but minor compared to talonavicular deformity

 

Soft Tissues 

 

Plantar fascia & intrinsics contracted

- long & short plantar ligaments

- contracture plantar fascia

- creates cavus

 

Tendons short

- T Achilles, T Posterior, FHL and FDL short

 

Ligaments short

- deltoid

- spring 

- calcaneofibular ligament

- posterior talofibular ligament

- bifurcate

 

Joint capsules contracted

- posterior ankle joint

- posterior subtalar joint

- talonavicular joint

- calcaneocuboid joint

 

Muscles atrophied

 

Classification

 

A.  Postural 

- passive correction possible

- usually corrects spontaneously within days / weeks

- well defined heel, no calf atrophy

- relatively normal foot size

- no transverse medial skin crease

 

Mild - nearly fully correctable

Moderate - partially correctable 

Severe - slightly correctable

 

B.  Rigid 

- uncorrectable

- small bean shaped foot, transverse crease, tight skin, atrophic calf

- heel difficult to palpate due to overlying fibrofatty tissue

 

C.  Teratogenic talipes 

- associated with spina bifida & arthrogryposis

- prognosis is much worse than idiopathic

 

Pirani Score

 

Pirani scoring system (0, 1/2, 1) (total out of 6)

 

Look (3)

- posterior crease / medial crease / lateral curvature

 

Feel (2)

- head of talus (anterior to lateral malleolus)

- empty heel

 

Move (1)

- DF range

 

Can be used to predict likelihood of needing TA tenotomy

 

Clinical Features

 

Look

 

Deformity / CAVES

Cavus - relative pronation of forefoot c.f. hindfoot

Adduction - forefoot

Varus - heel

Equinus - heel

Supination - midfoot

 

Posterior / medial crease

Curved lateral border of foot

Calf atrophy

 

Walk older child

 

Dynamic supination - require T anterior transfer

Metatarsus adductus

Foot progression angle - tibial torsion

 

ROM

 

DF

Eversion

 

Feel

 

Empty heel

Palpable talus - devils thumbprint anterior to lateral malleolus

Navicular fixed to medial malleolus

Os calcis fixed to the lateral malleolus

 

Exclude 

- spinal dysraphism - look at spine

- arthrogryposis

- neuromuscular disorders

 

Rotational Profile

 

X-ray

 

Usually not indicated

 

Ossification

- calcaneal & talus ossification centres are present at birth 

- cuboid appearing by 6 months

- navicular will not appear until 2-4 years 

 

Angles

 

AP talocalcaneal angle / Kite's angle

- ankle DF 15° & tube at 30° from vertical

- long axis talus parallel long axis of calcaneum

- normal 20-40°

- <20° hind foot in varus / clubfoot axes approach parallel

 

Lateral talocalcaneal angle

- long axis of talus parallel to long axis of calcaneum

- normal 25-50°

- < 25° indicates hindfoot in equinus

- clubfoot axes are parallel or negative

 

Talo-1st metatarsal angle / AP

- long axis of talus to long axis of 1st metatarsal

- normal 5-15° abduction

- clubfoot 0° to negative

 

 

 

Management

Non operative 

 

Ponseti casting

 

Aims of treatment

1. Correct the deformity early

2. Correct it fully 

3. Hold the corrected position until foot stops growing

- AFO

- Denis Browne Boots

 

Timing

 

Start 1 - 3 weeks

- let parents settle and get used to diagnosis

- explain method and length of treatment required

 

Casting

 

5 - 6 casts applied weekly

- apply SL, then convert to LL

- minimal wool

- someone holds the foot corrected

- tight about foot and ankle, loose calf

- mould about LM /MM / TA

- covert to LL as high up as possible with soft cast

- use soft cast for this

 

Correction

 

Thumb on navicular, underhand, IF on heel

 

1.  Correct cavus

- increase supination / elevate first ray

- matching forefoot to midfoot / hindfoot

- pronating foot worsens cavus

 

2.  Increase abduction serially

- concept is rotation of calcaneus under the talus

- aiming to correct the STJ

- abduction / ER corrects the varus

- use talar head as fulcrum (Kite's mistake - cuboid)

- maintain elevation of first ray - avoid pronation

 

3.  No attempt to correct equinus til varus / adduction completely corrected

- usually by week 5

 

Note

- forceful manipulation to correct equinus prior to correction of hindfoot varus

- will result in either a rockerbottom deformity or a flat top talus

 

Percutaneous tenotomy

 

Timing

- week 5 / 6

 

Indication

- abduction / ER 60o and DF < 10 - 20o

- 85% need tenotomy

 

Technique

- usually performed in OPD

- LA, beaver blade medially

- can go directly posterior

 

Post op

- ponsetti cast further 3/52 in abduction and DF

 

AFO

 

Once cast removed

- 23/24 hours

- 3/12

 

DB Boots

 

Denis-Browne / Mitchell boots

- worn at night until 4 years 

- shoulder width apart

- clubfoot 70o, normal foot 40o

- also corrects tibial torsion

- critical to success is compliance

- lack of compliance with DB boots strongly linked to recurrence

 

Results

 

Successful 90 - 95%

- 5% require PMR / Ilizarov correction

- 7 - 15% need T anterior transfer

 

Follow up

- until 8

 

Recurrence

- metatarsus adductus

- dynamic supination

 

Operative Management

 

Open clubfoot release

 

Timing

- aged 9/12 to one year

- usually sufficient for child up to three 

 

Going out of favour

- joint violating surgery

- may increase recurrence

- increase late stiffness

 

Approach Options

 

Cincinatti 

 

Incomplete circumferential incision

- perform prone

- good exposure and access, especially lateral

- disadvantage heel pad necrosis

 

Turco 

 

Posteromedial incision 

- curved from base of 1st MT above posterior tubercle of calcaneus to the T achilles

- difficult to explore the posterolateral corner

- may need a seperate lateral incision especially in older child

 

Norris-Carroll 

 

Two incisions

- curved incision from centre of os calcis to talonavicular joint 

- second incision halfway between T achilles & lateral malleolus

 

Clubfoot Releases

 

Medial

 

Identify and protect NV bundle

- first thing

- put vessiloop about them

 

Tendons behind medial malleolus

- T achilles z lengthened

- T posterior z lengthened

- single suture placed

 

Capsulotomy AKJ / STJ

 

Identify Knot of Henry above Abductor Hallucis

- reflect Abductor Hallucis downwards

- may be easiest to follow down from ankle

- release plantar fascia

- section / Z lengthen FDL / FHL

 

Open and reduce TNJ - K wire

 

K wire up through STJ

 

Lateral

 

Divide CFL

Open and reduce CCJ

- stabilise with K wire

 

Post op

- plaster for 6/52, then AFO 3/12

 

Results

 

 

Residual & Recurrent Deformity

Conditions

 

1.  Dynamic supination - weak peronei

2.  Metatarsus adductus with curved lateral border of foot 

3.  Tight T achilles

4.  Residual or recurrent equinovarus

5.  Overcorrection / valgus

 

Options

 

Soft tissue procedures

Bony procedures

 

Dynamic Supination

 

Anatomy

 

Tibialis anterior inserts into base first MT and medial cuneiform

 

Options

 

Split tibialis anterior tendon transfer / SPLATT

Full tibialis anterior tendon transfer / TATT

 

Both usually in combination with T achilles tenotomy

 

SPLATT

 

Indication

- < 3

- for dynamic supination / early recurrence

- correct with ponsetti cast 3/52 first

- transfer will only hold correction

 

Method

- supine, tourniquet

- 2 cm incision over base first metatarsal

- harvest lateral half of T anterior

- divide with knife distally and take off bone

- 2 cm proximal incision above extensor retinaculum

- insert tendon forcep under extensor retinaculum

- grasp lateral half of tendon

- pull proximally out of wound

- tendon will tear nicely along midsubstance

- 2 cm lateral wound over P brevis insertion into base 5th metarsal

- make tunnel under fat between lateral and proximal wound

- pass tendon forceps,  transfer T anterior subcutaneously 

- Pulvetaft weave to P brevis

- make split in P brevis, pass T anterior through

- tension tendon with foot held reduced / pronated

- suture onto itself multiple times

- Ponseti cast for 6/52

 

TATT

 

Indication

- > 3

- for dynamic supination / early recurrence

- need to have bony ossification lateral cuneiform

- indicated in 7 - 15% of cases

 

Method

- harvest full T anterior as above

- 3.5 mm drill hole through lateral cuneiform

- tie over button

 

Metarsus Adductus

 

Algorithm

- depends on age 

- depends if deformity in metatarsals or tarsus

 

Options

 

1.  ST release

- abductor hallucis brevis + plantar fascia

- young < 5

 

2.  MT osteotomies

- deformity distal to navicular

 

3.  Lateral column shortening  and medial ST release

- medial soft tissue release and medial column lengthening 

- shortening of the lateral column - decancellation of cuboid / closing wedge osteotomy

 

4.  TDRO / Tibial derotation osteotomy

 

Hindfoot EquinoVarus

 

Issues

 

Later age > 5

- soft tissue procedures insufficient

- bony procedures required

 

Options

 

1.  Lateral sliding calcaneal osteotomy / lateral closing wedge osteotomy

- varus

 

2.  Wedge tarsectomy

- correction of equinus

 

3.  Talectomy 

- rarely indicated

- arthrogryposis or severe neuralgic clubfoot

- resect cuboid also to maintain balance of columns

 

4.  Ilizarov

- excellent salvage

- correction of equinovarus

 

Hindfoot valgus

 

Aetiology

 

Overcorrection

 

Options

 

Lambrinudi - for fixed equinus in patients 10 years or older

 

Triple arthrodesis

- good correction of all elements of club foot 

- used in the late presenting child or late recurrence

- should not be used before foot has stopped growing because it shortens the foot / stops growth

 

 

 

Calcaneocavus

Definition

 

Deformity characterised by 

- dorsiflexion of calcaneus 

- plantarflexion of forefoot / plantaris / forefoot equinus

 

Both sides of foot have elevated arch

 

Aetiology

 

Weakness of triceps surae 

- polio - most common worldwide

- spina bifida

 

Polio LegPolio Leg

 

CP 

Spinocerebellar degeneration / Friedreich's Ataxia

CMT

Spinal dysraphism

Compartment Syndrome / Deep Posterior 

 

Pathology

 

Imbalance of T achilles and dorsiflexors

- dorsiflexion of calcaneus

- moves insertions of T achilles anterior

- further weakens the lever arm

 

Forefoot becomes flexed because of

- gravity 

- action of muscles during gait

 

Symptoms

 

Difficulty walking 

Obvious deformity 

Shoe fitting & wear problems 

Painful callosities

 

Signs

 

Elevated longitudinal arch 

 

Prominent heel with abundant callus on plantar aspect 

- called pistol grip deformity 

- usually claw toes 

 

Heel usually in neutral

 

X-ray

 

Lateral XR Foot 

 

1.  Calcaneal pitch > 30o

- dorsiflexion of calcaneus  

- < 20o normal

 

2.  Meary's angle > 10o

- talus - first metatarsal

 

MRI Spine

 

Exclude Spinal dysraphism

 

Management

 

Non-operative

 

Modification of shoe wear / orthoses

 

Operative

 

Principles

 

1. Correct deformity by soft tissue releases & osteotomy

2. Tendon transfer to calcaneus

 

Age 0-5

 

Indication

- spina bifida

- other conditions cause little disability

- don't operate

 

Operations

 

1.  T Achilles to fibula 

- prevents varus & stimulates fibula growth

 

2.  T Anterior to T Achilles

 

Age 5-12

 

Grice arthrodesis STJ + T Achilles transfer to fibula

 

Age > 12

 

Triple arthrodesis

 

 

Calcaneovalgus

Definition

 

Condition present at birth

- ankle dorsiflexed & in valgus

- foot abducted

 

Epidemiology

 

1 in 1000 births

 

Aetiology

 

Probably result of intrauterine positioning

- packaging defect

 

Associations

 

Oligohydramnios

DDH

 

Increased incidence of flexible pes planus in later life

 

Clinical

 

Foot can usually be completely corrected passively

 

DDx

 

Congenital Posteromedial Bow of Tibia

- foot dorsiflexed

- tibial bow can be palpated

- confirmed with Xray

- not harmful

- can be associate with LLD / 2 cm

- strong association with calcaneovalgus

 

Congenital Vertical Talus

- calcaneus in equinus

- rockerbottom deformity

- deformity can't be corrected

- confirmed on xray

 

Neurological Dysfunction

- spinal dysraphism L5

- absence of triceps surae function

- may be cutaneous manifestation on back

- confirmed with spine xray

 

Management

 

Usually resolves spontaneously in first year

 

If severe, treated with regular manipulation

 

Occasionally need plantarflexion-inversion splintage

 

 

 

 

 

 

 

Congenital Vertical Talus

Definition

 

Rockerbottom foot / Persian Slipper Foot

 

Epidemiology

 

Uncommon

 

50% bilateral

 

Doesn't delay walking

- may present in toddler with callus under talus head

 

Associations

 

Congenital anomalies

- CNS disorders

- spina bifida & diastematomyelia ~10%

- arthrogryposis

- neurofibromatosis 

- CDH

- Trisomy 15-18

 

Pathogenesis

 

Uncertain

- result of muscle imbalance

- arrested foot development

 

Theories

- contracture of T Achilles posteriorly pulling calcaneus into equinus 

- contracture EDL anteriorly pulling navicular dorsally

- absence of plantar intrinsics

 

Pathology

 

Bony

 

Calcaneus

- in equinus / valgus / laterally displaced

- sustentaculum tali hypoplastic & doesn't support head

 

Talus 

- hypoplastic & in plantar flexion 

- only posterior 1/3 articulating with tibia

 

Navicular

- displaced dorsally onto talus neck 

- wedge shaped

 

Forefoot abducted

 

Tendons

 

Posterior

- triceps contracted

 

Anterior

- T anterior, long extensors & peronei are contracted

 

T posterior & peronei subluxed anteriorly / become dorsiflexors

 

Joints

 

STJ facets abnormal

CCJ abnormal

 

Ligaments

 

Dorsal ligaments contracted

 

Interosseous Talocalcaneal Ligaments / Dorsal TN

- thickened and contracted

 

Attenuated spring ligament

 

Clinical Features

 

Plantar aspect of foot convex

- rocker-bottom appearance

 

Hindfoot

- heel in fixed equinus

- key differential positional calcaneovalgus

 

Forefoot

- dorsiflexed

- abducted

 

Head of talus prominent

- palpable medially in sole

 

Plus

- medial column long & convex

- lateral column abducted

- tight tendoachilles

 

Xray

 

Lateral

- talus vertical

- calcaneum equinus

- increased talo-calcaneal angle / near 90o

- TNJ dislocated

 

Maximum Dorsiflexion view

- fixed equinus, talus still vertical

 

Maximum Plantarflexion view

- irreducibility of midfoot onto hindfoot

- relationship of talus to navicular fixed & rigid

 

DDx

 

1. Severe positional calcaneovalgus

2. Severe idiopathic flatfoot (with oblique talus)

- can reduce the TNJ with DF

3. Valgus everted foot of CP 

4. Overcorrection of CTEV with broken midfoot

 

Management

 

Non-operative

 

Casting

 

Reverse Ponseti technique

- some good results reported

- stretches tissue preoperatively

 

Operative

 

Aims

 

Correct hindfoot equinus

Correct forefoot dorsiflexion

Reduce dislocated TNJ

 

Issues

 

One or two stage /  usually one stage

 

Timing

 

Between 12 and 18 months

 

Surgery

 

Cincinnati incision 

- patient prone

- from medial cuneiform to lateral malleolus

 

Release hindfoot equinus

- T Achilles lengthening

- posterior capsulotomy AJ & STJ

- release interosseous talo-calcaneal ligament

- release CFL / PTFL

 

Reduce TNJ

- capsulotomy TNJ & locate head

- K wire

 

Release forefoot dorsiflexion

-  lengthening of EDL, EHL & T Ant

-  ± calcaneocuboid capsulotomies 

 

Stabilise

- calcaneum fixed to tibia via wire through heel

- 2nd wire across TNJ in PF

- ± Wire across CCJ

 

Tibialis Anterior usually transferred to neck talus

 

Late Presenting

 

Age 2-6

- subtalar fusion if painful

- Grice arthrodesis

 

Adolescent

- triple arthrodesis

 

Metatarsus Adductus

Definition

 

Metatarsus varus

- adduction of the forefoot at the TMTJ

 

Intrauterine positional deformity / packaging defect

 

Epidemiology

 

1:1000

- M = F 

- bilateral in 50%

- 10% have CDH

 

Aetiology

 

Unclear

 

Theories

- intrauterine positioning defect

- prone nursing

 

NHx

 

85% resolve by age 3 years

 

Weinstein

- 31 patients 45 feet with 33 year followup

- 16 feet mild or mod deformity passively correctable, no treatment

- 29 feet partially flexible or rigid treated manipulation or casting

- good results in all the untreated feet

- 90% good results of the casted feet

 

No poor results

 

DDx

 

CTEV

ITT / PFA

Hyperactive Abductor Hallucis

Skewfoot

 

Clinical Features

 

Curved lateral border

- forefoot adducted & slightly supinated

- deformity usually fully correctable

- prominent base of 1st metatarsal

 

Heel Bisector line

- line through midline axis hindfoot

- should pass through second web space

 

Intoed gait

- exclude ITT

- exclude PFA

 

DDx CTEV

 

Metatarsus adductus

- full range of ankle dorsiflexion & mobile hindfoot

 

Classification

 

Grade 1 - actively correctable deformity

Grade 2 - passively correctable

Grade 3 - unable to passively correct

 

X-ray

 

Not routinely required

 

Management

 

Non-operative

 

Algorithm

 

< 6/12

- observation only

- sleep supine

 

> Age 6/12

- rigid deformity

- serial casting changed every 2 to 3 weeks

- 8-12 weeks treatment

- most will tend to partially recur over time

 

Operative Management

 

Indications

 

Controversial

 

Surgery if difficulty wearing shoes / severe / > age 3

 

Options

 

1.  Abductor hallucis release 

 

2.  MT osteotomy

 

3.  Supramalleolar derotation osteotomy

 

4.  Opening wedge osteotomy medial cuneiform, closing wedge osteotomy cuboid, osteotomies of 2-4

 

Pes Planus

Definition

 

No good definition

- flexible flat foot

- medial longitudinal arch of foot in weight-bearing is in contact with ground or closer to ground than 'normal'

 

Epidemiology

 

Common

- almost always bilateral

- strong familial tendency

 

Aetiology

 

Physiological

 

All infants have flat feet

- at birth foot is in calaneovalgus & there is no medial arch

- when child begins to walk, feet evert & ER 

- foot has large medial fat pat 

 

Arch begins to develop in 2nd & 3rd year to variable degree

 

Thus flatfeet are

- usual in infants

- common in children

- in normal range for adults

 

Compensatory

 

Due to another anatomical variation

 

1. Genu Valgum

- physiological knock-knees most pronounced at age 3-4

- leads to apparent flatfoot

- corrects by ~ age 6

 

2. Out-Toeing

- ER of foot causes body weight to fall anteromedial to ankle

- result is valgus of heel & flatfoot

 

3. Tight Tendo achilles

- lack of DF compensated by heel eversion & forefoot pronation

 

4. Joint laxity

- i.e. Marfan's, Ehlers-Danlos

 

History

 

Almost always asymptomatic

- may cause aching midfoot

- pain incidence may equal general population

 

Examination

 

On weight bearing have combination of

- flat longitudinal arch

- pronated forefoot

- valgus heel

 

Flexible flatfoot

1. Foot appears normal when suspended / NWB

2. Recreation of longitudinal arch & heel varus on toe raise / windlass

3. Recreation of longitudinal arch by passive DF of Hallux (Jack's test) with weight bearing

4. Mobile or hypermobile STJ

5. Weight bearing callus on lateral longitudinal arch

 

Physiological FlatfootJacks TestSingle Heel Raise Arch Restoration

 

Must look at back

- exclude spinal dysraphism

 

DDx

 

Congenital

 

Flexible

- compensatory - tight T achilles / out-toeing / genu valgum

- physiological

 

Rigid - CVT / tarsal coalition / skewfoot

 

Acquired

 

Trauma - midfoot fracture / Lisfranc / rupture spring ligament / rupture plantar fascia

Neuromuscular - CP, spina bifida, polio

 

X-rays

 

Pes Planus Xray Mearys Angle

 

Weight bearing lateral and AP

- Meary's angle - talo - first metatarsal < 100

 

Harris axial / oblique for coalition

 

Cobey's 

- hindfoot alignment view

- see that calcaneum is under the fibula not tibia

 

CT

 

Look for coalition

 

MRI

 

Identify

- coalition

- inflammatory arthritis

- tibialis posterior dysfunction

 

Management

 

Non-operative

 

NHx

 

Usually resolves by age 5 or 6

- 20% adults have some degree of asymptomatic flexible flatfoot

- no treatment needed unless symptomatic

 

Some patients will suffer from midfoot pain

 

Orthoses

 

Results

 

Wenger 1989 JBJS

- orthoses & shoe modifications have no effect on outcome

 

Will not reverse pes planus

 

Indications insoles

- relieve pain 

- allay parental anxiety

- improve life of footwear

 

Options

- soft - heel cup + arch support

- hard - custom moulded insole / UCBL insert

 

Operative

 

Indication

 

Disabling pain not responsive to non-operative measures

 

Options

 

Skeletally immature

- Grice arthrodesis

- subtalar arthroesis + plantarflexing medial cuneiform osteotomy

 

Skeletally mature

- medial sliding calcaneal osteotomy

- lateral column lengthening + 1st metatarsal plantarflexing osteotomy

 

Subtalar arthroeresis with plantar flexing medial cuneiform osteotomy

 

Concept

 

Sinus tarsi implants

- axis altering device / blocking

- resist excessive pronation

- prevent adaptive changes

 

Lateral column lengthening / Evans procedure + 1st Metatarsal Osteotomy

 

Concept

 

Lengthen lateral column

 

Have to combine plantarflexion first MT osteotomy to enable toe to touch floor

 

Pes Planus Lateral Column Lengthening FDL transfer and Metatarsal OsteotomyPes Planus Lateral Column Lengthening FDL transfer and Metatarsal Osteotomy

 

Technique

 

Incision

- oblique Ollier's / from tip of fibula

- must preserve peroneals and sural nerve

- reflect peroneals  superiorly

- elevate EDB

 

Osteotomy

- 1.5cm proximal to CCJ

- vertical incision in periosteum

- osteotomy between middle and anterior facets medially

- care to protect medial NV bundle

 

Lengthening

- use osteotome to free medially

- lamina spreader

- open 1 cm, bone graft

- fixation varies - plate, staple

 

Medial incision

- medial reefing of spring ligament

- tightening of tibialis posterior +/- FDL transfer

 

First metatarsal plantarflexion osteotomy

 

Polydactyly

Classification

 

Pre-axial / great toe / 15%

Central / 2-4 MT / 5%

Post-axial / 5th MT / 80%

 

Type A - articulated

Type B - rudimentary 

 

Epidemiology

 

2 in 1000 births

- 30% positive FHx

- autosomal dominant

 

Associated MT anomalies common

- block MT / Y-shaped / T-shaped /wide head

 

Treatment

 

Rudimentary / Type B

- ligate at birth 

- allow to auto-amputate

 

Articulated / Type A

- excise at 1 year

- do at any time before school

 

Pre-axial

- medial toe excised

 

Post-axial 

- usually lateral excised 

- unless have syndactyly to 4th

- address MT or syndactyly at same sitting

 

Syndactyly

 

Types

 

Polysyndactyly:  duplication + webbing

Zygosyndactyly:  webbing alone

Acrosyndactyly:  distal webbing / Apert's

 

Managment

 

Usually none required

 

 

Skew Foot

Associations

 

Larsen Syndrome 

- AD, very rare

- associated with ligamentous laxity and dislocations, amongst other things

 

X-ray

 

Hindfoot valgus

Talonavicular lateral subluxation

TMT adduction

 

Management

 

Difficult to treat

 

Technique

 

1.  Medial calcaneal sliding osteotomy

 

2.  Opening wedge cuboid osteotomy

 

3.  Closing wedge cuneiform osteotomy

 

4.  Metatarsal osteotomy

 

Tarsal Coalition

Definition

 

Congenital fibrous, cartilaginous or bony connection of 2 or more tarsal bones

- due to failure of segmentation

 

Peroneal Spastic Flat Foot

- tarsal coalition

- tarsal pain

- reduced STJ motion

- rigid pes planus

- peroneal muscle spasm / tightness

 

Epidemiology

 

Present in 6% of population

- symptomatic in 1% of population

 

Bilateral in 50%

 

20% multiple coalitions

 

AD with variable penetrance

 

Calcaneo-Navicular most common (2/3)

- talocalcaneal middle facet is next most common (1/3)

- rest uncommon

 

Associations

 

Symphalangism (congenital end to end fusion of phalanges)

PFFD

Fibula hemimelia

Other gross limb anomalies

 

Aetiology

 

Secondary to failure of differentiation & segmentation of mesenchyme

- supported by intertarsal bridges in fetal tissue

 

Pathology

 

Multiple coalitions may occur

- do 2 plane CT with fine cuts to look for other coalitions

- will get poor results if not addressed

 

May develop ball and socket ankle joint

- due to stiffness of STJ

- develop inversion / eversion in AKJ

 

Valgus deformity leads to adaptive shortening of peroneal tendons

- may cause reflex spasm of tendon

 

Classification

 

1. Location

- CN / TC / TN / CC

 

2. Ossification

- synostosis - completely ossified

- synchondrosis - partly cartilaginous

- syndesmosis - fibrous

 

NHx

 

Majority are asymptomatic & remain so in adulthood

 

If symptomatic, symptoms usually develop in adolescence when bar ossifies

- due to reduction in STJ movement & joint stress

 

Calcaneonavicular coalition

- 8 - 12 years of age

 

Talocalcaneal coalition

- 12 - 16 years of age

 

History

 

Present with

- recurrent ankle sprains

- pain over sinus tarsi or over sustentaculum tarsi

- vague aching pain aggravated by activity

 

Signs

 

Stiff STJ 

- especially talocalcaneal bar

- may still have movement if not ossified

 

Fixed Pes Planus 

- doesn't correct on heel raise or Jack's Test

- heel doesn't swing into varus

- valgus heel with talocalcaneal bar

 

Peroneal tendons may be shortened but rarely spastic

 

X-ray

 

Calcaneonavicular

 

Oblique xray

- often diagnoses CN bar

- Anteater sign - elongated process on calcaneus or prolongation of navicular 

 

Talocalcaneal

 

1.  Talar Beaking

- very suggestive of TC bar

- traction spur due to increased stress

 

Talar Beaking

 

2.  C sign

- seen on lateral xray

- continuous C shaped line

- from talus to sustenaculum tali

 

3.  Ball and socket ankle joint

- secondary to TC bar

 

4.  Harris axial view

- visualise talocalcaneal

- 40° axial view shows middle facet 

- ski jump view

 

MRI

 

May be helpful for cartilaginous or fibrous bar

 

CT scan

 

Very good for bony bars

 

DDx

 

Any condition that injures STJ

- traumatic / osteochondral fracture

- inflammatory / RA

- tumour / osteoid osteoma

- infection

 

Flexible flat foot

 

Management

 

Non-operative

 

Options

 

Avoid aggravating activities

Moulded longitudinal arch support

SL FWB cast for 6/52

NSAIDS

 

Operative

 

Indications

 

Persistent pain

Minimal degenerative changes

 

Options

 

Resection of bar

Isolated STJ fusion - degeneration of STJ only

Triple Arthrodesis - rigid planovalgus foot

 

Criteria for Resection

 

All relative

- young < 14 years

- absence of complete bony bar

- no degenerative changes 

- presence of talar beaking is not a contraindication

- no fixed deformity

 

Calcaneonavicular bar resection

 

NHx compared with talo-calcaneal bar

- possibly settle down long term

- better prognosis / less arthritis / present younger

- more likely amenable to resection

- most do well

 

Technique

 

Aim is 1 cm gap

 

Ollier approach

- 1cm distal to fibular tip

- obliquely across sinus tarsi

- to superolateral margin TNJ

 

Superficial Dissection

- protect superficial CPN

- EDL & P tertius anteriorly / peroneals plantarward

 

Deep dissection

- elevate EDB proximal to distal  

- beware of its motor branch from DPN

- show sinus tarsi / anterior process calcaneum

- expose bar

- may need to open TN and CC joints to know exact location

 

Resection

- resect 1cm of bone with osteotomes

- protect talus and STJ from damage

- check with on table oblique lateral II

- suture fat / EDB into defect / over button and felt pad

 

Post op

- 2 weeks POP

- moon boot / WBAT /ROM

- button out at 6/52

 

Results 

 

Gonzalez et al JBJS Am 1990

- 75 feet in 48 patients

- good or excellent results in 77%

- poor in 7%

- best results with cartilaginous coalition and patients < 16

 

Mubarak et al J Pediatr Orthop 2009

- CN resection and fat graft interposition

- 5% incidence of symptomatic regrowth requiring repeat resection

- 74% had improvement of subtalar joint motion

- 82% improvement of plantarflexion

- felt fat graft better choice than EDB as can completely fill gap

 

Talocalcaneal Bar

 

NHx 

 

1/3 develop arthritis

- operative results not as successful as CN

- resection more difficult

- results less predictable

- tend to be more conservative with this than CN bar 

 

Prognosis

 

Favourable

- < 16 years

- < 50% surface area of posterior facet 

- nil arthritic changes

- < 16o valgus

 

Poor

- > 50% of post facet 

- i.e. middle facet bar is > 50% of posterior facet

- heel valgus > 16°

- narrowing of STJ i.e. arthritic changes

- lateral talar process impinging on calcaneum

 

Options

 

Resection 

- is best option if failed non-operative

- always worth trying prior to arthrodesis

- try to return some STJ motion

 

Calcaneal Osteotomy

- re-centres heel

- realigns weight bearing

- not everyone uses it

 

Arthrodesis

- in mature foot > 12 years

 

Resection Technique 

 

Incision

- curved incision

- navicular tuberosity to medial border T Achilles

- 2cm superior to superior calcaneal tuberosity

 

Superficial dissection

- through flexor retinacular sheath 

- elevate T Post and FDL tendon anteriorly

- neurovascular bundle and FHL retracted plantarward

- identify posterior facet

 

Resection

- resection of bone until middle facet seen + mobile

- remove more bone from talus than sustentacular side 

- 2/3 of resection from talus

- key to operation is anterior process of calcaneus & follow posterior

- fat graft or silicon insertion

 

Post operative

- early ROM important

 

Talonavicular

 

Very rare

- ossifies at 3-5 years

- therefore symptoms early

 

 

Toe Walking

Epidemiology

 

70% male

70% positive FHx

20% learning difficulties

 

DDx toe walking

 

Early / late onset

Unilateral  / bilateral

 

1.  Habitual

 

Toddlers - child can heel walk

 

2.  Structural

 

Fixed equinus - CTEV / arthrogryposis

Short / tight T Achilles

Space occupying lesion in calf

 

3. Neuromuscular

 

CP

Spinal dysraphism

Duchenne's Muscular Dystrophy

CMT

 

4. Short Leg

 

Real (DDH)

Apparent (knee or hip deformity)

 

Natural History Habitual

 

Initially toddlers walk on toes 3-6/12

- graduate to strike on flat of foot

- rinally develop normal heel-toe gait

- mature gait pattern by 3 years

 

Idiopathic Toe walker

- normally bilateral

- usually from birth

 

Beware 5 year old who has recently begun to toe walk, especially unilateral

 

History

 

Gestational 

Birth 

Family history

How long on tip toes ?

Walking age 

All the time or not ?

 

Examination

 

Rule Out

 

1. CP

2. Duchenne's MD

3. CMT

4. Short Leg / DDH

5. Dysraphism

 

Hip

- Trendelenberg 

- LLD

 

Spine 

- signs spinal dysraphism

- scoliosis

 

Gait

- Heel / Toe walk 

- running coordination - hemiplegic / brings out very minor posturing / look at UL

 

Gower's sign

- Duchenne's muscular dystrophy

- ask to squat then stand up

- have to hand walk up their legs as severe proximal muscle weakness

 

Upper limbs

- for hemiplegia, increased tone

 

Foot

- active +/- passive ROM 

- sole of foot - if always on toes then heel will be soft

- Silverskiold

 

Neurological exam 

- especially abdominal reflexes

 

Investigations

 

Spine Xray / MRI

 

Gait analysis 

 

EMG 

 

Serum CPK

- DMD > 5000

- proceed to muscle biopsy if elevated

 

Nerve biopsy - rare but may need in peripheral neuropathy

 

Management

 

Non Operative

 

Stretches 

Serial casting 

Botulinum A toxin in CP 

AFO 

 

Operative

 

Percutaneous T achilles lengthening 

 

Timing

- ? about 7 years or older

- wait till at least > 4 years

- many resolve by age 10

 

Results

- 75% will still toe walk after treatment  

 

 

 

Hip

Acetabular Dysplasia

Acetabular Dysplasia

DefinitionLateral Subluxation Hip

 

Abnormal development of acetabulum with decreased femoral head coverage

 

Aetiology

 

DDH 

Perthes

SUFE

NMD e.g. cerebral palsy

Skeletal Dysplasia (MED etc)

 

Plus

- trauma

- infection 

 

Epidemiology

 

Females > Males

- keeping with DDH

- males usually with other diagnosis

- considerable number are asymptomatic diagnosis on X-ray

 

Problem of late teens / early twenties

 

NHx 

 

3 important factors

 

1.  Degree of lateral subluxation

- CE < 20° = 80% OA

- unknown if covering head decreases OA

- 20 - 50% so called primary OA due to dysplasia

 

2.  Young age

- acetabulum can remodel to age 5

- dysplasia improves only with concentric reduction

- most rapidly in first 6 months

 

3.  Symptomatic 

- natural history of asymptomatic subluxation is unknown

- likely to be much more benign

- it is known that early symptomatic acetabular dysplasia has a much poorer prognosis

- i.e. the twenty year old with a symptomatic hip will likely have severe arthritis in ten years

- untreated DDH said to lead to OA by age 50 in 50%

 

Weinstein JBJS July 2000

 

1.  Subluxation 

- all get severe OA & clinical disability

- age of symptoms depends on severity

 

2.  Dysplasia 

- NHx difficult to predict

- strong association between women & degenerative joint disease

 

3.  Dislocated hips (untreated) 

- may have no disability

- if have well developed false acetabulum may develop severe DJD

 

Clinical

 

3 peaks of patient presentation

- depends on degree of subluxation

 

1.  Severe subluxation - patient in teens

2.  Moderate subluxation - patient in 20 & 30's

3.  Minimal subluxation - postmenopausal patients

 

Acetabular Rim Syndrome Ganz JBJS Br 1991 

- patients with acetabular dysplasia found to have 20% incidence of labral tear

- complain of sharp knife like pain in groin and giving way or locking

- elicited by flexion and IR

- brings proximal & anterior femoral neck into contact with rim of acetabulum

- this is where labrum is likely to be damaged -> superolateral quadrant

 

X-ray Measurements

 

1.  Shenton's Line

- Cooperman found it to be best prognostic sign

 

2.  Acetabular Index 

- paediatric

- triradiate cartilage to lateral edge of acetabulum

- birth 30°

- at 2 years - 20°

- N = < 30°

- DDH > 35°

 

3.  Sharp's Acetabular Angle 1961

- adult 

- inferior tip tear drop to lateral edge acetabulum

- > 42° Abnormal

 

Sharp's Acetabular Angle /> 40

 

4.  Centre Edge Angle of Wiberg 1939

- Perkin's line and line to centre femoral head

- < 20° abnormal / dysplasia

- > 40° Protrusio

- demonstrated increased increased of OA smaller the CE angle

 

Centre Edge < 20 degrees

 

5.  Sourcil angle

- line of angle of sourcil (sclerotic weight bearing area of femoral head)

- horizontal line

- normal     < 10°

- dysplasia  > 10° 

 

Sourcil Angle /> 10 degrees

 

6.  Weinstein Extrusion Index

- Head Covered / Total Width

- < 75% head covered abnormal

 

7.  Lateral Subluxation 

 

Teardrop to femoral head

 

Ganz Group I   13+/- 4mm

        Group II    6+/- 2mm

 

8.  Peak to Edge Distance

 

Ganz group I    3+/- 5mm

         group II 16+/- 4mm

 

CT Reconstruction

 

Probably best anatomical view of hip prior to deciding on osteotomy

 

Management

 

Aim is to decrease cartilage stress below stress threshold

- by increasing joint surface area  & decrease joint reaction force

- decreasing later OA & providing a painless functional joint

 

Controversy regarding treatment painless hip  with CE < 20o

 

Options 

 

1. Femoral osteotomy

- varising ostetomy

 

2. Acetabular osteotomy

- Reconstructive - Salter / Ganz

- Salvage - Chiari / Shelf

 

3. Both

 

4.  Hip Arthroscopy

- debridement labral tear

 

 

 

Pelvic Osteotomies

Aim

 

Establish normal biomechanical forces about the hip

 

Decision Making

 

1.  Will the hip remodel? - < 8 years old

2.  Can congruency be obtained?

3.  How much correction is required?

 

Types of Pelvic Osteotomies

 

1.  Reconstructive Osteotomy

- redirectional

- reshaping

 

2.  Salvage Osteotomy

 

Reconstructive Osteotomy

 

Best option for the young patient with symptomatic acetabular dysplasia

- i.e. AI > 40o and CE < 20o with mild OA

 

Indications

- AI > 40o

- CE < 20o

- symptomatic dysplasia 

- congruent joint

- spherical head

- preserved articular space (relative) / mild OA

- minimum 70° flexion / extension arc

- subluxation can be reduced

 

A.  Redirectional

 

Single osteotomy - Salter

 

Double osteotomy - Sutherland

 

Triple - Steele / Tonnis / O'Hara Interlocking Modification

 

Periacetabular Osteotomy - PAO / Bernese / Ganz

 

B.  Reshaping

 

Pemberton / Dega

 

Salvage Osteotomies

 

Indication

 

If the OA is too severe for a reconstructive osteotomy

 

In the age of modern THR

- THR is probably a more reproducible option than a salvage osteotomy 

 

Options

 

Shelf /Staheli / Melbourne

Chiari

 

Specific Osteotomies

 

1.  Salter

 

Concepts

- single osteotomy /  innominate

- can do in older patient but difficult unless experienced with operation

- relies on motion at symphysis pubis to rotate acetabulum

 

Indications

- can't do bilateral Salters

- mild to moderate dysplasia / DDH

- concentric reduction

- near normal ROM

- no OA

- undeformed head

 

Advantage

- correct CE 10o

 

Disadvantage

- add 1 cm LL

- leave deficient posteriorly

 

Results

 

Salter CORR 1974

- 53 adults 4 year followup

- increased CEA by average 18° 

- decreased AI average 50° to 38°

- 63% no pain / 40% no limp (compared with all patients preop)

- 79% trendelenburg negative (compared with 96% positive preop)

 

Technique

 

Supine

 

Smith Petersen approach

- anterior 2/3 crest

- 5cm below ASIS

- between TFL and Sartorius

- protect LFCN medially

- tie ascending branch LCFA

- between G medius and R femoris

- clear glutei and iliacus off pelvis

 

Right angle about G Sciatic notch

- protect SGN and SGA

- gigli initially in notch

- anterior saw blade above acetabulum between AIIS and ASIS

 

Leg in figure 4

- rotate acetabulum anterolaterally through symphysis pubis

- x-ray shows narrowing of obturator foramen

- 30o iliac wedge crest

- fix with threaded K wires

- protect for 6 / 52

 

2.  Sutherland

 

Concept

- double osteotomy

- Salter plus osteotomy lateral to pubic symphysis

 

Disadvantage

- can be difficult to move because osteotomy further away from acetabulum 

 

3.  Steele 

 

Concept

- triple / innominate osteotomy

- salter with two pubic osteotomies

- similar to Tonnis but ostetomies are a bit further away from acetabulum

 

Indications

- for older child with less mobile pubic symphysis

 

Technique

- osteotomy at base of ischial rami via posterior approach

- osteotomy superior pubic rami

 

Advantage

- allows more correction whilst not laterally displacing the joint as a Salter tends to do

- can improve CE 20o

 

4.  Tonnis

 

Concept

- Salter osteotomy plus pubis and ischial between ischial tuberosity and acetabulum

- get better or easier correction because osteotomies are closer to acetabulum

 

5.  Ganz

 

Concept

- periacetabular osteotomy

- posterior column left intact for stability

 

Indications

- older adolescent and adults

- with residual dysplasia

 

Advantage

- excellent rotation with medialisation

- increase CE 27o

 

Technique

- isolate acetabulum from ileum / ischium via 3 part osteotomy

- separate superior rami osteotomy at base

 

6.  Dial

 

Concept

- curved osteotomy following periphery of acetabulum

- don't see sciatic nerve

- a lot of operation is done by feel

 

Disadvantage

- risk of acetabular AVN and fracture

- technically very difficult

 

7.  Pemberton 

 

Concept

- incomplete transiliac osteotomy

- cut into iliopubic and ilioischial limbs triradiate cartilage

- bends through triradiate cartilage

 

Indications

- only in those < 8 with open triradiate cartilage

 

Advantage

- decreases volume of acetabulum

- provides very good cover

 

Disadvantage

- may sublux the hip after reduction so always check

 

8.  Dega

 

Concept

- incomplete trans-iliac osteotomy

- rely on plasticity of superior acetabular margin rather than triradiate

 

Advantage

- excellent reduction of AI 

- improvement CE average 31o

- very stable

 

Technique

- anterior approach

- osteotomy sites 15 mm from articular surface

- hip capsule not violated

- anterior and middle portions ileum cut

- leave posterior wall and G sciatic notch intact

- directed downwards

 

9.  Shelf

 

Hip Lateral SubluxationHip Subluxation Reduced with AbductionShelf Osteotomy

 

Indications

- older child > 12 years

- congruent reduction impossible

 

Concept

- extra-articular bony buttress over uncovered anterolateral portion of  femoral head

- iliac wing graft placed over hip capsule

- place graft under reflected head of RF

- can be modified depending on where cover is needed

 

Disadvantage

- if not weight bearing (i.e. too high) will undergo resorption

- if too low, will cause pain

 

Stahelli Technique

- take off reflected head of rectus

- supra-acetabular slot, matchstick corticocancellous iliac graft

- graft strips are placed into slot which is checked radiographically to ensure as low as possible

- rectus tied over the top

- pack morselised graft over the top 

 

10.  Chiari

 

Concept

- medial displacement to develop shelf

- cartilage interposed to develop fibrocartilage

 

Results

 

Luiket al  JBJS 1991

- 82 of Chiari's original patients

- 20 required THR

- 75% good

- best results: 80% coverage, < 45 years, high osteotomy slope, adequate medialisation

 

Reynolds JBJS 1986

- 90% success at 5 years

 

Pitfalls

1. Distal fragment must go medially & adducted

2. Must angle osteotomy anterolaterally distally to posteromedial proximally

3. Anterior defect needs bone grafting

 

Specific Indications

 

DDH

 

Dislocation / 18 months

- Salter

- Dega

- Pemberton

 

Residual Dysplasia

 

Before Maturity

- Salter (through pubis)

- Dega

- Pemberton (through triradiate)

 

After Maturity

- Tonnis

- PAO i.e. Ganz

 

Salvage

- Chiari / Shelf

 

Perthes

- Salter

- Triple

- Shelf

 

CP / Neuromuscular

- Dega / Triple

- Shelf / Chiari

 

 

Coxa Vara

Definition

 

Deformity of proximal femur with neck-shaft angle <125°

 

Characterised by

- coxa vara

- decreased femoral anteversion

- limp / trendelenberg

- stress fractures

- early OA

 

Classification   

 

"ACDDC"

 

Acquired

 

2° to underlying disorder

- rickets

- renal osteodystrophy

- hyperparathyroidism

- Perthes disease

- infection

- trauma with early closure physis

- tumour

- SUFE

 

Congenital Coxa Vara

 

Dysplasia

- MED

- SED

- Achondroplasia

- Cleidocranial dysostosis

- Fibrous Dysplasia

 

Developmental / Infantile

- progressive disorder that develops in early childhood

- due to limb bud abnormality

- not congenital really infantile as appears after birth

- includes PFFD & congenitally short femur

 

Cretinism  (hypothyroid)

 

Developmental Coxa Vara

 

Epidemiology

- rare

- sex & side incidence equal

- bilateral in 1/3

- increased familial incidence - AD

 

Aetiology

 

Unknown / Multifactorial

 

Theories:

1. Metabolic abnormality - deficiency in proximal femur ossification

2. Excessive intrauterine pressure - causes depression in femoral neck

3. Non-specific mechanical abnormality - occurs during development

4. Vascular insult - arrest in neck development

5. Localized dysplasia - faulty maturation of cartilage & bone in femoral neck

 

Histology

 

Abnormality in medial proximal physis & adjacent neck

- cartilage

- 2° metaphyseal bone

 

Abnormality characterised by

- increased width of physis

- loss of progress of columns

- nests of cartilage in metaphysis

- porotic metaphyseal bone

 

NHx

 

Mild

- epiphyseal angle < 45°

- corrects spontaneously

 

Severe

- epiphyseal angle > 60°

- neck - shaft angle < 110o

 

Bilateral severe coxa vara with OA

 

Issues 

 

1.  Limp / trendelenberg

 

2. Stress fracture of femoral neck

 

3.  Early degenerative changes 

- untreated get severe early OA & often require THR early

 

Symptoms

 

Present at walking age with abnormal gait

- painless limp

 

Signs

 

Patient is short with hyperlordosis of spine & waddling gait

- limb-length discrepancy

- trendelenburg sign

- mimic DDH 

 

Gait

- short-leg

- trendelenburg sag 

- abductor lurch

- if bilateral - waddling gait

 

Decreased ROM 

- especially abduction & IR

 

Radiology

 

Inverted Y

- inferior sclerotic metaphyseal triangle

- pathognomonic of developmental

 

Varus femoral neck

- neck-shaft angle < 125° (normal is 150° in infant)

- difficult to define with severe disease

 

Hilgenreiner's Epiphyseal angle 

- angle between Hilgenreiner's & Physeal line

- normal < 25°

- < 45° should resolve

- 45-60° - watch

- > 60° will progress

 

Also

- decreased femoral anteversion / retroversion

- coxa breva

 

Management

 

Goals

 

Correction of varus angle

Conversion of femoral neck forces from shear to compression

Correction of LLD

Establish correct abductor tension

 

Management based on Epiphyseal Angle

 

<45°    - no treatment

45-60° - observe

>60°    - valgus osteotomy

 

Operative Indications

 

1.  Epiphyseal Angle > 60° 

2.  Epiphyseal Angle 45-60° with limp & progression of varus

 

 

Aims

 

Valgus derotation subtrochanteric osteotomy

- need to overcorrect to 150˚

- epiphyseal angle < 40o

- correct to anteversion 10o

 

Technique

 

Lateral approach

- K wire in central head

- mark distal and proximal with drill hole for rotation

- open periosteum and protect with homans

- sub-trochanteric osteotomy with saw

- application of 150o Synthes offset locking plate

- need also IR of about 20° at time of osteotomy

 

May require

- adductor tenotomy

- femoral shortening

- GT transfer

 

Plaster spica for 6 weeks post-op

 

If bilateral do about 6 months apart

 

Complications

 

1.  Loss of correction 

- related to undercorrection

 

2.  Premature physeal closure 

- related to increased pressure

- seen in 90% cases

 

3.  Greater trochanter overgrowth 

- associated with premature physeal closure

 

4.  Acetabular dysplasia 

- associated with premature physeal closure and undercorrection

 

 

 

 

DDH

Background

Definition

 

Developmental dysplasia of the hip

- the femoral head does not have the normal relationship with the acetabulum

- child with subluxable, reducible or irreducible hip

- may not be present at birth (hence not called CDH)

 

Instability

- ability to dislocate or reduce femoral head into or out of acetabulum

 

Dislocated 

- complete loss of contact

 

Irreducible 

- unable to be reduced

 

Subluxation 

- partial contact

 

Dysplasia 

- an abnormal acetabulum 

- it is both shallower than normal & its inclination is more vertical than normal

 

Epidemiology

 

Incidence 

- DDH = 2: 1000 (requiring treatment)

- instability 10 : 1000 

 

Influenced by

- presence of screening

- age of baby at examination

- experience of examiner

 

Factors

- females 7x 

- first-born

- L > R (L 2/3 of cases / LOA lie)

 

Bilateral 20%

 

Associations

 

Congenital Torticollis - 20%

Metatarsus Adductus - 2%

 

Screening

 

Nursery screening has decreased incidence of late dislocation

- decrease of 85% in incidence of walking CDH

- increase of early instability

 

Aetiology

 

1.  Ligamentous laxity

 

Different in sexes

- maternal hormones

- female baby have increased response to relaxin 

 

Familial hyperlaxity in males

- usually familial hyperlaxity with collagen alteration

 

2.  Positional

 

A.  Breech position 

- important risk factor

- 25% of DDH were breech

- normally 2.5% of births

 

Female breech

- 1 :35 = dislocated hips

- 1:15 = unstable hips

 

B.  Oligohydramnios

 

C.  Twins

 

D.  Firstborn - ? small uterus 

 

E.  Increased L>R

- usually LOA (vertex) position 

- adduction left hip against mother's spine

 

E.  Post-natal

- decreased where babies carried astride waist (Eskimos and Africans)

- increased where babies strapped to cradle board (Indians)

 

3.  Genetic

 

Nature of genetic predisposition unclear

- may be related to familial ligamentous laxity

- increased in Indians and Lapps

- decreased in Chinese and Africans

 

If child has DDH

- 5% of siblings DDH

- 1% of brothers 

- 10% of sisters 

 

Parent 

- 12 % for child

 

4. Teratologic Dislocation 

 

Associated with other malformations

- irreducible at birth

- Arthrogryposis

- Larsen's

 

Pathology

 

Newborn

- hip is spontaneously dislocating & reducing

- minimal anatomical changes

- lax joint capsule

- shallow acetabulum

- flattening of posterosuperior labrum

 

Dislocated

 

Acetabular Dysplasia

- muscle action encourages proximal and lateral migration of head

- acetabulum and head rely on each other for development

- unreduced acetabulum becomes vertical and shallow

- becomes more anteverted

 

Femoral head

- dislocates upwards & backwards

- head becomes more globular & less spherical

- neck becomes more anteverted

 

Capsule

- enlarges & narrows at isthmus 

- where iliopsoas crosses

- Zona Orbicularis

 

Ligamentum teres

- longer & thicker

 

Pulvinar

- fills with thickened fat pad

 

Transverse ligament

- pulled superiorly and blocks inferior socket

 

Labrum

- may invert

 

Subluxation

 

Space between head & medial wall of acetabulum widens

 

Dysplasia of acetabulum develops

- vertical

- shallow

- increased anteversion

 

NHx

 

50% of unstable hips resolve without treatment

 

Subluxation 

- acetabular dysplasia

- always leads to early OA

 

Dislocation

- long term follow up 

- 60% significant pain & disability

- 40% no pain but abnormal gait 

- increased incidence of pain & OA with well-developed false acetabulum

 

History

 

Suspected in Neonate

- female

- firstborn / breech / twins 

- FHx of DDH / ligamentous laxity

- metatarsus adductus / torticollis / calcaneovalgus

 

Infant

- difficulty with nappies due to limited abduction

- shortening of thigh

 

Child

- delay in walking or running

- limp

- increased lumbar lordosis

- unilateral toe walking

- intoeing

 

DDx

 

PFFD

Septic dislocation

SUFE

Coxa vara

Trauma

Neurological - CP, Spina bifida

 

Screening

 

All neonates within 48 / 24

- treatment begun ASAP

- ultrasound "At risk" population

 

Beware

- CTEV / torticollis

- breech

- FHx

 

Examination Neonate

 

Thigh folds

 

Reduced abduction

- dislocated

 

Ortolani Test

- hip is out, tests reduces hip

- thumb on adductor tubercle & ring finger on GT

- place in 90° flexion

- abduct both hips & lift GT forward

- clunk of reduction felt 

 

Barlow Provocation Test

- push hip back out

- one hand holds pelvis

- adduction to 10o while axial pushing thigh backward

- dislocates in this position over post lip / feel clunk of dislocation

- may feel sliding of subluxable hip

- very deficient lip may give false negative with no clunk

 

Hip may be

- stable 

- subluxable

- dislocatable 

- dislocated (reducible / not reducible)

 

Clicks

- not significant

- innocent clicks secondary to ligamentum teres / iliopsoas / iliotibial band

 

Examination Infant

 

Unilateral

- decreased abduction (< 60° suspicious, < 45° definite)

- apparently short femur - Galleazi's

- extra thigh folds

 

Bilateral

- more difficult

- symmetrical decrease of Abduction

 

Signs shortening above the GT

 

Klisic's line 

- line from ASIS to GT

- should point to umbilicus

- points horizontally in DDH

 

Nelaton's line

- line from ischial tuberosity to ASIS

- thigh adducted & flexed

- normally GT caudad to it

- in DDH GT cephalad to it

 

Examination Child

 

Unilateral

- limp

- abductor lurch / trendelenberg gait

- short-leg component

- decreased ROM / mild FFD / decreased abduction

- full rotation

 

Bilateral

- increased lumbar lordosis

- waddling gait / bilateral trendelenberg gait

- bilateral decrease ROM

 

X-ray

 

Timing

- AP after 6/12

- when SFE ossified 

- obturator foramina should be symmetrical / no rotation

 

Quadrants

 

Hilgenreiner's Line

- horizontal through triradiate cartilages

 

Perkin's Line

- vertical through lateral edge of bony acetabulum

 

4 quadrants

- ossific nucleus usually lies in inferior / medial quadrant

 

Findings

 

1.  Delayed development of ossific nucleus / smaller

 

2.  Ossific nucleus in upper / outer quadrant

 

3.  Disruption Shenton's Line

- curved line along inferior neck

- inferior border superior ramus

 

4.  Head to Teardrop Distance

- lateral tear drop to medial ossification centre

- use metaphyseal edge if no SFE

- wide medial joint space compared with normal side

 

5.  Acetabular Index

- angle between Hilgenreiner's & acetabular lines

- 30° at birth to 20° at 2 years

- Normal < 30°

- DDH > 35°

 

6.  Centre - Edge Angle

- angle between Perkin's & CE line

- measure of coverage of femoral head

- Normal 30°

- DDH < 20° Protrusio >40°

 

Von Rosen's view

 

Technique

 

AP pelvis with legs abducted 45° & IR 20°

- lines along femoral shafts should pass through centre acetabulum & intersect at sacrum

- DDH line not through acetabulum & intersects to side of sacrum

 

Indication

 

Use in late presentation

- if femoral head reduces with von rosen then suitable for pelvic osteotomy only 

- if head appears to articulate on edge of acetabulum may have  false acetabulum

- then must do open reduction, femoral osteotomy & pelvic osteotomy

 

Ultrasound

 

Background

 

Best imaging before 4 - 6 months when pelvis cartilaginous

- very accurate

- static & dynamic images

- coronal view most important

 

No role in general screening as is too sensitive

- many hips with alpha angles 50 - 60o will resolve

 

Alpha Angle

- between ilium & bony roof acetabulum

- normal > 60°

 

Beta Angle

- between ilium & cartilage roof / labrum  

- normal < 60o

 

Dynamic stress

- is the head reducible

- perform Ortolani's under ultrasound

 

Graaf's Classification

 

Type Alpha Beta    
1 > 60 < 60 Normal  
2A 50 - 59   Immaturity < 3/12  
2B 50 - 59   Delayed Ossification > 3/12  
2C 43 - 49 < 77    
2D 43 - 49 >77 Labrum everted  
3 < 43 >77 Dislocated  
4 <43 >77 Dislocated & Labrum interposed  

 

Arthrography

 

Normal hip shows projection of labrum

 

Abnormal hip shows

- widened medial joint space / medial pooling

- blunting / infolding of labrum superiorly

- enlargement of ligamentum teres

- neolimbus bulge in acetabular cartilage

- hourglass constriction of capsule

 

Useful in evaluating reduction intra-operatively

 

MRI / CT

 

Indication

- assess post operative hip reduction when child in spica

 

Management Algorithm

 

0 - 6 months

 

Attempt closed reduction

- Pavlik harness 

- von Rosen splint

 

6 - 18 months

 

Closed reduction

- adductor tenotomy / hip spica

 

Open reduction

- medial approach

- Smith Petersen approach

 

After age of 1 consider adding

- acetabular osteotomy to address acetabular dysplasia

- FDRO if required to obtain reduction

 

18 - 3 years

 

Dislocated hip

- open reduction + acetabular osteotomy

- femoral osteotomy if difficulty reducing hip

 

Acetabular dysplasia

- hip reduced but large acetabular index

- > 30o and not improving with time

- acetabuloplasty

 

> 3 years

 

Dislocated hip

- open reduction

- acetabular osteotomy to correct acetabular index

- hip will be difficult to reduce so need to shorten femur / FDRO

 

Complications Management

 

AVN

 

Aetiology

 

Result of treatment

- doesn't occur in untreated DDH

 

1.  Excessive abduction / splint / traction

2.  Forceful closed reduction

3.  Vascular damage at open reduction

 

Reduced by

 

Adductor tenotomy

Femoral osteotomy

Avoid medial reduction

 

X-ray Classification

 

Kalamachi and McEwan 1980

 

1. Nucleus only 

- temporary irregular fragmentation of SFE

- head will subsequently be normal

 

2. Lateral Physis 

- early lateral premature fusion

- femoral head will tilt into valgus

- lateral portion of femoral neck short

 

3. Central physis involved

- femoral neck will be short / coxa breva

- coxa vara secondary to due to GT growth

 

4. Whole physis involved

- femoral neck will be short & in valgus

- GT overgrowth

 

Outcome

 

Coxa breva / Coxa valga / Coxa vara

Relative overgrowth of GT

LLD

Trendelenberg gait

 

Management

 

1. Trochanteric Physiodesis

- to reduce overgrowth

- if <7

 

2. Trochanteric transfer

- if trochanter has reached level of femoral head

- transferred distally & laterally

 

 

 

 

 

 

Management 0 - 6 months

Aim

 

To obtain and maintain a concentric reduction without complication

 

Timing

 

0 - 6 /12

- best time for treatment

- maintain reduction of head & allow normal acetabular development

 

Equivocal Hip

 

Obtain ultrasound

 

Graaf 2A and 2B

- Alpha angle 50 - 59o range

- many will resolve without treatment

- reassess at 3/12 with ultrasound and 6/12 with xray

 

Graaf 2C or more / Alpha angle < 49o

- manage as below

 

Algorithm

 

1.  Hips subluxed or dislocated but reducible

 

Splint 6/52

- ultrasound 2/52 to confirm head enlocated

- check splint every 2 weeks (pressure sores / fitting correctly / femoral nerve / safe zone)

 

Ultrasound at 6/52

- alpha angle > 60° / no further splint

- alpha angle < 60° / 3/12 treatment in total

 

Results

- very successful in Graaf 2C / D and 3

- 50% successful in Graaf 4

 

Follow up

- xray at 6/12 until 2 years

- final assessment 12 years

 

Hips dislocated & irreducible

 

Management

- trial Pavlik harness 4/52

 

Failure treatement

- closed reduction +/- open reduction after 6/12

 

Hips locatable, but unstable in safe range abduction

 

Management

- trial harness only in safe range

 

Failure treatment

- closed reduction +/- open reduction after 6/12 age

 

Treatment Options

 

A.  Pavlik harness

- most common in world

 

B. Von Rosen Splint

 

C.  MUA / Spica

 

Pavlik Harness

 

Advantage

- can reduce dislocated hip

- able to clinically check reduction with harness on

- can ultrasound with harness on to check is reduced

 

Contraindication

 

Muscle imbalance / spina bifida

Stiffness / arthrogrypotic

Age over 8/12

 

Technique

 

Chest strap - at or slighly below nipple line

Shoulder straps 

Boots attached to chest strap

Anterior straps located in anterior axillary line 

- tightened to achieve 100o-110o flexion 

- no more or risk femoral nerve palsy

Posterior straps lightly tightened

- allow adduction to within 7 cm of knees touching

- maximum abduction 30 - 50o to reduce risk AVN

 

Follow up

 

Review 1 week

- ensure enlocated with US
- ensure no femoral nerve palsy

 

Repeat US at 6/52 and 3/12

 

Results

 

Mostert et al J Paediatr Orthop 2000

- prospective study of 41 hips treated with Pavlik harness

- 97% success in 29 Graaf type III

- 50% success in 12 Graaf type IV

 

Complications

 

1.  Failure to achieve reduction

 

Require closed +/- open reduction at 6/12

 

2.  AVN

 

Very low if safe ranges respected

 

Peled et al CORR 2008

- pavlik harness used in 78 Graaf 3 or 4 hips

- no AVN in any patient

 

3.  Femoral nerve palsy

 

Probably due to very chubby thighs / excessive flexion

- suspect in persistently crying baby

 

Murnaghan et al JBJS Am 2011

- review of 30 babies with femoral nerve palsy from Pavlik harness

- 87% presented in first week

- more likely in larger babies with more severe DDH

- femoral nerve palsy strongly predictive of treatment failure

 

Von Rosen Splint

 

Indications

 

Best suited to newborn

 

Disadvantages

 

Non dynamic

- hip must be reduced / reducible

 

Cannot ultrasound with splint on

- must be removed

 

Technique

 

Padded malleable aluminium splint

- 2 shoulder uprights

- 2 legs for thighs

- 2 wings for torso

 

Different sizes depending on weight

 

Hips in 60-90° flexion & 45° abduction (safe zone)

 

Parents don't adjust

 

Results

 

Wilkinson et al JBJS Br 2000

- suggested better outcomes than Pavlik harness

- fewer required operative reduction

 

Complications 

 

Skin irritation

 

AVN 2%

 

Treatment failure

 

 

 

 

Management 6 - 18 months

Two groups of dislocated hips

 

1.  Late presenters

2.  Failures of splint in those < age 6/12

 

Options

 

1.  Adductor tenotomy + closed reduction

- most surgeons will attempt this initially

- risk of AVN wilth forceful reduction / excessive abduction

 

2.  Open Reduction

- for failure of closed reduction

 

Timing

 

When safe for anaesthetic

- generally 6 - 12 months of age

 

Adductor Tenotomy / Closed Reduction

 

Timing

 

> 6 months

 

Issue

 

If irreducible or unstable / outside of safe zone need to proceed to open reduction

 

Technique

 

GA

 

Percutaneous adductor tenotomy

- assess range of maximum abduction

 

Closed reduction

- hip abducted / flexed

 

Ramsey's Safe Zone

- position between re-dislocation & maximum abduction 

- has to be at least 20°

- i.e. if have to position hip within 20o of maximum abduction to obtain reduction

- high risk of AVN

 

MUA / Arthrogram

- confirm reduction

- inject contrast

- nil medial pooling in abduction

- head reduced

 

Apply hip spica

 

Post operative

 

Hip Spica for 3/12

- change at 6/ 52 as child grows

- keep in safe zone / Abduction < 60o / Flexion 90o

 

Confirm reduction CT / MRI

- assess medial joint space

- < 5 mm = Good outcome

- > 7 mm = Poor outcome

 

Open Reduction

 

Concept

 

Rely on reduction of the head stimulating the acetabulum

 

Indications

 

1. Unstable hip

- have to abduct > safe zone to maintain reduction

 

2. Irreducible hip

 

3. Unacceptable widening of medial joint space

 

5 Blocks to reduction

 

1.  Capsule with hourglass constriction from psoas

2.  Labrum

3.  Thickened ligamentum teres

4.  Inverted inferior transverse ligament

5.  Pulvinar / thickened fat

 

Surgical Options

 

Medial Approach

Anterior Approach

 

Issues

 

> 1 year old

- consider adding osteotomy

- pelvic or femoral

 

Medial / Ludloff Approach

 

Advantage

- direct and simple access to blocks to reduction

- adductors / iliopsoas / inferior capsule / inferior transverse ligament

 

Disadvantage

- risk of AVN / injury to medial circumflex femoral

- can't perform capsulorraphy

- not suitable > 1 year as cannot combine with pelvic osteotomy

 

Approach

- superficial interval between longus and gracilis

- deep interval between brevis and magnus 

- anterior and posterior branches obturator nerve on brevis

- divide psoas (MCFA is medial to tendon)

- take circumflex vessels off capsule

 

Smith Petersen approach + / - FDRO

 

Advantage

- can perform capsulorraphy

- reduced risk AVN

- can perform pelvic ostetomy (Salter / Dega)

 

Disadvantage

- more difficult to access blocks to reduction

 

Approach

- vertical incision not very cosmetic

- use horizontal / bikini incision to reduce hip

- between sartorius and TFL to access blocks to reduction

- split apophysis if performing pelvic osteotomy

- separate lateral approach if adding FDRO

 

 

 

 

Management > 18 months

Dislocated Hip

 

Issue

 

Hip has been out for some time

- degree of acetabular dysplasia evident

- less time for remodelling

- increased instability if not addressed

 

Management

 

Open reduction + FDRO / Pelvic Osteotomy 

- usually perform pelvic osteotomy to correct acetabular dysplasia

- reserve FDRO for > 3 years / or if difficult reducing hip

 

Post operative

 

Hip spica for 3/12

- change at 6/52

- CT to confirm enlocated

 

Acetabuloplasty

 

Redirectional / Salter

 

From G. Sciatic notch anteriorly to between ASIS and AIIS

- gigli saw posteriorly

- oscillating saw anteriorly to guide cut

- use apophysis as bone graft

- covers acetabular defect anterolaterally

- stabilised with K wires

 

Disadvantages

 

1.  Provides anterolateral cover

- if combine with FDRO at same time can dislocate the head posteriorly

- i.e. is quite technically difficult to do both femur and acetabulum together

 

2.  Cannot do bilaterally as hinge on pubic symphysis

 

Reshaping / Pemberton or Dega

 

Pemberton

- transiliac into tri-iradiate

 

Dega

- incomplete transiliac

- relay on plasticity of acetabular roof

 

Periacetabular osteotomy

- osteotomy follows acetabular curve

- use curved osteotomes

- insert iliac crest bone graft

- fix with pins

 

Advantage

- simple

- can do bilaterally

 

> 3 years dislocated hip

 

Management

 

Open reduction + Pelvic Osteotomy + DVRO with shortening

- need to add DVRO to obtain reduction

 

Exception

 

> 4 yrs and bilateral

- don't treat

- shown to have poor results

- head is aspherical

- transform painless hips to painful

 

2 - 3 years Acetabular Dysplasia

 

Management

 

Hip enlocated / Acetabular index > 300 and not improving

- pelvic osteotomy

 

 

Surgical Techniques

Hip Arthrogram

 

Technique

- under II

- 10 mls contrast mixed with 10 mls saline

- prime the line

- spinal needle

- do not spill contrast over front of hip as will obstruct view

 

A. Lateral approach

- 45° angle with thigh

- insert just inferior & anterior to the greater trochanter

- advance 5-10 cm

- enter joint medially

 

B.  Anterior approach 

- preferred technique

- palpate femoral artery just below inguinal ligament

- femoral head is halfway between ASIS and pubic tubercle

- insert needle 2.5 cm below ASIS, aim to go behind NV bundle

- at 45° angle to skin

- aim for anterior neck

 

C.  Medial approach 

- MUA & arthrogram in young child

- excess extra-articular contrast will pool inferior & medial to joint so not to obstruct view of joint

- flex & abduct leg

- insert needle inferior to adductor longus tendon

- use II

- advance below femoral artery to head or neck

- generally inferior aspect of neck just below head

 

Open reduction via Smith-Peterson approach

 

Disadvantage

- damage LFCN

- stiffness (large dissection)

- access to inferior capsule and transverse ligament difficult

 

Advantage

- reduced risk of AVN

- can perform pelvic osteotomy through approach

 

Technique

 

Incision

- use bikini incision modification

- start 3cm below ASIS

- parallel to inguinal ligament 7cm

- longitudinal causes excess scarring, especially in young girl

 

Superficial dissection

- interval between sartorius & TFL

- LFCN retracted medially on top of sartorius

- ascending branch LCFA tied off in interval

- superior plane continued proximally by splitting apophysis 

- if needed to perform pelvic osteotomy

- peel glutei off llium to expose supra-acetabular region

 

Deep dissection

- between G medius and RF

- both heads rectus divided

- reflected head blends with capsule and superior rim acetabulum

- straight head to AIIS

 

Release

- psoas released under iliacus

- careful capsulotomy at acetabular edge

- remove capsule off lateral acetabulum

- to medial corner under direct vision

- any blocks to reduction removed

- excision of ligamentum teres

- transverse ligament division

- pulvinar removed

- evert inverted labrum 

 

Gentle reduction of head

 

Tight capsulorrhaphy performed 

- especially superiorly to remove lax capsule

 

Pelvic osteotomy

- if over 1 year of age with dysplasia

- redirectional or reshaping

 

Femoral osteotomy 

- performed if excessive traction at time of reduction

- avoid AVN

 

Spica cast applied

 

Medial Open Reduction

 

Disadvantage

- risk of AVN via MCFA damage

- only < 1 year as no access to periacetabular region

- unable to perform capsulorraphy

 

Advantage

- easy access to blocks to reduction

- especially psoas / inferior capsule

- reduced blood loss

 

Technique

 

Position

- supine

- hip abducted & flexed / frog legs

 

Landmarks 

- adductor longus & pubic tubercle

 

Incision 

- parallel to & 1cm below groin crease

- over adductor longus

 

Superficial dissection

- between gracilis / posterior & adductor longus / anterior

 

Deep dissection

- between adductor magnus & adductor brevis

- protect anterior branch obturator nerve on brevis

- protect posterior branch obturator nerve on magnus

- retract adductor brevis anteriorly

- will expose lesser tuberosity with psoas tendon inserting onto it

- isolate psoas tendon before division

 

Dangers

- protect MCFA as it passes between adductor brevis & psoas at LT

- runs around the medial side of psoas / anterior to psoas tendon

- fully isolate psoas before dividing

 

Release

- release psoas at LT

- T Capsulotomy

- divide transverse Lligament

- ligamentum teres can be used to pull head into acetabulum

- suture to anteromedial capsule

- may need to place radial splits in limbus 

 

Salter Osteotomy

 

Redirectional

- corrects anterior and superolateral acetabular deficiency

- less than 5 years but > 18/12

- mild - moderate dysplasia

- need concentric reduction

 

Technique

- Smith Peterson approach

- iliac apophysis split

- subperiosteal dissection to sciatic notch reflecting gluteals

- Gigli saw - osteotomy through GS notch

- emerge anteriorly between ASIS and AIIS

- acetabulum rotated anteriorly and laterally

- triangular graft from crest

- K wire fixation

 

Complication

- 25% leg lengthening

 

Periacetabular osteotomy

 

Dega 

- leaves posterior wall intact

- relies on plasticity of superior acetabulum

 

Pemberton

- osteotomy includes posterior column

- bend through tri-radiate cartilage

 

Smith - Petersen approach

- split apophysis

- curved osteotome

- parallel superior dome of acetabulum

- 15 mm above

- can leave posterior column intact

- bone graft +/- K wires

 

Femoral Derotaton and Varising Osteotomy

 

Technique Antero-lateral 

 

Open reduction as above

 

Separate lateral approach

- elevate vas lateralis

- 2 x K wires centrally in neck, short of physis

- check II

- open and protect periosteum with homans

- mark distal and proximal femur with drill holes to check rotation later

- osteotomy with microsagittal saw 1 cm below LT

- don't need to resect wedge with Synthes offset locking plate

- may need to shorten

- decrease anteversion by IR distal fragment

- check range of ER of hip / should be reduced

 

Osteotomy fixed Synthes Offset Locking Plate

- 125o for some varus

 

 

 

 

 

PFFD

Definition

 

Proximal Focal Femoral Deficiency

- congenital malformation

- characterised by failure of normal development of the proximal femur

 

Epidemiology

 

Rare

- 1 in 50 000

 

Bilateral in 15% 

- usually more severe

 

Association

 

Fibular hemimelia in 2/3

ACL deficient

 

May also be associated with

- CTEV

- Cleft palate

- CDH

- Congenital spinal deformity

- Dysraphism

 

Aitken's Classification

 

Class A

- short femoral shaft with coxa vara

- head of femur present, neck may be absent early

- adequate acetabulum

 

- bony connection between head & neck present at maturity

- may be pseudarthrosis at point of connection

 

Class B

- pseudoarthrosis between head and shaft

- shorter femoral shaft with small bony tuft on proximal end

- head of femur present

- adequate acetabulum

 

 

Class C

- no femoral head

- acetabulum severe dysplastic

- very short femoral shaft with bony tuft proximally

 

Class D

- femoral head & acetabulum completely absent

- femur consists of condyles only

 

Signs

 

Child with very short leg

- large shoe raise

- LLD above GT

 

DDx of shortening above GT

- DDH 

- PFFD

- Tom Smith arthritis / post septic arthritis

- Tumour

 

Also

- short involved thigh segment

- characteristic posture (Flex / Abducted / ER hip)

- Ship's Funnel Sign 

 

Bulky upper thigh segment

- sharply tapering to knee

 

Issues

 

1.  Pseudoarthrosis

2.  Instability of the hips

3.  Coxa vara

4.  LLD

5.  Inadequate lateral femoral condyle

6.  Inadequate proximal musculature

7.  Malrotation

 

Management 

 

Class A & B

 

Femoral heads present

 

1.  Stabilize pseudarthrosis with IM Rod

2.  Hip stabilisation 

- pelvic osteotomy

- femoral osteotomy

3.  Limb lengthening

 

Class A

 

1.  Realign Coxa Vara

- subtrochanteric osteotomy

 

2.  Equalise LLD

- lengthen femur or

- shorten / epiphysiodesis other femur

 

Note:

- if lengthen femur need to watch knee doesn't dislocate 

- ACL deficient

- may have to bridge knee with external fixator

 

Class B

 

1.  Fuse pseudarthrosis 

 

2.  Limb lengthen or amputate as required

 

Class C & D

 

No femoral heads

 

Stabilise hip

- pelvic support osteotomy

- osteotomy proximal femur

- supported by ischium

- distal osteotomy for mechanical axis

- need lengthening as well

 

Foot above contralateral knee

- knee fusion + symes

- essentially AKA

 

Foot below contralateral knee

- rotate then fuse knee / Van Ness Rotational Osteotomy

- ankle acts as knee joint / essentially BKA

- BV become anterior

- require good functioning foot

 

Bilateral

 

Usually Class C or D

- can usually walk well without prosthesis

- surgical procedures usually detract from mobility

- should not be treated surgically unless such severe foot deformity that cannot ambulate without prosthesis / Symes

 

 

 

 

 

 

Perthes

Aetiology & Classification

Definition

 

Idiopathic condition of the immature femoral capital epiphysis involving varying degrees of osteonecrosis

 

AKA

- Legg-Calve-Perthes disease

- coxa plana

 

Described in separate publications in 1912 by

- Legg (Boston)

- Calve (France)

- Perthes (Germany)

 

Epidemiology

 

M: F    4:1

 

1 in 740 males

 

Onset usually at age 4-8

- early as 2 to late as 12

 

Children small and thin & hyperactive with delayed bone age

 

Bilateral - between 8 - 24%

- will not be symmetric

- if symmetric think other causes

 

Risk Factors

 

Sex : M: F 4:1

Geographic Location - 1/1200 US; 1/12 000 UK ; More common in urban areas

Social Class - Higher incidence in lower socio-economic classes

Race - Caucasian > Asian > African (extremely rare)

Perinatal Factors - Maternal Smoking ; Breech ; Prematurity/ Low Birth Weight ; Older Parental Age

 

Aetiology

 

Uncertain

 

Multiple ischemic events causing AVN

- ? lateral epiphyseal blood vessel occlusion

- sole blood supply 4-8 years

 

Theories

 

1.  Microtrauma to retinacular vessels

 

2.  Increased synovial pressure 

- e.g. transient synovitis

 

3.  Venous HTN 2o to thrombotic occlusion

- hyperthrombotic state

- hypofibrinolysis 

 

Glueck 1996 JBJS

- 75% coagulation abnormality  

- decreased Protein C ± S / increased lipoprotein A

- this has never been a found association in any study since

 

Blood supply immature femoral head

 

Medial and lateral circumflex femoral 

- cruciate and trochanteric anastomoses

- extracapsular ring at base of neck

- retinacular vessels run in capsule

- intracapsular ring at base of head

- lateral epiphyseal artery most important

 

Ligamentum teres

- artery viable up to age 2

 

Associations

 

Short stature & delayed maturation 

 

Attention Deficit Hyperactivity Disorder / ADHD

 

Trauma

- parents often relate onset of symptoms to traumatic event 

- this association not clearly established

 

Irritable hip ~ 1%

 

Pathology

 

Head

 

Various areas of necrosis / revascularisation

- reossification from periphery

- typically anterior & lateral

 

Ossified epiphysis small

- stops growing

- cartilage keep growing with blood from synovium

- increased head to teardrop appearance but head does not subluxe

 

Head deformity secondary to collapse

- biological plasticity during creeping substitution

- head subjected to uneven & excess forces

- flattening or saddle deformity 

- anterolateral extrusion of cartilage

 

Coxa Magna 

- flattening of head with lateral extrusion of physis + periosteal new bone

 

Physis

 

Avascular

- growth arrest

- disordered cartilage columns in physis

 

Coxa Breva

- physeal arrest with short neck & long GT 

- LLD & Trendelenberg gait

 

Classification

 

Chronological

- Waldenstrom

 

Extent of femoral head involvement 

- Catteral

- Salter-Thompson

- Herring

 

End-result

- Mose 

- Stuhlberg

 

Waldenstrom Stages

 

History : Swedish Surgeon ; Described disease 1910 prior to being named by Legg-Calve-Perthe in 1912

 

1. Initial

 

Duration : 3- 6 months

 

Xray

- may be normal

- small, sclerotic epiphysis

- joint space widening

- increased density of ossific nucleus & cessation of growth

 

2. Resorption / fragmentation

 

Duration : 6/12

 

Pathologic Process

- necrotic bone irregularly resorbed / creeping substitution

- replaced with vascular fibrous tissue

- may be associated with collapse

 

XRay - Fragmentation of Physis

 

3.  Reossification

 

Duration : 1.5 - 3 years

 

Pathologic Process

- starts at margins & progresses centrally

- eventually new areas coalesce & epiphysis regains normal strength & density

 

XRay

- Better defined shape

- Return of bone density

 

4. Remodelling

 

Duration : Occurs until skeletal maturity

 

Xray - May have flattening of the head and neck

 

Herring Lateral Pillar

 

AP xray when disease in fragmentation / divide femoral head into 3 pillars

- lateral (25%)

- medial (25%)

- central (50%)

 

Group A

- no lateral pillar collapse or radiolucency

- all become Stuhlberg 1 & 2

 

Group B

- > 50% of lateral pillar height maintained

- outcome depends on age

- < age 9 almost all become Stuhlberg I and II

- > age 9 30% become Stuhlberg II / 70% become Stuhlberg III or IV

 

Group C

- pillar < 50% normal height

- majority do poorly

 

Catterall

 

Technique

- uses AP & lateral xray

- 4 groups

- significant inter & intra-observer error

 

Group I

- < 25% involved

- anterocentral head

 

Group II

- 25-50% head

- anterolateral region / lateral column intact

 

Group III

- 50-75% head involvement

- moderate collapse 

 

Group IV

- 100% of head sequestered

- severe collapse

 

Salter-Thompson

 

Group A

- Catterall 1 and 2

- < 1/2 of head involved

- viable lateral pillar 

- good prognosis

 

Group B

- Catterall 3 and 4

- > 1/2 head involved, loss of lateral pillar

- poorer prognosis

 

Mose

 

Quantifies degree of sphericity

- graded according to variance from perfect circle in either AP or lat

- many authors do not agree that Mose sphericity is accurate predictor of long term outcome

- good or fair Mose rating = good result

- poor Mose rating not necessarily = poor result

 

Good - no deviation on the 2 views 

Fair - up to 2 mm variance 

Poor - 3 mm or more variance 

 

Stuhlberg

 

Based on congruency & sphericity

 

Class 1 

- normal 

- 0 % OA

 

Class II:  Spherical & Congruent

- femoral head spherical (mose circles overlaid on AP / lateral of femoral head show <2mm variation)

- At least 2 other abnormal features including Coxa Magna , Coxa Breva or Abnormally Steep Acetabulum

- 15% OA

 

Class III:  Aspherical Congruity

- head ovoid (mushroom or umbrella shaped) and aspherical i.e Mose circles show > 2mm variation  

- 60% OA in middle age

 

Class IV: Aspherical Congruous Incongruity

- head flat, steep acetabulum

- 75% OA usually in middle age

 

Perthes Stuhlberg Class IVPerthes Stuhlberg Class IV lateral

 

Class V:  Aspherical Incongruous Incongruity

- head flat, normal acetabulum

- 80% OA at young age before 50

 

Natural History

 

Osteoarthritis 10x more prevalent than general population

 

Only 40% have normal radiographs

 

20-40 years after onset of symptoms

- 80% active & pain-free

- only 10% have THR

 

At 50 years after onset of symptoms

- 50% have disabling pain

- 40% have THR

 

Weinstein

- probably best you can say is function deteriorates with time

- 50% THR at 50 years

 

Prognostic Factors

 

1. Age of onset

- most important

- < 6 good Stulberg I-II

- 6-9 variable Stulberg I-IV

- > 9  Stulberg III & IV

- related to amount skeletal growth & thus remodeling available

 

2. Sex

- F worse than M

- ? early maturation, less time to remodel

 

3. Extent head involvement

- quantified by Caterall / Salter-Thompson / Herring classification

 

4. Containment of head

- extrusion and asymmetric growth leads to worse prognosis

 

5. ROM

- hip irritability causes adductor tightness

- loss of abduction ROM prevents remodeling head by acetabulum

 

7. Lateral Pillar Collapse

 

Catterall "Head at Risk" Signs

 

Only lateral subluxation shown to be prognostic 

 

5 Xray

 

1. Lateral subluxation head

2. Lateral epiphyseal calcification

3. Gage Sign - V shaped defect in lateral physis

4. Horizontal physis angle

5. Metaphyseal cysts

 

4 Clinical

 

1. Progressive loss ROM

2. Adduction contracture

3. Flexion with abduction

4. Obesity

 

Gage's sign

- lateral bulge of metaphysis

- extruded cartilage may calcify lateral to acetabulum 

- gives appearance of head extrusion

- coxa breva

 

Symptoms

 

Most present early 

- 75% during necrotic / fragmentation stages

- insidious painless limp commonest

- ache groin / anterior thigh or knee

- exacerbated by activity

 

Examination

 

Short stature 

- usually short with delayed bone age

 

Antalgic / Trendelenberg gait

 

Decreased ROM

 

Early

- synovitis with tight adductor and/or psoas

- loss abduction in extension and flexion

- loss IR / flex into ER

 

Late

- bony impingement

- "hinge subluxation" 

- lateral extrusion, flattening & enlargement of head prevent normal motion

 

LLD usually small

- apparent LLD from adduction contracture

 

Occasionally thigh / gluteal wasting in advanced cases

 

Xray

 

Grade stage of Perthes

Lateral Pillar classification

 

US

 

Nonspecific findings of synovitis usually found

- thickening synovial membrane vs. synovitis

- synovial effusion

 

CT

 

Perthes CT

 

MRI

 

Advantage

- can assess amount of cartilaginous head outside of acetabulum

- very good way of assessing containment of cartilage head

 

DDx

 

AVN

MED / SED

Hypothyroidism

 

MED

- bilateral and symmetrical

- acetabular involvement

- no metaphyseal cysts

- other physeal involvement therefore consider skeletal survey in those with bilateral "perthes"

 

MED

 

Complications of Perthes

 

1. LLD 25% 

 

Causes

- subchondral fracture & collapse 

- arrest proximal femoral epiphysis

- adductor contracture

- varising osteotomy

 

2. Trendelenburg gait

 

Proximal greater trochanter

- collapse of epiphysis / coxa breva

- varus osteotomy

 

Management

- growth arrest of greater trochanter at 10 years or distal transfer

- femoral neck lengthening osteotomy

 

3. Hinge abduction

 

Extrusion of epiphysis laterally

 

Management

- treat with cheilectomy once physis closed

- has poor reputation 

- may worsen arthrosis by exposing fresh bone

- if done prior to physeal closure may cause SUFE or growth arrest

 

4. Labral tears 

 

Rare

- abnormal stress by head on lateral acetabulum

 

5. Secondary OA

Management

Aim

 

Prevent head deformity & secondary OA

- interfere as little as possible with child's development

 

Goals

 

1. Restore & maintain ROM

- allow abduction which covers anterolateral extruded head 

- important range is abduction in extension

- aim 30o abduction or 75% of contralateral side 

- can consider adductor tenotomy

 

2. Head containment i.e >80 % coverage of the femoral head

 

3. Relief of symptoms

 

Options

 

1. Observe

- normal ROM

 

2. Symptomatic treatment

- physio for decreased abduction

 

3. Definitive early treatment

- Herring B & B/C > 8 years old

- femoral varising osteotomy / containment

 

4. Late surgical treatment of deformity / degeneration

- Shelf osteotomy

- Valgus osteotomy

- GT osteotomy

 

Containment 

 

Concept ~ Petrie

- location of capital femoral epiphysis within acetabulum

 

Mechanism

- molding action

- subjects head to molding action of acetabulum

- leads to more spherical head / more congruous joint 

- if the head remains in the acetabulum it usually remains spherical

- subluxed head associated with deformity

 

Herring et al JBJS Am 2004

 

Methods

- 337 patients with 345 affected hips in a prospective multicenter study

- all patients were between 6.0 and 12.0 years of age at the onset of the disease

- none had had prior treatment

- followed up until skeletal maturity

- no treatment / brace treatment / range-of-motion exercises / femoral osteotomy / innominate osteotomy

 

Results

- no differences in outcome in hips with no treatment / bracing / range-of-motion therapy

- no differences between the hips treated with a femoral varus osteotomy / innominate osteotomy

- no effect on children with a chronologic age of 8.0 years or less / skeletal age of 6.0 years or less at the onset of the disease

- > 8 at time of disease onset with lateral pillar B group and B/C border group did better with operative

- 8.0 years old or less at the onset of the disease in lateral pillar group B did equally well with nonoperative and operative treatment

- hips in lateral pillar group C had the least favorable outcomes with no differences between the operative and nonoperative groups

- female patients > 8 at onset did significantly worse than male patients

 

Summary

 

Age onset < 8 (chronological ; skeletal age 6) - no treatment

Ages onset > 8 and B or BC - containment surgery

Class C - salvage / Shelf

 

Varsing osteotomy as good as Salter

 

Non-operative Containment

 

Bracing

 

Concept

- placing hip in abduction whilst allowing hip motion & ambulation

- worn until reossification phase of lateral epiphysis 

- usually 6-18 / 12 

- no evidence for effect

 

Petrie Abduction Casts 

- two long leg casts with abduction bar

- 30o abduction & 5o IR

 

Scottish Rite Brace

- two thigh cuffs with abduction bar

 

Physio

 

Concept

- if can maintain good abduction, then no need for varising ostetomy

- see child every 6 weeks to 3 months

- ensure no adduction contracture

- hydrotherapy

 

Operative Containment

 

Advantage

 

Permanent containment enhances remodeling

 

Disadvantage

 

Risks of surgery

 

Options

 

Femoral varising osteotomy

Acetabular osteotomy

 

Indications

 

> 8 at onset and B or B/C

No hinge abduction

 

Hinge abduction

 

Concept

- abducted hip does not obtain full coverage of cartilage / full containment

- hinging on abucted portion of femoral head

- a varising osteotomy with increase point loading and worsen symtoms

 

Diagnosis

1.  MRI with hip in abduction

2.  MUA pre varising osteotomy

- hinging / rose thorn appearance

- medial pooling

- i.e. whole cartilaginous head not entering acetabulum

 

Treatment

1.   Trial adductor tenotomy / physio +++

- if hinge abduction resolves can proceed to operative intervention

2.  Valgus osteotomy

- salvage

 

Lloyd-Roberts Varising Femoral Osteotomy

 

Concept

- seat head deep in acetabulum

- remove vulnerable anterolateral portion from acetabular edge

- decrease JRF on head

 

Aim

- sufficient varus to permit entire ossified epiphysis to be covered by ossified acetabulum

- derotation can be added to reduce anteversion and increases cover

 

Indications

- Herring grade B or B/C

- > 8 years at age on onset

- must be able to obtain full containment of cartilaginous head

 

Technique

 

Patient supine on radiolucent table

- preop antibiotics

- frog leg laterals when needed

 

Lateral approach

- elevate vastus lateralis

- incise and reflect periosteum, then protect with homanns before osteotomy

- "be good to the periosteum and it will be good to you"

- drill holes in femur proximal and distal to osteotomy site in line with each other

- allows guide to rotation after osteotomy

- place cannulated wires into femoral head now

- leave short of physis, check AP and lateral

- subtrochanteric osteotomy with saw / osteotome

- ER distal fragment (no more than 15o)

- apply Synthes proximal locking plate

- has offset otherwise have to step cut femur

- multiple angles available

- aim for 115o of varus

- avoid excessive varus < 100o to keep GT distal to femoral neck

 

Advantage

- operates on affected side of joint

- less demanding than acetabular surgery

- results equivocal

 

Disadvantage

- LLD increased

- coxa vara

- trendelenberg gait / may require GT fusion or advancement in future

- have to remove metal in future with risk refracture

- potential difficulty of THR in future / insertion of femoral stem

 

Salter Innominate Osteotomy

 

Concept

- redirects acetabulum to provide coverage for anterolateral head

 

Pre-requisites

- full or almost full ROM 

- reasonable congruence on abduction / X-ray in full abduction confirms containment

- fragmentation stage

 

Advantages

- avoids shortening, coxa vara

- nil trendelenberg

 

Disadvantage

- more difficult

- lengthens leg

- operate on normal side of joint

- increase joint pressure

 

Late Salvage 

 

Indications

- failure of containment techniques

- significant femoral head deformity with hinge abduction

 

Options

 

1. Shelf Arthroplasty

- laterally subluxed femoral head in older child

- allows some continued remodeling of the femoral head

 

2.  Cheilectomy femoral head

- wait till physis closed

- extruded rim of bone & cartilage excised

- poor results

 

3.  Valgus Osteotomy

- hinge abduction > 12 years

- redirect normal portion of head to acetabulum

 

4.   GT osteotomy

- distal & lateral transfer of GT 

- aim to decrease JRF

- may relieve trendelenberg gait

 

 

 

SUFE

Background & Management

Definition

 

Displacement of proximal femoral epiphysis in the immature hip

- due to imbalance of mechanical and endocrine factors 

 

Epidemiology

 

Age Peak Incidence : M 12-14; F 11-13; Slight downward trend due to earlier maturation of children

L hip > R

10 / 100 000

 

Bilateral SUFE

 

No endocrine abnormality

- 20% at time of of diagnosis

- another 20% during diagnosis

- up to 60% with long term follow up

 

Endocrine abnormality

- up to 75%

 

Risk Factors

 

Demographic Factors

- Increased Weight and Height (50% over 95th percentile weight; 50% over 97th percentile height); Average BMI 27  

- Race : Increased risk in Black pts (4X) ; Polynesian ; Hispanics

- Sex : Male 2.5 x risk

- Family History - 7 % risk to other family members

 

Hip Mechanical Factors - increased shear forces

- increased slope (at adolescence growth plate goes from horizontal to vertical position)

- increased retroversion Southwick angle > 14

- reduced neck shaft angle

 

Endocrinopathies

Imbalance of

- Testosterone - causes physeal fusion

- Growth hormone - causes physeal hypertrophy / weakens

 

Causes

- hypothyroidism

- hypopituitary

- acromegaly or growth hormone supplementation

- CRF/ Renal Osteodystrophy

 

Other

Connective Tissue Disorders - Marfan's / Downs / Ehlos Danlos

Chemo therapy / DXRT

 

Pathology

 

Widened hypertrophic zone 

- constitutes 60% of physeal width 

 

Abnormality at Hypertrophic & Proliferative Zones junction

- failure occurs here

- disordered chondrocyte columns

- decreased number of cells

- cells smaller

- increased number of dead and degenerative cells

 

Head remains in acetabulum via L. Teres

- neck displaces anterosuperior on physis and ER

- head slips posterior / inferior on neck

 

Classification

 

Chronological

 

Acute < 3/52 symptoms

Acute on Chronic 

Chronic >3/52

 

Morphological 

 

Southwick Slip angle

- Wayne Southwick ; 1st Chairmen of Orthopaedics at Yale University

- lateral X-ray  / frogs legs

- epiphyseal-shaft angle 

- angle of interest is the angle of the affected side subtracted from the normal contralateral side ; if contralateral SUFE then 12 degres as normal    

- <30° / 30- 50 / >50°

- mild / moderate / severe

 

Stability

 

Loder JBJS 1993

 

Stable 

- able to weight bear / 0% AVN

 

Unstable 

- unable to weight bear / AVN rates 10 - 60%

 

History

 

Overweight adolescent boy hip or knee pain; 30% present only with knee pain

 

Examination

 

Limps

Walk with ER (chronic) i.e increase in foot progression angle

Obligate abduction and external rotation with flexion

Limitation IR / Abduction

LLD (real and apparent)

 

X-ray

 

AP

 

SUFE AP

 

Trethowan Line / Kleins Line

- line along superior neck usually transects 20% head

- originally described as AP but can also be used as lateral

 

Widened physis

 

Inferomedial remodelling in chronic slip

 

Metaphyseal Blanch Sign of Steel

- Increased cresenteric density in the metaphysis due to overlapping of the metaphysis with the epiphysis

 

Capener's Sign

- the posterior acetabular margin normally cuts the medial corner of the metaphysis

- in a slip the whole of the metaphysis remains lateral to the acetabular margin

 

Frog Leg Laterals / Shoot through lateral

 

SUFE Lateral

 

Shoot through lateral

- best to avoid frog leg lateral as may displace slip

 

Posteriorly displaced & angulated

 

Measure Southwick Angle

- calculate severity of slip

- also estimates risk of slip of other side / looking for retroversion

 

Management

 

Aims

 

1.  Prevent further slip / obtain physis fusion

- 30% will continue untreated

 

2   Prevent deformity and OA

- MUA / ORIF / osteotomy

 

3.  Avoid complications 

 

Algorithm

 

Loder RT et al. What is the best evidence for treatment of slipped capital femoral epiphysis?

Journal of Pediatric Orthopaedics. 2012 Sept : 32 (Suppl 2) S158 - 65

 

Stable : Insitu Pinning current gold standard

 

Unstable : Two schools of thought

  1) < 24 hours old

- Consider treatment as a surgical emergency

- urgent reduction (gentle traction, flexion and internal rotation) +/- joint decompression

- probably results in lowest AVN rates (Petersen et al - refer below)

- alternatively consider discussion with tertiary paediatric referral centre

 

  2) > 24 hours

- Discuss with specialist paediatric centres

- possibility of surgical dislocation and realignment i.e. Modified Dunn Procedure

 

In Situ Pinning

 

Gold Standard (Techique refer below)

 

CT post operatively

- ensure no screw protrusion

 

TWB 6/52

 

Serial Xray

- ensure epiphysis doesn't grow off screw

- screw can break / can lose position

- observe til physis closes

- no indication to remove pin

 

Results

 

Weinstein et al JBJS Am 1991

- 40 year follow up of 155 hips

- some pinned in situ / some realigned / some reduced

- rates of OA / AVN / chondrolysis increased with severity of slip

- rates of OA / AVN / chondrolysis increased with reduction / realigned

- regardless of severity of slip, pinning in situ had best results with lowest complications

 

Closed Reduction Prior to Pinning

 

Disadvantage

 

Traditionally associated with higher risk AVN

 

Advantage

 

Theorectical

- may decrease AVN in severe, unstable hips

- prevent severe deformity / late OA

 

Indication

 

Acute & unstable < 24 hours

 

Results

 

Peterson et al J Paediatr Orthop 1997

- 91 cases of severe, acute, unstable slips

- 42 closed reduction < 24hrs AVN 7%

- 49 closed reduction > 24hrs AVN 20%

- hypothesised that had acute obstruction of epiphyseal vessels 

- timely decompression allows revascularisation

- treat an acute unstable slip as per a fracture

- these have up to 50% AVN rate anyway

- emergency operation

 

Chen et al J Paediatr Orthop 2009

- 30 acute, unstable slips

- 25 closed reductions and 5 open reductions with release hematoma

- 4 cases of AVN

 

Open Reduction Prior to Pinning

 

Indications

- severely displaced slips

 

Reasoning

- moderate or severe slips do poorly in long term

- best treatment is intra-capsular reduction or osteotomy

- risk AVN either way

- acute open reduction easier

- also decompress hip

 

Modified Dunn Procedure

 

Ziebarth et al. Capital realignment for moderate and severe SCFE using a modified dunn approach.

Clin Orthop Relat Res. 2009; 467(3): 704 - 16

- Ganz type transtrochanteric approach

- Z shaped capsulotomy to preserve superior vessel along neck, along anterior acetabulum and inferior neck

- capsule banana skinned off neck

- hip dislocated via adduction and external rotation and transection of the round ligament

- epiphysis taken off neck, still attached to capsule

- intraoperative monitoring of epiphyseal perfusion via 2mm hole drilled in the anterior neck or via insertion of ICP monitor into the epiphysis

- neck debrided to avoid tensioning of posterior vessels

- head replaced and pinned as per normal

 

Results

 

Sankar WN et al. The modified Dunn procedure for unstable slipped capital femoral epiphysis: a multicentre prospective.

JBJS (Am). 2013; 95:585- 91.

- 26% Rate of AVN

- 15% Revision of metalwork rates

- therefore capable of restoring anatomy but ongoing risk of AVN and metalwork complications.

 

Prophylactic Pinning

 

Issues

 

Can justify but may cause complications 

- i.e. chondrolysis, subtrochanteric fracture secondary to screw

 

Incidence bilateral slips

- unknown

- may be > 35%

- high incidence of asymptomatic and mild contralateral slips

 

Major indications

- young i.e < 10 years

- unreliable parents

- geographic isolation

- Secondary SUFE eg endocrinopathy

 

Technique of Pin in Situ

 

Set up

 

1.  Supine on radiolucent table

- very easy to set up

- much faster if pinning both sides / reduced set up

- theoretical risk of displacing slip / inadvertant manipulation

- lateral by flexing and full ER of hip / frog legs

 

2.  Traction Table

- easy to get AP and lateral

- need 2 set ups for bilateral pinning

- takes longer in this regard

 

Technique

 

Draw anterior and lateral lines

- get AP, draw line mark using radiopague ruler to centre of head

- get lateral, repeat

- intersection of points is incision site

 

Stab incision

- guide wire percutaneously to neck

- more anterior entry point on femoral neck required the more the epiphysis is displaced posteriorly

- more anterior entry point ensures less likely to start at a subtrochanteric position and risk stress fracture

- central in head on both views

- ensure don't penetrate head

- cannulated drill

- 6.5/ 7.0/ 7.3 screws

- 8-10 mm or 4-5 threads across physis

- do far and away screening / approach withdraw; circumferential screening

- this ensures screw is not in joint

 

Complications

Chondrolysis

 

Definition

- loss of 50% joint space or < 3mm joint space

 

Epidemiology

- female 2:1

- 10-20% of SUFE 

 

Causes

 

1.  Head penetration

- use only 1 screw

 

2.  Immunologically mediated global loss of articular cartilage

- can occur in no treatment of SUFE

 

Clinical

- painful and reduced ROM

- flexion / adduction contracture

 

Management

- rest / ROM exercises / NSAID

- ilizarov distraction - unknown long term results

- 50% resolve

- very difficult problem - may need THR / arthrodesis

 

AVN

 

Incidence

- 5 % in chronic slips

- 20% in acute on chronic slip  

- up to 50% in unstable slip

- much worse if MUA

 

Risk factors

 

1. Reduction 

2. Posterosuperior quadrant pin placement

3. > 1 pin 

4. Severity slip 

- ? results skewed by increased MUA in this group

5. Unstable SUFE - unable to weight bear / Loder

 

Salvage options

 

1.  Hip distractor

2.  Valgus osteotomy

- redirect WB area away from AVN

 

Subtrochanteric Fracture

 

Cause

- too low screw entry

- multiple drill holes to get correct entry

 

Management

- > 15 reconstruction nail

- < 15 DHS or DCS

 

Further physeal slipping

 

Cause

- poor screw positioning

- young patient

- failure physeal fusion

- removal of screw before fusion

 

Cam FAI

Principle

Epiphysis heals in a posterior and inferior position = abnormal contact of relatively anterior neck on the acetabulum

 

Incidence

- common with moderate - severe nonrealigned

 

Options

1) Joint Preserving

 - Arthroscopic - Osteochondroplasty

 - Open - Osteochondroplasty with open surgical dislocation +/- Dunn Osteotomy at the level of the femoral neck  

 

2) Joint Sacrificing - THR for advanced cases

 

OA

 

Natural History

 

Not completely known

- still occurs in uncomplicated treatment SUFE 

- ? 10% Adult OA

- even unknown if pistol grip deformity is from SUFE

 

Weinstein

- mild slip 36% no degenerative changes

- moderate or severe slip all had OA

Osteotomy

Deformity

 

Varus / extension / external rotation

 

Options

 

Intertrochanteric

Base of Neck

Subcapital

 

Osteotomy

 

Valgus / flexion / internal rotation

 

Intertrochanteric / Southwick 

 

Technique

- biplanar

- valgising / flexion / internal rotation 

- removal anterolateral bone wedge below LT

 

Advantage

- no AVN / safest

- improves hip motion

 

Disadvantage

- worsens LLD

- difficult THR

 

Base of neck / Kramer

 

Technique

- closing wedge anterior and superior

 

Disadvantage

- risk AVN

- shortens neck further - worsens LLD

 

Intra-capsular / Subcapital

 

Types

 

Dunn 

- trapezoid osteotomy

 

Fish 

- cuneiform

 

Advantage

- anatomical correction

 

Disadvantage

- risk AVN

 

 

 

 

 

Septic Arthritis

Clinically

 

Unwell / irritable

Fever

Not feeding

Unable to weight bear / limp

Knee pain

 

Examination

 

Fever

Limited / painful ROM

Hip held flexed and externally rotated

- maximises volume of capsule

 

Lab tests

 

WCC > 12 000

ESR > 40

CRP > 10

Blood cultures

 

Joint Aspiration

- Innoculate directly into culture bottles to increase yield of fastidious organisms eg Kingella

- WCC >50 with >80% PMN suggestive

 

Xray

 

May demonstate hip subluxation

 

Ultrasound

 

Confirms hip effusion

 

MRI

 

Can be useful

- identifies proximal femoral osteomyelitis

 

Issue

- requires GA usually

 

Kocher Criteria

 

Kocher MS et al. Differentiating between septic arthritis and transient synovitis of the hip in children : an evidenced-based clinical prediction algorithim. JBJS (Am). 81 (12): 1662 - 70.

- Criteria :

   ESR > 40

   WCC > 12

   Fever >38.5

   Inability to weight-bear

- Risk of Septic Arthritis - 0/4 - .2% ; 1/4 3%; 2/4 40%; 3/4 93% ; 4/4 99%

 

Pathology

 

Staph aureus most common; MRSA increasingly common

Group B Strep

Kingella Kingae (fastidious organism; increasingly common in < 3 years)  

Gonococcus (Sexually active adolescents)

 

Operative Management

 

Indications

- raised inflammatory markers

- fever

- ultrasound with effusion

 

Technique

 

No antibiotics unless positive blood culture

 

Smith Petersen approach

- remove window of capsule

- washout +++

- don't close capsule

- leave in drain

 

Drill proximal femoral metaphysis / femoral neck

- diagnose / decompress osteomyelitis

 

MUA

- ensure hip is stable

- may require posteroperative spica if unstable

 

Post operative

 

Antibiotics

- broad spectrum initially

- targetted antibiotics with positive culture

- IV until child well and inflammatory markers normalised

- oral until 6 weeks

 

TWB 6/52

 

Xray follow up minimum 2 years

- AVN

- chondrolysis

- physeal arrest

- subluxation / dislocation

 

 

Transient Synovitis

Definition

 

Transient synovitis of unknown aetiology with complete resolution

 

Epidemiology

 

Most common cause of hip pain

- 1 /1000

 

May occur in 3% of children with majority not seeking medical attention

 

Wide range of age

- from 9 / 12 to teenagers

- usually 3 - 8 years

- peak 6 years / same as Perthes

 

More common in

- boys 2:1

- caucasian

- never bilateral

 

Aetiology

 

1/3 viral 1/3 trauma 1/3 unknown

 

1. Infection

 

? Infectious condition

- supported by frequent association with current or antecedal illness

- viral or bacterial

- most commonly ENT

- no specific viral antibodies found

 

2. Trauma

 

May be contusion of hip

- history of trauma in 30%

 

3. Allergic hypersensitivity

 

May be hypersensitivity response

- 25% have atopia

- dramatic response to antihistamines & steroids

 

Pathology

 

Culture negative synovial effusion

 

Synovial hypertrophy 2° to non-pyogenic inflammatory reaction

 

NHx 

 

Limited duration of symptoms

- average 10 days

- may be 8 weeks

 

Recurrence uncommon < 10%

 

Associated with Perthes Disease in 1.5%

- probably not causative

 

Clinical

 

Acute onset of unilateral hip pain

- usually hip & groin

- may be thigh & knee

 

Findings

 

Limp

Inability to weight bear

Hip held in flexion & ER

Protective muscle spasm

Restricted ROM especially IR

May be low-grade fever < 38°

 

Blood Tests

 

May be mild elevation of 

- WCC

- ESR

- CRP

 

Xray

 

Usually normal

- exclude other conditions

 

May be mild changes in hip

- coxa magna / femoral neck widening

- ? 2° to hypervascularisation

- not symptomatic

 

US

 

May show effusion

 

Differential Dx

 

Septic Arthritis

- see Miscellaneous / Infection / Septic Arthritis

- Kocher criteria (WB / ESR / fever / WCC)

Osteomyelitis

 

Perthes / SUFE / JRA 

 

Management

 

Important to rule out other pathology

 

Symptomatic only

- rest in bed and analgesia until FROM shown to decrease recovery time & recurrence

- traction only for severe cases that fail to settle

- NSAID

- PWB on crutches until limp resolves

 

 

Knee

ACL Avulsion Fracture

Myers & McKeever classification

 

Type I:   Undisplaced avulsion fracture

 

Type II:  Anterior portion displaced & hinged

 

ACL Avulsion Type 2

 

Type IIIA:   Displaced

Type IIIB:   Displaced & Rotated

 

Type 3 ACL Avulsion

 

Management

 

I - III A 

 

Attempt closed reduction

- anaesthesia / II control

- long leg cast in full extension

- trap fragment under condyles

 

Block to reduction

- usually medial meniscus / intermeniscal ligament

 

ACL avulsion reduced in extension

 

IIIB & Failed MUA

 

1.  Medial arthrotomy / arthroscopically

- need to remove portion of fat pad for visualisation

- intermeniscal ligament usually blocks reduction

- removed callus / hematoma

- replace fragment under intermeniscal ligament

- want to impact fragment to tension stretched ACL

 

2.  Repair Options

 

A.  No 2 suture

- bunnell suture through ACL above bony fragment

- pass 2 x limbs through bony fragment

- insert beath pins to retrieve suture limbs through tibial insertion

- two separate tunnels

- tie over bony bridge in tibia

- protect in plaster in extension

 

B. ORIF physeal sparing screw

- II guided

- directed posteriorly and obliquely

 

Outcomes

 

Stability

 

Some stretch of ligament occurs, but most knees are stable

 

Malunion

 

Occasionally fragment can block full extension

 

ACL Avulsion MalunionACL Avulsion MRI Malunion

 

 

 

 

 

 

ACL Rupture

Issues

 

1.  More common recently

- more high level sport

 

2.  High risk of reinjuring knee from instability

- can suffer permanent severe chondral and meniscal damage

 

3.  Risk of physeal arrest high if bone block across physis

- risk is growth arrest with ACL reconstruction

 

Epidemiology

 

Hemarthrosis

- 60% children have ACL tear

 

ACL tear

- 20% have mensical injuries

 

Most occur within 6-12 months of skeletal maturity

 

Management

 

Non operative

 

Issues

 

1. Non complicance children / young adolescents high

- high risk of chondral and meniscal damage

 

2.  Best to delay surgery if able til close to maturity

- avoid growth arrests

 

Technique

 

No sport / ACL brace / ACL rehabilitation

- until 2 years from skeletal maturity

 

Results

 

Aichroth et al JBJS Br 2002

- 60 children with ACL deficient knees 1980-1990 

- average age 12.5 years

- 23 patients treated conservatively, NHx was severe instability & poor knee function

- 15 knees had meniscal tear, 3 osteochondral fractures, 10 knees developed OA changes

- 37 knees with Hamstring ACL reconstruction average age 13 years

- no physeal arrest and satisfactory results in 80%

 

Operative

 

Indications

 

1.  Meniscal tear / displaced / blocking extension

 

2.  Failure non operative treatment

- continued instability

- high risk of chondral damage

 

3.  Within 2 years of maturity

 

Problems

 

Growth arrest / angular deformity / LLD

 

Predicting growth potential

 

Skeletal age with Greulich-Pyle atlas

Anderson table predict growth remaining using height and growth potential

 

Options

 

Extra-articular / over the top

Intra-articular

- physeal sparing

- partial transphyseal

- transphyseal

 

Extra-articular / Over the Top

 

Indications

- > 5 years to maturity

- avoid physeal injury

 

Procedures

 

McIntosh procedures

- ITB over the top

- combined intra-articular and extra-articular reconstruction

 

Technique Kocher JBJS Am 2005

 

Harvest

- lateral incision

- entire ITB taken

- left attached distally, detached proximally

- tubularised with no 5

 

Arthroscopy performed

- removal of ACL stump

- minimal notchplasty to avoid injury perichondral ring distal femur

 

Femur

- ITB passed extra-articular around lateral femoral condyle

- over the top position

- passed out anteromedial portal

 

Tibia

- 4 cm incision over anteromedial tibia

- clamp passed into knee under intermeniscal ligament

- groove for tendon made in epiphysis in this area

- graft passed through

 

Fixation femur

- knee 90o and foot ER 15o

- sutured to lateral intermuscular septum / extra-articular

 

Fixation tibia

- 20o flexion

- II used to assess location of growth plates

- groove made in proximal tibia

- graft sutured in place

 

Post op

- TWB 6/52

- restricted ROM 0 - 90o first 2 weeks

 

Results

 

Kocher et al JBJS Am 2005

- 44 patients average age 10

- ITB extra-articular physeal sparing / McIntosh modification

- 2 revisions for graft failure at 5 and 8 years

- no angular or leg length deformity

- excellent IKDC and Lysholm scores

- pivot shift normal in 31 and nearly normal in 11

 

Intra-articular reconstruction

 

Indications

- < 5 years to maturity

 

Assessment of Bone Age

 

Xray right hand / Greulich-Pyle atlas

- estimate bone age

- estimate amount of growth from femur and tibia

 

Theory

 

4 strand hamstring graft

 

Tunnels < 5% physeal area do not cause growth disturbance

- i.e. 6 - 8 mm drill hole

- need to keep vertical to minimise area

 

Tunnels 7 - 9% of growth area

- if leave transphyseal tunnels empty or have bone inside

- will form physeal bars

- if place soft tissue across (i.e. graft) will not form physeal bar

 

Options

1.  Physeal sparing

2.  Partial transphyseal

3.  Transphyseal

 

A.  Physeal sparing / transepiphyseal

 

Technique

 

Avoiding tibial physis

- tunnel anterior tibial epiphysis / trans epiphyseal

- graft secured with screw post / staple into tibial metaphysis

 

Avoiding femoral physis

- femoral tunnel horizontal and remains in femoral epiphysis

- transphyseal

- entrance in ACL origin

- use II guidance to spare the physis

- tunnel at 90o

- secured with endobutton

- 'over the top' position

 

Results

 

Anderson et al JBJS Am 2003

- 12 immature patients

- no LLD, stable knees

 

B.  Partial transphyseal

 

Technique

 

Tibial tunnel transphyseal

- keep small (6 mm)

- keep vertical

 

Femoral tunnel as above

- over the top

- physeal sparing

 

C.  Transphyseal

 

Technique

 

Analogous to adult reconstruction

 

Preventing growth arrest

- tunnels < 7% physeal area (7mm tunnels)

- soft tissue interposition across physis

- tunnels are vertical as possible

- central in the physis to avoid angular deformity

- single pass, wash +++

- anchorage away from physis

- endobutton for femur

- short screw / staple / post for tibia

- graft only across physis

 

Results

 

Kocher et al JBJS Am 2007

- 61 knees in patient average age 14

- 3% / 2 patients revision for graft failure at 14 and 21 months postoperatively

- pivot shift normal in 51 and nearly normal in 3

- no angular or LLD

- 3 cases of arthrofibrosis requiring MUA

 

 

 

Congenital Knee Dislocation

Epidemiology

 

Rare 

- 2:100 000

- 1/100 as common as DDH

- 1/3 bilateral 

- F:M 2:1

 

Associations

 

45% DDH

30% CTEV

 

Larsen Syndrome

Down's Syndrome

Arthrogryposis

 

Aetiology

 

Unknown

 

Two Theories

1. Intra-uterine packaging defect

2. Quadriceps intrinsic contracture / fibrosis

 

Pathology

 

Knee hyperextended 

- tibia dislocated anterior to the femur

 

Also

- quadriceps are tight

- absent cruciates

- patella hypoplastic or absent 

- patella alta

- valgus deformity

- hamstrings act as extensors

 

Classification

 

Type 1 

- can passively flex to 90°

- Subluxation / Hyperextension are minimal

 

Type 2

- can flex to 45°

- Moderate subluxation

 

 

Type 3

- -90 to 10o flexion

- complete dislocation

- no contact

 

DDx

 

Congenital Recurvatum of the Knee

- similar condition, but knee located 

- hyperextension is correctable, but flexion is limited

- splint knee to increase flexion

- KFO to maintain flexion

- good prognosis

 

Management

 

Non operative

 

Type 1

 

Attempt closed reduction & Pavlik harness

 

Type 2 & 3

 

Gentle MUA & serial casting

 

Once 90o, Pavlik harness 3/12

 

If DDH as well

- reduce knee first

- usually splint from foot to hip till 45° flexion 

- then Pavlik

 

Operative Management

 

Indication

 

Failure non-op treatment

 

Surgery often in syndrome e.g. arthrogryposis

 

Technique

 

Open reduction at 6/12 of age

- quadriceps engthening via anterior approach

- anterior capsulotomy / capsulectomy to reduce tibia onto femur

 

Ligamentous instability later very common

 

 

 

 

 

 

 

 

Congenital Patella Dislocation

Epidemiology

 

Rare

- ? dislocates in utero or post-natal period

 

Usually bilateral

 

Aetiology

 

Familial

 

Associated with Down's / Larsen's & Arthrogryposis

 

Pathology

 

Persistent lateral patella dislocation

- hypoplastic flat LFC

- aberrant lateral insertion quadriceps

- hypoplastic flat patella

 

Examination

 

FFD knee

Valgus knee

 

Plus

- laterally rotated patella 

- absent active extension

- difficult to palpate high & lat patella

 

Common diagnosis as toddler after started walking

 

Xray

 

Unrewarding till > 3 years

- doesn't usually ossify til 2 - 3 year

- can be delayed until the 6th year

- ossification complete about puberty

 

US / MRI

 

Demonstrate dislocated patella

 

Management

 

Timing

 

Operate when diagnosis made

- gives chance for PFJ to remodel

 

Operation

 

Technique

1.  Large lateral release

2.  Medial plication / VMO advancement

3.  Roux Goldthwaite

 

+/- Galeazzi - Semitendinosis transfer

+/- Hamstring release for FFD

 

Later problems with PFJ incongruity common as have two flat surfaces & hence early location better

 

Roux-Goldthwait procedure

- lateral 1/2 patella tendon detached 

- transferred beneath intact medial 1/2

- sutured to medial tibia (to insertion of sartorius) 

- modification is transfer medial 1/2 patella tendon to MCL

 

 

 

 

DDx Adolescent Knee Pain

 

Remember hip pain as a cause from SUFE / Perthes

 

Aetiology

 

Hip - Perthes / SUFE / Infection

 

PFJ - malalignment, chondromalacia, tendonitis

 

Cartilage - OCD

 

Meniscus (+/- discoid)

 

Ligament injury

 

Osgood Shlatters

 

Synovial Plica

 

Trauma

Tumor 

Infection

Inflammatory

 

 

Osgood-Schlatter's disease

DefinitionOsgood Schlatter's

 

Condition of traction apophysis into which part of patellar tendon inserts

- pulling osteochondritis of tibial tubercle

 

Epidemiology

 

Early teens

- M:F

- corresponds with growth spurt

- M 12-14 

- F 11-13 

- 1/3 bilateral

- athletes 21% vs 5% normal

 

Aetiology

 

Traction injury

- due to recurrent tensile microtrauma to an immature patella insertion 

- usually no history of injury

 

Symptoms

 

Activity related pain

- especially kicking & jumping

- pain on direct blow

- difficulty kneeling

 

Signs

 

Tender lump over tibial tuberosity

Pain on active extension

 

Osgood Schlatters

 

X-ray

 

Exclude other causes

- enlarged fragmented tibial tuberosity

 

CT scan 

 

May define ossicle in patellar tendon

- 10%

 

Management

 

Usually resolves spontaneously

- NSAID oral and topical

- hamstring stretching

- restriction of provocating activities

 

Steroid injections contra-indicated

 

Removal of ossicle if remains painful and mobile

 

Excision Osgoods 1Excision Osgoods 2Excision Osgoods 3

 

 

 

Leg Length Discrepancy

Congenitally Short Femur

Incidence

 

20 - 30 % LLD

 

Features

 

Intact femur radiographically +

- foot at contralateral mid-tibial level

- cruciate ligament deficiency

- coxa vara

- hypoplastic lateral femoral condyle with resulting valgus knee

- sclerosed lateral cortex

 

Amount of femoral shortening is variable patient to patient

 

Shapiro Type 1 growth disturbance

- i.e. LLD increases with time

 

Examination

 

LLD

Genu valgum

Fat thigh

High riding patella

 

Foot held externally rotated

 

X-ray

 

Hypoplastic femur

Coxa vara

Lateral sclerosis

 

2 groups

 

Simple femur hypoplasia

 

Femoral hypoplasia with coxa vara

 

Management

 

Align early then lengthen in primary school years

 

1. Realignment may involve

 

Coxa vara

- proximal femoral valgising osteotomy

 

Valgus knee

- Supracondylar osteotomy / guided growth 8 plates

 

2. Lengthen tibia and femur

 

Caution for dislocated knee

- ACL deficient

 

3. Contralateral epiphysiodesis

 

 

 

 

 

 

 

Fibula Hemimelia

Definition

 

Deficiency of bony elements on lateral side of lower limb

- characterised by hypoplastic or aplastic fibula

 

Types

 

Terminal

- abnormal foot

 

Intercalary

- normal foot

 

Epidemiology

 

Most common congenital deformity of long bones

- M:F   2:1

 

May be associated with PFFD

 

Sporadic 

- tibial hemimelia can be inherited

 

Presentation

 

LLD

 

Femur

- PFFD with type C / severe

- always slightly short femur

 

Knee

- cruciate deficiency

- hypoplastic LFC / valgus knee

 

Tibia

- anteromedial bowing

 

Ankle 

- equino-valgus / ball and socket

- tarsal coalition

 

Foot

- small limb / foot

- absent lateral ray(s)

- lateral tarsal bones fused or absent

 

UL deficiency

 

Classification Coventry

 

Type 1:  Short or partial proximal absence unilaterally

 

1A:  Stable ankle joint ± ball & socket joint

1B:  Unstable anklejoint / equinovalgus

 

Fibular Hemimelia

 

Type 2: Complete absence unilaterally

- tibia short & bowed anteromedially, dimple at apex

- associated with lateral ray deformity & coalitions

 

Type 3:  Bilateral

- severe

- associated with PFFD & upper limb deficiency

 

DDx

 

Fibular hemimelia / dysplasia is a spectrum

- child may have only gracile fibular and short leg in tibia and femur

- attributed to fibula hemimelia as no ACL, short femur etc

- can be pseudoarthrosis of tibia

- realise when tibia fractures and then doesn't heal

 

Management Issues

 

1.  Foot / ankle deformity

 

Concept

 

If less than 2 rays or unstable ankle

- cannot salvage

- symes / BKA

 

Indication for Syme's Amputation

- severe deficient foot that will not serve any useful purpose

- grossly unstable ankle

- associated tibial deficiency such that LLD & unstable AKJ preclude leg lengthening

- associated PFFD 

 

2.  LLD 

 

Usually > 5 inches

- can predict based on difference at presentation 

- can treat if have normal foot

 

Usual Options

- < 3cm shoe lift

- 3-5 epiphysiodesis

- 5-10 limb lengthening

- 10-16 limb lengthening + epiphysiodesis

- > 16 amputate

 

Technique

 

5cm at a time

Usually start when child 7-8, can understand reason for operations

 

3.  Tibial bowing

 

Least important

- usually corrects

 

 

 

Growth Plate Arrest

Aetiology

 

Trauma

- most common

- SH 4

- high energy SH 2 / 1 i.e. distal femur

 

Infection

 

Thombotic / embolic

 

Ischaemia (arteritis)

 

Radiotherapy

 

Classification

 

1. Central

- tenting 

- slows longitudinal growth +/- angulation

 

2. Peripheral

- can lead to rapidly progressive angular deformity

- especially small bar

 

Distal radius premature lateral growth arrest

 

3. Linear

- especially SH4

- effect depends on site and size

 

X-ray 

 

To assess deformity (angular and LLD)

 

CT

 

To accurately map size and position of bar

 

Management

 

1.  Epiphysiolysis

 

Indications

- < 50% growth plate

- > 2 year growth

- healthy skin

 

Technique

 

Excision of bar and interposition graft (fat)

- excision with high speed burr

 

Peripheral

- via direct approach to growth plate

 

Central 

- more difficult

- via metaphyseal window (avoid damage to the perichondral ring)

 

2.  Epiphysiodesis 

 

Concept

- remove active part of growth plate

- prevent angular deformity

 

Technique

- use drill under II guidance

- 4.5 mm

 

3.  Osteotomy

 

Indication

- correct angulation post growth plate fusion / epiphysiodesis

 

 

 

Hemihypertrophy

Definition

 

Asymmetry between L and R side more than due to normal variation

 

Upper limb hemiatrophy

 

Prevalence

 

Difficult to determine because no precise definition

 

Difficult to differentiate hemihypertrophy from normal variation

- enlargement soft tissues & bones also

 

Incidence

- 1/13 000 combined

- 1/86 000 non-syndromic

 

Non syndromic hemihypertrophy associated with 4% incidence tumours

 

Aetiology

 

Unknown

 

Non-syndromic is sporadic

 

Clinical Features

 

Rarely apparent at birth

- thicker skin, more hair, accelerated bone age on involved side

- ipsilateral paired organs increased in size also

 

Associations

 

Non-syndromic hemihypertrophy

 

Inguinal hernias

 

Genitourinary

- renal cysts

- cryptorchidism

- sponge kidney

- horseshoe kidney

 

Scoliosis

- non-structural

- compensatory to pelvic obliquity

 

Syndromic hemihypertrophy

 

Associated with cutaneous and vascular lesions

 

Classification

 

Total 

- involvement of ipsilateral paired organs

 

Limited 

- also divided as per extent of involvement

  

Classic hemi - UL & LL

Segmental - UL or LL

Facial - head and neck only

Crossed - opposite UL & LL

 

Diagnosis

 

Normal population may differ up to 1-2 cm in length and circumference

 

Rush and Steiner 1946

- 1000 US army recruits

- 23% equal leg lengths

- 77% 0.75 cm

- 15% > 1 cm

 

1cm difference

- in adolescent within normal

- in infant in pathological range   (4.2%)

 

Hemiatrophy V's hemihypertrophy

 

Compare length of L and R tibia and femora with normal standards

 

Hemiatrophy 

- no association with tumours

 

Non-syndromic hemihypertrophy / Beckwith-Weidermann syndrome

- are at increased risk for intraabdominal tumors 

 

DDX of non-syndromic hemihypertrophy

 

1. Beckwith- Weidmann syndrome

2. NF

3. Klippel-Trenaunay-Weber syndrome

4. Proteus syndrome

5. Malignant tumours

6. Olliers

7. Fibrous dysplasia

8. Poliomyelitis

9. Spastic hemiplegia of CP

10. Russell-Silver synd (short, cafe-au-lait spots, clinodactaly, hemiATROPHY)

11. JCA or haemophilia (secondary to hyperaemia)

12. XRT

13. Conradi-Hunnerman (chondrodysplasia punctata)

 

(5 - 13 may have contra-lateral shortened limb)

 

50% of hemihypertrophy will have syndrome

 

Beckwith-Weidermann Syndrome

 

Features

 

Pre / postnatal overgrowth, birth weight > 90 %

- neonatal hypoglycaemia

- macroglossia

- hemihypertrophy occurs in 13%

 

Predisposition for embryonal tumors (Wilms)

 

Aetiology

 

Genetic abnormality Chr 11 p15

- near gene for IGF -II

- AD transmission but most sporadic

 

Management

 

Need abdominal US

 

BWS with hemihypertrophy c.f. BWS without hemihypertrophy 

- risk embryonal tumour

- 27 V's 9%

 

Neurofibromatosis

 

NF1 may have overgrowth digit or limb

 

Klippel-Trenaunay-Weber

 

Characteristed by Hemihypertrophy + vascular malformations

 

AV Malformation Arm

 

Problems

- varicose veins

- port-wine nevi

- cutaneous and subcutaneous capillary haemangiomas

- cavernous haemangiomas

 

Proteus Syndrome

 

Name after Greek god who could change shape

 

Vascular + lymphatic malformations + lipomas

 

Features

- haemangiomas

- lipomas

- lymphangiomas

- macrocephaly

- hyperostosis skull

- overgrowth long bones

 

 

LLD Aetiology

A.  General Conditions

 

Hemi-hypertrophy or atrophy

 

Idiopathic

Klippel-Trenaunay-Weber syndrome

Proteus syndrome

Beckwith-Weiderman syndrome

Russel-Silver syndrome (atrophy)

 

Skeletal dysplasia

 

Chondrodysplasia punctata

Ollier's / Maffuci's

Multiple hereditary exostoses

NF

Fibrous dysplasia

 

Neurological

 

Closed HI

Polio

Spina bifida

CP

Spinal dysraphism / tumor / injury

Peripheral nerve injury

 

B.  Specific Regions

 

Tumour / trauma / infection / radiotherapy physis

 

Hip

- PFFD

- Coxa vara

- SUFE

- DDH

- Perthe's

- Tom Smith arthritis / post septic arthritis

 

Femur

- congenitally short femur

 

Leg

- fibula hemimelia

- tibial hemimelia

- bowing

 

Foot

- CTEV

 

Effect of LLD

 

Initially thought gives pain in back and contralateral knee and hip

- this may be debatable

 

1.  Short leg gait

- increased energy expenditure is the only proven effect of LLD

 

2.  Back 

- pelvic tilt and secondary scoliosis

- initially compensatory

- can become fixed

- ? causes LBP - now thought no different from general population

- most children's spines adapt (not so with adults)

- up to 2 cm easily tolerated

 

3. Contralateral Hip 

- uncovering of hip on long leg side in stance

- AKA long leg arthropathy

- ? increased OA 

- no evidence 

 

4.  Contralateral Knee 

- ? increased knee pain in athletes

 

Growth

 

2° Proliferative Zone chondrogenesis

 

Proximal Femur

- 3mm / year

- 15% leg

 

Distal Femur

- 9mm  / year

- 37% leg

- 70% of femur

 

Proximal Tibia

- 6mm / year

- 28%

- 60% tibia

 

Distal Tibia

- 3mm / year

- 20%

 

Growth Cessation

 

14-15 Girls

16-17 Boys

 

Relationships

 

LL vs Chronological Age

- steady growth occurs

- growth spurt in early adolescence

 

LL vs Skeletal Age 

- Green & Anderson curves

- Growth vs Skeletal age

- shows no growth spurt

- because growth spurt & maturation spurt occur together

 

Skeletal Age & Chronological Age

- maturation can occur at different rates

- if early, are tall early & stop growing early

- can have spurt where mature more than age

- pass through skeletal ages more quickly

 

History

 

Determine cause

Determine deformity

 

 

 

LLD Assessment & Quantification

Quantification of LLD

 

X-ray

 

1. Teleroentgenogram

- single exposure both legs

- long film with ruler

- Parallax errors

 

2. Orthoroentgengram

- same long Xray

- separate exposures hip, knee & AJ 

- eliminates parallax error

- problem artefact 

 

3. Scanogram

- similar separate exposures

- film moved between exposures

- smaller film

- multiple exposures

 

CT scan

 

Software measures distances 

- accurate to 0.2 mm

- legs must be in same position

- fast

 

Skeletal Age

 

1. Greulich- Pyle Atlas

 

Xray Left hand (non dominant)

- correlated with Green- Anderson table LL

- less accurate < 6

- improved accuracy by focusing on hand bones rather than carpal bones

 

2. Tanner- Whitehouse Atlas

- more refined

- 20 landmarks graded L Hand

- more accurate

- can't use as not correlated with LL

 

Prediction of Growth

 

Note that all methods have an inherent error of 12 months

- gives accuracy to 1.5 cm

 

Need > 3 measures 4/12 apart for all methods

- If inadequate data wait till older or wait till skeletally mature

- If acquired event caused LLD, can plot onto graph

 

1.  Menelaus "Rule-of-thumb" Method

 

Less accurate

- based on chronological age

- only valid from age ten

- convenient / easy / simple

 

Basic rules

- girls stop growing at 14 

- boys stop growing at 16

- distal femur 9 mm 

- proximal tibia 6 mm 

- distal tibia / proximal femur 3 mm 

 

Calculate how much growth lost from fusion of physis / Predict effect of epiphysiodesis

- Effect = Physis rate x years of growth Left

 

2.  Green & Anderson tables

 

Growth remaining method

- uses skeletal age

- requires graph

- estimates growth potential in distal femur and proximal tibia at various skeletal ages

- separate charts for girls and boys

 

3.  Moseley

 

Straight - Line Graph Method  

- uses Green & Anderson data

- applied to a chart

 

At least 3 measurements each time

1.  Length long leg

2.  Length short leg

3.  Skeletal age

 

Do so 3 times separated by 3-6 months

- accuracy improves with increased plotting

 

Plot the points for long and short leg on a vertical line for chronological age of either boy or girl

- create 2 lines for short and long leg over time

- line of best fit

- gives LLD at maturity at right of graph

 

Technique

- plot Long leg length on long leg line against skeletal age

- plot Short leg length on short leg line against skeletal age

- able with at least 3 measures to create line of best fit

- extend lines to maturity

- difference is LLD

 

Growth rate of each leg = slopes

- parallel or divergent 

- AKA static or progressive

 

Then use Menelaus rule of thumb to determine appropriate age for epiphysiodesis

 

4.  Paley multiplier

 

State of the art

- 2000

- take LLD for boy or girl

- multiplier for chronological or skeletal age

- predicts LLD at maturity

 

Patterns of LLD

 

Adds to difficulty

- static

- progressive

- regressive

 

Shapiro 

 

1982 5 developmental patterns

- 75% types I and II

 

I Increasing

- LLD increases at constant rate

- hemihypertrophy / atrophy

- tibial pseudoarthrosis

 

II Increasing plateau

- similar early, but annual rate of increase diminishes thereafter

- Perthes

 

III  Plateau

- discrepancy increases, then stabilises

- fracture femur

- DDH

- Polio

 

IV Increasing- decreasing

- similar to III, then late increase at end of growth

- DDH

- hemihypertrophy

 

V Decreasing

- Initial increase, steady, then decrease

 

Progressive LLD

 

Progression Rate = Change LLD / Time

 

Final LLD

- add Current LLD to Prog Rate x Time to Skeletal maturity

 

 

 

 

 

 

LLD Examination

Four Physical Outcomes

 

1.  Symmetrical Stance & Level Pelvis 

 

A.  LL Equality

- components equal with no deformity

 

B.  Components equal with bilateral symmetrical deformity

- eg bilateral varus knees

 

2.  Symmetrical Stance with Oblique Pelvis 

 

Uncompensated LLD

 

3.  Asymmetrical Stance & Level Pelvis 

 

A. Fully Compensated LLD

- flexed contralateral knee 

- equinus ipsilateral ankle

 

B. Sagittal deformity with ipsilateral sagittal compensation

- FFD knee with equinus & hip flex OR

- fixed equinus with flex knee & hip OR

- FFD hip with equinus & flex knee

 

C.  Coronal deformity with contralateral coronal deformity

- valgus of knee & contralat varus of knee

 

4. Asymmetrical Stance with Oblique Pelvis 

 

A.  Partly compensated LLD

- partly flexed contralateral knee

- partly equinus of ipsilateral ankle

 

B. Coronal hip deformity with sagittal compensation

- fixed hip adduction with contralateral knee flexion / ipsilateral equinus

- fixed hip abduction with ipsilateral knee flexion / contralat equinus

 

C.  Sagittal deformity with coronal compensation

- FFD knee c ipsilateral hip abduction

- fixed equinus c ipsilateral hip ADD

 

Leg length Examination

 

1.  Look

 

Posture

- flexed knee

 

Signs hemihypertrophy

- NF
- haemangiomas / lipomas (Proteus, Klippel-Trenau-Weber, Beckwith)

 

Scars

- trauma, infection

 

2.  Gait

 

Children

 

Compensate well 

- walk on toes short leg usually / equinus

- flexion long knee uncommon as energy++

 

Adults

 

Compensate less well

- walk with bilateral heel-toe gait

- vaults over long leg

- excess sagittal head motion

 

3.  Measure LLD
 

A.  Functional LLD

- on blocks

- heels flat, nil knee FFD (if able)

- correct pelvic tilt

- should correct scoliosis

 

Conclusion

- if can make pelvis stable

- ASIS equal

- blocks are a quantitative measure of functional LLD

 

B.  Apparent LLD

 

Lying on bed

- measure from xiphisternum to medial malleolus

- no correction for contractures

 

C. Real / True LLD

 

Must correct for deformity in coronal and sagittal plane

 

Exclude

- hip adduction / abduction contracture

- hip FFD

- knee FFD

 

Scenarios

1.  Hip FFD

- pillow under both thighs

2.  Knee FFD

- pillow under both knees

3.  Hip adduction contracture won't correct to neutral

- measure each leg crossed over the other

4.  Hip abduction contracture won't correct to neutral

- place both legs in similar position

 

If there is a contracture, perform the above measures

- then meaure the intercalated segments

- from ASIS to medial joint line

- medial joint line to medial malleolus

 

4.  Identify site of shortening

 

Galeazzi

- must not forget can have small foot / old calcaneal fracture / wasted buttock

- hips and knees flexed

- side by side

- look for tibial / femoral shortening

 

If shortening above knee, find out if shortened above greater trochanter

- i.e. hip deformity

 

Bryant's triangle

- line perpendicular to GT and ASIS

- distance between

- quantify in fingerbreadths

 

Nelaton's line

- line from ischial tuberosity to ASIS

- GT should be on or below line

 

Klisics line

- GT to ASIS

- should aim to umbilicus

- will be more parallel

 

5.  Other

 

Examine knee stability

- can have problems lengthening femur if ACL deficient

- i.e. fibula hemimelia

 

 

 

 

 

LLD Management

Goals

 

1. Equal leg lengths

- goal is mild to moderate isolated discrepancy

 

2. Unequal leg lengths

- goal with paralysis / equinus foot

- aim 1-2 cm short for clearance

 

3. Level Pelvis

- should use blocks to estimate functional correction

 

4. Vertical LS Spine

- vertical spine more important than level pelvis

 

5. Equalisation with Prosthesis

- goal with excessive LLD

- usually femur < 1/2 normal length or LLD >15cm

 

Principles

 

Correct coexisting deformity first

- can decrease discrepancy

 

May require correction of other problems first

- release of contractures

- correct angular deformity

- correct spinal deformity

- excision of bony bridge

- completion of partial arrest

 

Treatment guidelines by projected discrepancy

 

0 - 2 cm

- no treatment, heel raise

 

2- 6 cm

- shoe lift, epiphysiodesis

- maximum subtalar joint can take

 

6 - 15 cm 

- lengthening + epiphysiodesis

 

> 15 cm 

- amputation

 

Prosthesis

 

Shoe Lift / sole raise

 

Good treatment if 2-5 cm

- aim is to improve gait

 

Note

- > 5 cm ankle sprains

- joint unable to cope

- need patten boot (steel reinforcements of STJ)

 

Guidelines

- 0 - 1.5cm heel raise

- 1.5 - 5cm heel and sole raise

- 5 - 12cm patten and boot

- 12 - 20cm patten and AFO / prosthesis

- 20+cm prosthesis

 

Epiphysiodesis

 

Advantage

 

Often treatment of choice

- low morbidity

- accurate

- can shorten at level of deformity

 

Disadvantage

 

Operate on normal leg

- get loss of height & proportions

 

Indications

 

Anticipated LLD 2-6 cm

 

If > 6 cm LLD 

- excessive height loss

- knees at different level 

 

Phemister Technique

 

Create bony bridge

- medial & lateral approaches

- access window removed

- physis excised with curette

 

Percutaneous Technique 

 

Under II guidance

- percutaneous incision

- medial and lateral drilling into physis

- angle drill anterior and posterior

- entry points anterior, middle and posterior

- see white growth plate on drill

 

Complications

 

Continued longitudinal growth or angular deformity

- failure to eliminate growth plate

 

Femoral Shortening

 

Indications

 

Maximum 2 - 6 for reasons above

 

Consider if

- too old, insufficient growth remaining

- can't predict LLD and wait til maturity

 

Usually performed over a nail

 

Tibial Shortening

 

Easier 

Max 3cm

 

Growth Stimulation

 

Stimulate physis by hypervascularity 2° irritative

- insert vicryl into growth plate

- moderate success

 

Leg Lengthening

 

See Limb Lengthening

 

 

 

 

 

Limb Lengthening

Definition

 

Concept of distraction osteogenesis

 

Popularised by Ilizarov in the Soviet Union 

- initially in the 1940's

- popularised in the 1980's

- also created the circular fixator

 

Corticotomy

- low energy osteotomy

- attempt to minimise damage to blood supply and periosteum

- avoid use of saw

- minimise thermal necrosis

 

Callotasis

- gradual incremental distraction of a fracture callous after a latency period

- intramembranous ossification in zone of distraction

- type 1 collagen with osteoid laid on collagen

 

Advantage

 

Maintains height & proportions

- Avoids operating on normal side

 

Disadvantage

 

Difficult

- steep learning curve

- complication rate 10 - 250%

 

Indications

 

General rules

- LLD 6-15 cm

- over 15 cm risks outweigh benefits

- < 20% limb

- 5cm in femur / 5 cm in tibia

 

Limitation is due to muscles / ligaments / nerves

- can repeat at staged procedures

- i.e. 5 cm per bone at any given time

 

Site 

 

Site of deformity best

 

Metaphyseal lengthening easiest

- large cancellous surface area

- thin cortex

- best blood supply

 

Contra-indications

 

Mentally or medically unstable

- long and demanding process

- 12 months or more

 

Unstable joints

 

Associated neurology

- Weakness /  Insensate

 

Technique

 

1. Osteotomy & Frame / Nail

2. Latency Period

3. Distraction Period 

4. Consolidation Period

 

Maintaining blood supply via periosteum is the key

 

1A.  Osteotomy with Periosteal Preservation

 

Open Corticotomy

 

Drill-holes & closed osteoclasis / Di Bastiani

 

Used in the femur

- percutaneous skin incision

- multiple drill holes first to weaken bone

- osteotome to complete

- apply force to complete fracture

 

Usually stablise initially with temporary external fixator

- perform osteotomy

- insert nail / ISKD

- remove frame

 

Tibial technique with Gigli saw

 

Proximal corticotomy in metaphyseal bone

- below tibial tuberosity

- frame on initially to stabilise

- incision lateral crest tibia, elevate periosteum lateral tibia

- incision medial border tibia, elevate periosteum medial tibia

- pass artery clip between periosteum and bone on one side

- pass wire on other side, retrieve with clip

- pass suture, then pass gigli saw

- perform subperiosteal corticotomy, periosteum intact

 

Wagner Osteotomy

 

Contra-indicated now

- osteotomy and acute resection

- distraction

- bone grafting and plating

- superceded by lizarov techniques

 

1B. Devices

 

Need stable device or risk non-union with fibrocartilage

 

Uniplanar Device 

- Wagner, Orthofix

- simple

- no angular correction

- "Cantilever Loading"

- problems with pin loosing

- often necessary in femur

 

Ilizarov

- multiplanar correction

 

IM nail  / ISKD

 

Self lengthening nail

- movement of leg induces lengthening mechanism

- maximum of 5 cm

- if only want 3 cm, lengthen 2cm before insertion

 

Advantage

- all internal 

- no pin site complications

- easier for patient

 

Disadvantage

 

Runaway nail 

- lengthens too quickly

- cause contractures and nerve injury

- nothing can be done about this

- incidence 5%

 

Jammed nail 

- take to theatre to unblock

- may have to debride callus if lengthening too slow

 

2.  Latency period 

 

Usually 1/52

- allow callus to form before distraction

- reduce latency period in child as may start to ossify

- increase latency period in diabetics / steroids

 

3.  Distraction Period

 

Rate / Regenerate

 

Optimum 1mm / day turning 4x / day

- balance premature union vs non union

- slower in adults / diabetics

- i.e. 2-3 x day

 

Stop if

- poor new bone formation

- nerve palsies

- joint subluxation

- joint contracture 

 

4.  Consolidation

 

1 month per cm or

Double the distraction period

 

Complications

 

Pin infection

 

Management

- early oral antibiotics

- patient should have script at home

- take if pin site appears red or begins leaking fluid

- increase frequency of pin site cleaning

 

Prevention

- tight well tensioned pins

- don't place pins throught muscle

 

Nerve  injury

- demyelinate if lengthen > 6%

 

Contractures

- muscle elongates poorly

- max 1mm / day

- congenital LLD more susceptible than acquired

 

Risk

- T achilles contracture in tibia

- FFD / adduction femur

- knee FFD

 

Device failure

- broken pins, loss of position

 

Fracture

- inadequate consolidation before device removal

- 10 - 15% incidence

- can result in loss of length or angular deformity

 

Premature Consolidation

- unable to distract

- break pins / wires

 

Poor regenerate

- inadequate latency period

- too rapid distraction

- poor blood supply

 

Joint subluxation

- ligamentous insufficiency

 

Delayed or non union

- constant observation

 

Bone growth impairment

- damage to physis

- likely secondary to pressure

- best if delayed until after skeletal maturity

 

Psychological Stress

 

Distraction Physiolysis

 

Concept

- tibial lengthening by distraction across physis

- similar results to metaphyseal lengthening

 

Indications

- need extra length in skeletally immature

 

Complication

 

Physeal injury risk

 

Chondrodiastasis if < 1mm / day

 

 

 

Tibial Hemimelia

Definition

 

Paraxial deficiency of skeletal elements on medial aspect of lower limb

 

Epidemiology

 

Only skeletal deficiency with a documented familial occurrence 

- AD

 

1/ 1 000 000

 

Bilateral 30%

 

Clinical

 

Leg short +++

 

Tibial Anterolateral Bowing

 

Foot fixed in severe varus

- can mimic CTEV

- sole facing perineum

 

Knee

- FFD

- may be unstable

- no quads mechanism

 

Associations

 

Cleft hand

Reduplication of toes

CDH 20%

 

Classification

 

MRI to assess extent of proximal failure

 

1.  Unilateral Complete Type 1

 

No proximal tibial remnant

- usually foot abnormalities

- distal femur is hypoplastic & ossification delayed

- knee is featureless / unstable

 

Tibial Hemimelia Complete

 

Management

- amputation early

- around 1 year before child gets attached to it

 

2.  Unilateral Partial Type II

 

Well developed proximal tibia & knee joint

 

Tibial Hemimelia Partial

 

Management

 

A.  Knee

- proximal tibiofibular synostosis to prevent proximal migration

- fuse distal fibula to end of tibia

- then either symes or fuse fibula into calcaneus

 

B.  Ankle foot

- distal tibial deficiencies

- get equinovarus deformity similar to club foot

- tibiofibular synostosis

- then either keep foot if good or Symes

 

3.  Bilateral

 

Management

 

A.  Bilateral through knee amputation

 

B.  Can try to make fibula into tibia and perform symes on one side

- Brown procedure

- need good quadriceps

 

 

 

 

Miscellaneous

Acutely Limping child

DDx

 

CDH / LLD

 

Transient Synovitis/ Septic Arthritis

 

Perthes 

 

SUFE

 

Inflammatory Arthritis

 

Timeline

 

0-2 DDH

2-5 septic arthritis

2-10 synovitis

5-10 perthes

10-15 SUFE

 

5 questions

 

Is limp due to pain?

What type of limp?

Is limp gradual / sudden?

Any localizing features?

Is the child unwell?

 

Examination

 

Gait

Standing

Supine on couch

 

Investigations

 

X-ray

Bone Scan

US / MRI

 

Haematological tests

 

FBE / ELFT's

ESR / CRP

RF / ANA

 

 

 

 

Arthrogryposis Multiplex Congenita

Definition

 

AMC characterised by non progressive congenital rigidity of 2 or more joints

- Arthrogryposis means curved joint

- Arthrogryposis refers to 150 syndromes

 

Types

 

1.  Amyoplasia

- involves all 4 limbs

 

2.  Distal Arthrogryposis

- AD

- type 1 98% hand / 87% feet

- type 2 involve other systems

 

Epidemiology

 

1 in 3000 live births

-  hereditary pattern unknown

 

Aetiology

 

Fibrosis of periarticular soft tissues during joint development

- leads to development of incomplete fibrous ankylosis

- unknown cause

 

Numerous theories

- CNS abnormality / anterior horn cell 

- environmental factors - toxins / virus

- packaging defect

- oligohydramnios

- limitation of foetal movement

 

Pathology

 

Muscles replaced by fibrous tissue

Spinal cord decreases in size, especially at the limb enlargements

- decreased number of anterior horn cells

 

Clinical feature

 

Normal intelligence

 

Wooden doll facies

 

Usually involves all 4 limbs (AMC)

- joint contractures with webbing

 

Marked limitation of joint ROM

- no skin creases

- scant subcutaneous tissue & muscles

- skin tense & glossy

- ± dimpling at joints

 

Typical Deformities

 

UL

- shoulders adducted and IR

- elbows extended

- wrists flexed, pronated & UD

 

LL

- hips flexed, ER & abducted / dislocation

- knees flexed

 

Scoliosis

 

Also

- teratologic DDH

- teratologic CTEV

- congenital patella dislocation

 

Management

 

Goals

 

Achieve maximum function

Permit mobility / ability to transfer

Enable Feeding & hygiene

 

Principles

 

Lower limbs first

- plantigrade feet first

- extend knees

- reduced hips

 

Upper limbs

- ability to bring hand to mouth / elbow flexion

- ability to toilet & push off when rising with other hand

 

Timing

- correct all LL deformity by 2 years to allow walking

- address UL deformities later

- allows bimanual function to be established

- allows functional assessment

 

Algorithm

- full-time bracing until age 6

- night splints until skeletal maturity

- soft tissue procedures in young child

- bony procedures as approach skeletal maturity

 

Talipes Equinovarus

 

Infant

- rarely responds to bracing

- requires PMR before walking age

- AFO's required post-operatively until skeletal maturity

- failure be require talectomy

 

Older child

- triple arthrodesis is procedure of choice

 

Knee Flexion Contracture

 

Non-operative

- daily passive ROM

- serial casts

- night splints

- surgery for resistant cases

 

Operative

 

Surgical release 

- capsule

-  ± collaterals & cruciates

- hamstring lengthening

- serial casting post-op

 

Salvage

- supracondylar osteotomy near skeletal maturity

 

Hip Dislocation

 

Operative

 

Open reduction ± femoral shortening & derotation osteotomy

- age 1

- when knee FFD corrected

- accept situation if reduction not achieved by 2 years

- because of increased risk of unilateral failure with pelvic obliquity & scoliosis consider accepting if bilateral 

 

Hip Contractures

 

Issues

 

Hip FFD > 30°

- increases lumbar lordosis

- increases knee flexion contracture

- crouch gait

 

Non operative

- correction of knee contracture

- maintaining patient prone

 

Operative

- subtrochanteric osteotomy near skeletal maturity

 

Hand

 

Little to offer for severe deformity

 

Wrist flexion

 

1.  Hand stiff

 

Non operative

- pronated flexed wrist enables forearm radial borders to appose each other to produce pincer grip

 

2.  Hand functional

 

Treat FFD

- Gives same range of wrist movement, but in more functional position

 

A.  Dorsal closing wedge capitate / FCU transfer

B.  Carpectomy

C.  Wrist fusion

 

Shoulder Internal Rotation

 

Issue

 

Should be addressed before elbow corrected

 

Management

 

External rotation osteotomy in proximal shaft

 

Elbow Extension

 

Issue

 

Need one elbow bent & one straight

 

Operative

 

Options

- unilateral posterior capsulotomy & triceps transfer

- steindler flexorplasty

- pectoralis major bipolar transfer

 

Scoliosis

 

Brace may be successful

Often requires segmental fusion

 

 

Congenital Sclerosis

DDx  MD COP MOOO

 

Diaphyseal dysplasia (Engleman’s)

Metaphyseal Dysplasia (Pyles Disease )

 

Caffey's / Infantile Cortical Hyperostosis

Osteoarthropathy Familial Hypertrophic

Pyknodysostosis 

 

Melorheostosis / Mastocytosis / Myelosclerosis

Osteopathia striata

Osteopetrosis

Osteopoikilosis (bone islands)

 


 



 

 

 

 

Developmental Milestones

 

3 months - head control / lifts head when prone

6 months - rolls over

9 months - sits up without support

 

12 months - walks with one hand support

15 months - walks by themselves

 

2 years - ascends stairs & can run forwards

3 years - pedals tricycle

4 years - balances on one foot

5 years - hops on one foot

 

 

 

 

Down's Syndrome

Epidemiology

 

Trisomy 21

 

1 in 800

 

Aetiology

 

Advanced maternal age

- 1/ 50 mothers > 45

- however mothers < 30 still most common

 

Genetics

- 95% non disjunction during meiosis

- 4% are result of translocation (Chr 21 joins to Chr 14)

- 1% are mosaics

 

General Features

 

Mental retardation / Developmental delay

 

Short stature

 

Hypotonic

 

Simian crease (palmar)

 

Clinodactyly 

- coronal deformity fifth ray

- can see at US screening

 

Ligament Laxity 

- patellar subluxation 

- planovalgus feet

 

Characteristic Facies 

 

Foreshortened head

Eyes slant upwards

Prominent epicanthic folds

Ears low-set 

Nose flattened

Lips parted

Macroglossia

 

Medical Complications

 

Cardiac defects (ASD VSD)

- 50%

 

Endocrine disorders

- hypothyroidism 15%

- DM 1%

 

Duodenal atresia 10%

 

Alzheimers 70%

 

Orthopaedic Issues

 

2 main orthopaedic problems

- atlanto-axial instability

- hip instability

 

Also

- scoliosis

- spondylolithesis

- SUFE

- patella dislocation

 

Atlantoaxial Instability

 

Clinically

- present in 20%

- diagnosed with dynamic views

- usually causes no problems

- occasionally have neurological defects from dislocation

 

Asymptomatic

 

Children shouldn't

- play contact sports

- dive

- perform gymnastics

 

Symptomatic

 

Children should undergo C1/2 fusion 

 

Hip Instability

 

Epidemiology

- 3% incidence dislocating hip

- doesn't present at birth

- presents later in life

- can be highly unstable

 

Aetiology

- ligamentous laxity

- capsule thin and deficient

- increased femoral anteversion

- acetabulum shallow and anteverted / deficient posteriorly

 

Examination

- walk ER to maintain location

- can dislocate hip spontaneously

 

Investigation

- MUA arthrogram demonstrates subluxation

 

Treatment

- FDRO + capsular plication

- spica 6/52, abduction brace 6/12 at night

 

Results

- 1/4 poor results, continued instability

- difficult problem to treat

 

 

 

 

 

 

Juvenile Chronic Arthritis

Classification

Definition

 

JRA / JCA

- heterogenous inflammatory joint disease

- °infective aetiology 

- > 3/12 duration & < 16 years old

 

Systemic / Pauciarticular / Polyarticular

 

Epidemiology

 

1: 10000

- M = F

- Different subgroups have associated with different HLA types

 

Aetiology

 

Unknown

- ?  Immune aberration to infectious agent

- Similar to RA

- Genetic predisposition

 

Classification

 

Must be > 6/52

Classify after 6/12

 

1. Systemic JCA 

- Still's disease

- fever, rash, lymph nodes, hepatosplenomegaly

- 20%

- poorest long term prognosis

 

2. Pauciarticular JCA 

- < 4 joints

- 30%

 

Divided into

A. Early onset < 6

- ANA

- F > M

B. Late onset

- like Ankylosing Spondylitis / HLA B27

- M > F

 

3. Polyarticular JCA >5 joints

- 60%

- most common

 

Divided into

A.  Seronegative

B.  Seropositive

- more severe with joint destruction

 

Systemic / Still's Disease

 

Epidemiology

 

Usually < 3 years old

- M = F 

- usually not ANA or RF +ve

 

Systemic symptoms

 

Febrile ~ 40°

Maculopapular rash 

Malaise

Nodes

Hepatosplenomegaly

Joint symptoms occur weeks after onset

 

Joints

 

Initially mild swelling & pain

- may progress to Polyarthritis

- commonly Knee / Wrists / Ankle & Midfoot

- flexor Tenosynovitis

- MCPJ & PIPJ swelling

 

Usually resolves with systemic illness

- long term affects hips and cervical spine

 

Rash

 

Fluctuates with the fever 

- may only appear at night or in evening 

- i.e. not when seen in clinic

- salmon pink

 

Investigations

 

ESR ~ 100

Increased WCC + Neutrophilia

Mild Anaemia

Increased IgG

RF negatgive

Increased CD4 T Cells

 

Prognosis

 

Children almost always get spontaneous resolution of disease as teenager

- left with degenerative joints

- moderate to severe arthritis in ~ 50% 

- frequent involvement of hips

 

Stunted growth 

- steroids make stunting even worse

 

Pauciarticular

 

Very typical case presents with monoarticular arthritis of knee

- < 5 joints in 1st 3/12 

- no Stills symptoms

 

A. Early onset / 50 - 60% ANA Positive

 

Present

- <6 years 

- F>M

 

Medium joints

- Knee / AJ / Elbow

- Flexor Tenosynovitis

- PIPJ 

 

Chronic Iridocyclitis

- 50%

- resolves after few years

- follow up until late teens for eyes

 

B Late onset / Positive HLA B27

 

Present

- > 9 years

- M > F 

- positive FHx

- RF negative

 

Most develop Sacroiliitis & Spondylitis

 

LL affected 

- KJ / AJ / Hip

 

Enthesitis

- Plantar Fascia / TA / Tib Tubercle

 

Iritis

- commonest cause of blindness in western world 

- can be treated with steroids 

- must all see ophthalmologist every 3 months until 20 years old even if in remission

 

Overall excellent prognosis 

- minimal drug treatment required

- Main problem is actually the iritis

 

Polyarticular

 

Present

- > 5 joints within first 3/12 

- F > M 

- ANA positive in 50%

- symmetrical polyarthritis

- small & large joints

 

A. Seronegative

 

More common 

- 85% of polyarticular

 

Affects KJ most commonly

- wrists / ankle / C spine

- flexor tenosynovitis

- 1st MTPJ

 

B. Seropositive / Juvenile RA

 

Older girls

- Small joints of hands & feet

- nodules

- +ve RF

 

Rapidly progressive with joint destruction

- more likely to be persistent into adulthood

 

X-ray

- non-specific changes early

- have severe periarticular osteopenia

- later joint erosion & deformity

 

 

 

Management

Nonoperative Management

 

Therapy

 

Physio

- prevent contractures

- prevent wasting

 

Splints

- dampen infection

- aid ADL's and schoolwork

 

Medications

 

NSAID

- mainstay of treatment

- paediatric suspensions available

- avoid Salicylates because of Reye's disease

 

IV Immunoglobin

- Still's

 

Methotrexate

- increased use

- much higher doses in kids

- attempt to prevent progression

- aggressive treatment in polyarticular disease with poor prognosis

 

Gold & Penicillamine 

- seropositive

 

Sulphasalazine

- spondyloarthropathy

 

Systemic Steroids

 

Issue

- doesn't affect progression of disease 

- se limited by SE: stunting / osteoporosis

 

Indications

- unresponsive Still's / Polyarthritis

 

Alternate day treatment

- minimal stunting

- avoid suppression hypothalamic - pituitary axis

 

HCLA 

 

Indications

- flares of pauciarticular disease 

- knee / flexor sheaths

 

Advantage

- non specific temporary palliative treatment

- allows good pain relief

- early mobilisation

- therefore preventing contractures / muscle atrophy

- preserving function

 

Infliximab

 

Monoclonal anti TNF antibody

- effective in 60%

- 5% develop allergic reactions

- 10% develop new autoantibodies

 

Operative Management

 

Joints

 

Shoulder

- may require soft tissue releases + synovectomy

 

Elbow

- may need synovectomy + radial head resection

 

Wrist

- very frequently involved

- short ulna

- ulnocarpal deviation (c.f. radial deviation in adult RA)

- require synovectomy / fusions

 

MCPJ

- radial deviation (c.f. ulna deviation in adult RA)

- synovectomy / realignments / replacement

 

Finger

- much less likely to be involved than adult form

- Boutonniere

- Swan neck quite rare

 

Thumb

- may develop flail thumb secondary to erosion

 

Hip

- Otto pelvis

- bilateral protrusio

- atlantoaxial subluxation

 

Synovectomy

 

Most common in wrist / hand / elbow

- limits pain and improves function in short term

- does not affect long term outcome or ROM (> 3 years)

 

Complications

 

Growth defects

- growth retardation with steroids

 

Epiphyseal disturbances 

- external tibial torsion

- dysplasia of distal ulna (commonly short)

- dens hypoplasia 

- scoliosis

 

Iridocyclitis

- pauciarticular disease

- can lead to blindness

 

Amyloidosis

- long standing Still's

- may be fatal

 

Prognosis

 

Polyarticular

- juvenile or systemic usually only last about 6/52

- 75% long remission with little residual disability

 

Poor prognosis

- systemic with multiple joint involvement

- RF +ve

- onset < 1 year old

- long active period disease > 5 years

 

Death uncommon

- infection early

- amyloidosis late

 

Marfan syndrome

Definition

 

Inheritable disorder of connective tissue

 

Epidemiology

 

Male = Female

1 in 5000

 

Nil geographical variation

 

Aetiology

 

AD

 

Gene

- FNB1

- fibrillin

- chromosome 15

 

Now diagnosed by genetic studies

 

Pathology

 

Fibrillin is a component of elastic fibres

 

DDx

 

Homocystinuria

- no FHx

- diagnose via urinalysis

- presents the same way

 

Ehler Danlos

- very fragile, mobile skin

 

Ehler Danlos Fragile Skin

 

Stickler's

- mental impairment

 

Clinical Features

 

Major criteria

- FHx

- pectus excavatum requiring surgery

- pectus carinatum

- arm span : height ratio > 1.05

- wrist sign (LF and thumb overlap)

- thumb sign (projects past ulna border palm)

- scoliosis > 200

- pes planus

- protrusio

 

Minor

- pectus excavatum

- minor scoliosis

- thoracic lordosis

- high arch palate

- joint hypermobility

 

Need 2 major or 1 major / 2 minor

 

Skeletal

 

Arachnodactyly

- long fingers and toes

 

Thumb sign

- protrudes past ulna border palm

 

Pectus excavatum / carinatum

 

Ligamentous laxity

- genu valgum

- PFJ instability

 

Scoliosis

- 60% patients

- right thoracic

- 20% need OT

- complicated by thoracic lordosis

 

Spondylolithesis

 

Dural ectasia

 

Flat feet

- ligamentous laxity

 

Protrusio

- consider triradiate cartilage fusion

 

Elbow joint contractures

 

Extra-skeletal

 

High arched palate

 

Lens dislocation

- also short sighted, astigmatism

 

CVS

- AR, MR

- aneurysm

- dissection

 

Spontaneous Pneumothorax

 

 

 

Neurofibromatosis

Epidemiology

 

NF1 / Von Recklinghausen 

- most frequent of these disorders 

- 1/3000 - 1/4000. 

 

NF2

- VIII nerve schwannomas

- nil musculoskeletal manifestations

 

Aetiology

 

AD

- 50% due to new mutation

 

Gene protein neurofibromin

 

Diagnosis

 

Young children 

- multiple café-au-lait spots that appear in the first year of life.

 

The NIH Consensus Development Conference Statement

- diagnostic criteria for NF1 are met in an individual who has two or more of the following:

 

1.  Six or more café-au-lait spots over 5mm in prepubertal individuals and over 15mm in postpubertal individuals

2.  Two or more neurofibromas of any type or one plexiform neurofibroma

3.  Freckling in the axillary or inguinal region

4.  Optic glioma

5.  Two or more Lisch nodules (iris hamartomas)

6.  A distinctive osseous lesion such as sphenoid dysplasia or thinning of the long bone cortex with or without pseudarthrosis

7.  A first degree relative (parent, sibling, or offspring) with NF1 by the above criteria.

 

Clinical Features

 

Café au lait

 

< 2 normal

6 or more > 0.5cm, smooth edges

Aid early suspicion of NF1

 

Neurofibroma

 

Cutaneous

- raised over the skin

- bluish in colour

- increase in number ofter puberty and with pregnancy

- nil malignant potential

- develop from small cutaneous nerves

 

Plexifom

- bag of worms feeling

- usually develop from major nerves

- potential to become malignant

 

Axilllary / Inguinal Freckling

 

Aids early diagnosis

- second most common feature in children

- 80%

 

Lisch Nodules

 

Hamartomas in iris

- 90% patients over 6

 

Optic Glioma

 

&0% found in NF1

- usually benign and small

- can sometimes rapidly increase in size

 

Elephantiasis

 

Dermatological manifestation

- large soft tissue masses

- rough, raised vilous skin

 

Verrucous Hyperplasia

- skin overgrowth

- velvety, soft papillary

 

Issues

 

Malignancy

- 30 - 50 years

- large deep lesion

- increasing in size

- apin

 

Neurofibrosarcoma NF Leg MRI Coronal T2Neurofibrosarcoma NF Leg MRI Axial T1

 

Congenital Tibial Pseudoarthosis

- normally 1 per 140 000

- 3% of NF1

- see article

 

Spinal Deformities / Kyphosis / Scoliosis

- 30% have spinal deformity

- dystrophic and non dystrophic

- recommend earlier fusion at curves > 35o

- higher incidence of pseudoarthrosis

- all require MRI prior as high incidence of intraspinal lesions

- see Paediatrics / Spine / Scoliosis / Other / Neurofibromatosis

 

 

 

 

Neuromuscular Conditions

Charcot Marie Tooth

Hereditary Motor Sensory Neuropathies I-III

 

Epidemiology

 

1: 3000

 

Definition

 

Group of inherited peripheral sensory & motor neuropathies

- includes Charcot Marie Tooth Disease

 

Classification

 

Hypertrophic vs Neuronal HMSN 

 

I   Hypertrophic CMT

II  Neuronal CMT

III Infantile Hypertrophic Neuropathy - Dejerine-Sottas

IV  Refsum's disease

 

Orthopaedic Complications

 

Cavovarus Foot

 

Claw Toe

 

Hip Dysplasia

 

10 % CMT

- muscle imbalance promotes femoral anteversion & valgus neck

- 2° acetabular dyplasia

 

Management

- as per usual

- NB care with sciatic nerve as more susceptible than normal

- usually bony surgery

 

Scoliosis 

 

50%

- management as per idiopathic

 

Hypertrophic CMT HSMN I

 

Definition

 

Demyelinating peripheral neuropathy

- repetitive demyelination & remyelination leads to nerve hypertrophy = Onion bulb

- more demyelinated fibres than myelinated

 

Characterised by

- demyelination

- areflexia

- decreased nerve conduction velocity

 

Aetiology

 

AD 

- triple dose of peripheral myelin protein 22 

- chromosomal linkage on chr 17 (17p11.2) 

- gene encodes periph myelin ptn 22

 

Histology 

 

Demyelination & fibrosis

- 'Onion-bulb' appearance

 

Clinical Features

 

Present in children / teens

 

Lower limbs more affected

 

Areflexia

 

Symmetrical distal muscle weakness and wasting

- weakness progresses to all muscles distal to knee

- develop high-stepping drop-foot gait

- inverted champagne bottle

 

Stork legs CMT Stork Legs CMT 2

 

Glove and stocking sensory loss

 

Cavo-varus

- initial mild cavus & toe clawing

- becomes rigid 

 

CMT Foot 1CMT Foot 2

 

Upper limb involvement 

- begins later 

- starts with intrinsic wasting

- mixed median and ulnar 

- then radially supplied muscles

 

CMT Hands 1CMT Hands 2

 

NCS

 

Motor nerve conduction velocities decreased by > 50%

 

Diagnosis

 

Nerve Biopsy

 

Genetic testing

- mutation in 17q11 or X-linked connexin 33

 

Neuronal CMT HSMN II

 

Differences

- axonal neuropathy without demyelination

- reflexes preserved 

- normal nerve conduction velocity

 

Aetiology

 

AD

- abnormality in spinal cord neurones

 

Histology 

- neuronal degeneration with preservation of myelin

 

Clinical Features

 

Present in teens / early adulthood

- less disabling than HSNM I

- upper limb involvement less severe than Type 1

 

Reflexes present 

 

Glove and stocking sensory loss only mild

 

More profound distal lower limb involvement than Type 1

- stork-leg appearance

- wasted distal thigh / leg muscles

- flail foot

- calcaneocavus deformity

 

CMT Stork LegsCMT Cavus

 

Investigations

 

Motor NCS normal

 

Infantile Hypertrophic Type III

 

Aetiology

 

Dejerine-Sottas disease

- AR (no normal gene for peripheral myelin ptn 22 present)

 

Pathology

 

Hypertrophic interstitial neuropathy

- histology shows segmental demyelination & marked nerve thickening with Perineural fibrosis

 

Clinical Features

 

Presents in infancy

- pes cavus & drop foot

- stocking-glove sensory loss

- significant spinal deformity

- confined to wheelchair by 30s

 

Investigations

 

Markedly slowed motor NCS

 

Refsum's disease Type IV

 

AR

- rare disease with Cranial Nerve involvement

 

 

Duchenne's

Aetiology

 

X linked recessive

Lack of dystrophin

 

Epidemiology

 

1:3500

Boys only

 

Decreased intellectual capacity / average IQ 80 - 90

 

Present

 

Delay in walking

- test CPK in any boy not walking by age 18 months

 

Toe walking

 

Parents have noticed waddling gait and stiff feet

 

Signs

 

Gower's 

- have trouble rising from floor

- rear in the air first

- use arms to walk up legs

 

Proximal muscle weakness

- symmetrical

 

Calf hypertrophy

- pseudohypertrophy

 

Toe walking

 

Lumbar lordosis

 

Investigation

 

Creatinine Phosphokinase / CPK

- normal < 200

- DMD > 5000

 

Muscle biopsy

- absent dystrophin protein

- definitive diagnosis

 

NHx

 

Loss of independent walking by 10 years

Rapid scoliosis progression by 14 years

Wheelchair bound by 15 years

Death in late teens (<20 years) due to respiratory infection

 

Management

 

Non operative Management

 

Physiotherapy / occupational therapy

 

Prevent contractures

- hip adduction / tendoachilles

 

AFO's

 

Steroids

 

Concept

- prolongs walking

- improves strength

- delays scoliosis

- improves respiratory function

 

Begin

- age 7 or 8

 

Complications

- AVN

- mood swings

- cataracts

- short stature

- Cushingoid features

 

Operative Management

 

Scoliosis

 

Bracing not effective / can compromise respiratory function

 

Need early posterior fusion when scoliosis 20o

 

Malignant Hyperthermia

 

All patients high risk

- must be screened preoperatively

- give prophylactic dantrolene

- avoid inhalational anaesthetics / suxamethonium

 

Contractures

 

SEML's

- hip adductors

- T achilles

- T posterior

 

 

 

 

 

Friedreich's Ataxia

Definition

 

Hereditary Spinal Cerebellar Ataxia

- degenerative

- onset by age 10

 

Epidemiology

 

AR

 

Pathology

 

Spinal cord shows atrophy of

- cerebellar tracts

- posterior columns

- dorsal root ganglia

- PNS

 

Clinical Features 

 

Cerebellar features

- ataxic gait

- dysarthria

- tremor

- nystagmus

- difficulty with fine motor hand control 

 

Neurological examination shows

- muscle weakness

- decreased vibration sense

- decreased proprioception

- absent DTR

- upgoing plantar response

- positive Rhomberg's Sign

 

May be unilateral at first but becomes bilateral

 

NHx

 

Remissions & stable periods seen

- progression usually slow

- usually confined to wheelchair by 30

 

NCS

 

Marked decreased sensory action potential

Slight decrease in motor conduction velocity

 

Medical Complications

 

Bulbar palsy / Cardiomyopathy

- either may cause death

- usually by age 40

 

Orthopaedic Complications

 

Pes Cavus

 

Scoliosis

 

Similar curve pattern to Idiopathic form

- manage similarly

 

 

 

 

Muscular Dystrophies

Types

 

Duchene

Becker

Myotonic

Spinal Muscular Atrophy

Juvenile Dermatomyositis

Guillian Barre

 

Becker Muscular Dystrophy

 

Similar to Duchene but milder

- mild proximal muscle weakness

- calf hypertrophy

 

Myotonic Dystrophy

 

Most common form of adult muscular dystrophy


Inheritance

 

AD

- chronic

- slowly progressive

- highly variable

- often associated with intellectual impairment

 

Clinical Features

 

Gradual onset of muscle weakness

- myotonia - difficulty grasp release

- expressionless face - typical

 

Check mother's grip

- difficulty with grasp release

 

Variable expression

- muscle weakness

- talipes, pes cavus and scoliosis

- dysphagia and constipation

- drop foot, high stepping gait

 

High anaesthetic risk

 

Investigation

 

CK Normal

 

EMG - pathognomonic pattern

 

DNA analysis

 

Spinal Muscular Atrophy

 

Anterior horn cell degeneration

 

Types

 

Type 1 

- die soon after birth from resp failure

 

Type 2 

- normal until 6 months

- never learn to walk independently

 

Type 3 

- milder

- normal milestones for first year

- present similar to Duchene

- flat footed (compared with Duchenne which tip toe walk)

- reflexes are depressed

 

Signs

 

Proximal > distal weakness

- suppressed reflexes

- extremely floppy

 

Develop

- respiratory compromise

- scoliosis

- flexion contractures

 

Investigations

 

CK Normal

DNA testing

 

Juvenile Dermatomyositis

 

Onset insidious age 2 onward

- miserable weak child 

- butterfly facial rash

- toe walk

- violaceous rash

 

Management

 

Steroids and immunosuppressives

 

Guillain Barre

 

Demyelinating polyneuropathy

- common in childhood

- may be life-threatening

 

20% present with waddling gait like Duchene

- most present with distal > proximal weakness 

- minimal sensory findings

 

Management

 

Immunoglobulin

 

 

 

Osteogenesis Imperfecta

Defect

 

Abnormality of type 1 collagen

- amino acid substitution of glycine with another amino acid

- prevents triple helix formation

 

Many many deformities described

- some 286 mutations of Type 1 collagen described

 

Sillence Classification

 

There are actually now 7 

 

Type I

- mild

- AD

- blue sclera

 

Type II

- lethal in utero

- AR

- blue sclera

 

Type III

- severe

- AR

- white sclera

- multiple deformities without intervention

- wheelchair bound and non ambulatory

 

Type IV

- moderate to severe

- white sclera

- AD

- very rare

 

DDx

 

NAI

Rickets

 

Diagnosis

 

Skin biopsy

- assessment of type 1 collagen

- fibroblast cell culture

 

DNA study

 

Skeletal Manifestations

 

Frequent multilevel fractures with limb deformity

- tibia

- femur

- forearm

 

Osteogenesis Imperfecta ForearmOsteogenesis Imperfecta Wrist

 

Osteogenesis Tibial BowOsteogenesis Imperfecta Tibial Bow 2

 

OI ForearmOI Shoulder

 

Coxa vara

- femoral neck shaft < 110o

- correct with multiple osteotomies

 

Kyphoscoliosis

 

Surgery

- indicated for curve reaching 40o as will be progressive and non flexible

- > 60o impairs respiratory function

 

Problems

- bony fragility

- increased use of pedicle and TP hooks and sublaminar wires

 

Basilar impression / invagination

 

Very serious problem

- the foramen magnum invaginating into the posterior fossa

- leads to stenosis with resultant hydrocephalus 

- compression of the cerebellum, brain stem, and cervical cord

 

Lateral cervical spine radiograph

- upward migration of the cervical spine into the base of the skull

- deformity may be subtle and requires careful scrutiny of the radiographs

 

Various descriptions of the characteristic skull shape

- Darth Vader

 

Extraskeletal Manifestations

 

Blue sclera 

- due to translucent sclera can see underlying choroid and blood vessels

- due to defect type 1 collagen

 

Dentinogenesia imperfecta 

- teeth appear brownish or bluish

- are soft, translucent and prone to cavities

- defect type 1 collagen

 

Hearing problems

- defect hearing bones

 

Fragile skin and fragile capillaries

 

Valvular Disease

 

Mitral prolapse and Aortic Regurgitation

 

Management

 

Nonoperative Management

 

Bisphosphonates

 

Increase BMD and cortical thickness and cancellous trabeculation

- reduces bone pain

- decrease fracture rate

- maintains shape of long bones and vertebrae

 

Mechanism of action

- inhibits osteoclasts

 

Pharmocokinetics

- very poor bioavailability (.6 - 7)

- incorporated into skeleton

- long half life there (1.5 - 10 years)

 

Problems

- need period off medications

- doesn't decrease fracture healing but probably does decrease osteotomy healing (i.e. avoid around time of IM rodding)

- osteonecrosis of the jaw: a rare side effect in adults, not seen in children

 

Results

 

Glorieux et al NEJM 1998

- increased BMD by 40%

- reduced fracture rate by 1.7 per year

- evidence new bone formation in vertebrae

- did not alter rate fracture healing

 

Bone marrow transplant

 

Increased mesenchymal stem cells

 

Operative Management

 

Goals

 

Prevent fractures

Treat or prevent deformity

 

Technique

 

Multilevel osteotomy with IMN

- must be expandable or get fracture and deformity below level

 

Expandable telescopic IM rods

 

Concept

- lengthen as the bone grows

- male and female portions

- lock in epiphysis top and bottom

 

Sheffield Rods

- T piece

- 20% revision at 5 years

 

Fassier-Duval Rods

- epiphyseal portion is threaded

 

 

 

Osteopetrosis

DefinitionPelvis Osteopetrosis

 

Inheritable bone disorder characterised by defective osteoclasts

- resulting in hard and brittle bone

 

Pathophysiology

 

Defect of osteoclasts

- unable to acidfy Howship's lacunae

- unable to resorb bone

- missing ruffled border

 

Heterogenous group

- 60% defect of proton pump

- 5% carbonic anhydrase defect

 

Inability to remodel bone with thickened sclerotic cortex

- impinges on medulla

- woven bone architecture

- thickened cancellous trabeculae

 

Unresorbed calcified cartilage

- prone to fracture

 

Forms

 

1.  Malignant AR

 

Few survive first decade

- bony overgrowth of medulla

- blocks hematopoiesis

- anaemia/neutropenia/thrombocytopenia

- need bone marrow transplant to survive

 

Cranial nerve compression

- overgrowth foramina

- optic, auditory, facial n

 

Fracture

 

2.  Intermediate AR

 

Moderate anaemia

Some nerve compression symptoms

Frequent fractures in first decade

 

3.  Benign AD 

 

Most common

- diagnosed after fracture

- 40% asymptomatic

- due to variable penetrance

 

Clinical Features

 

1.  Frequent long bone fractures

- femur and tibia

 

2.  Long bone deformity

- callus slow to organise

- lateral bowing of femur

 

3.  Hip

- coxa vara

 

4.  OA

- common in hip

- compression of cartilage on hard bone

 

Hip Osteopetrosis OA

 

5.  Spondylosis

 

6.  Osteomylelitis

- 10 % patients

- most often in mandible

- lack of marrow vascularity

- impairment of white cell function

 

7.  Cranial nerve compression

- 20-25%

- optic, auditory, trigeminal, facial

 

X-ray

 

Generalised sclerosis

- subchondral sclerosis in pelvis

 

Endobone

- bone within bone

- failed resorption of primary spongiosa

 

Vertebral end plate thickening

- rugger jersey vertebrae

 

Erlenmeyer flask deformity

- widened club like metaphysis

- in children

 

Transverse banding of metaphyses

 

Management

 

Non operative

 

1.  Interferon gamma

 

Mechanism

- increases bone resorption

- improves hematopoiesis and leucocyte function

 

2.  High dose vitamin D / diet low in calcium

 

Mechanism

- stimulates osteoclasts

 

3.  Bone marrow transplant

 

Indication

- children with malignant form

 

Operative Management

 

Fractures

 

Attempt to treat non operatively if possible

- ORIF difficult

- nil medullary canal

- break drill bits

 

ORIF

 

Plates often fail

- IM load sharing devices best

 

Increased time to union

 

Difficulty drilling, inserting screws and pins

- drills over heat and break

- advance 1cm at a time

- frequent cleaning and cooling

- alternate drill bits

 

Longer surgical time

 

Arthroplasty

 

Technical difficulties

 

Unable to use hand reamers

- recreate medullary canal

- burrs needed

- use II to check

- short narrow stems

- can consider resurfacing

 

Complications

 

Increased risk intra-operative fracture

 

Increased risk infection

 

 

 

 

 

Sprengel Shoulder

Definition

 

Congenital elevation of shoulder

 

Epidemiology

 

AD & Sporadic 

 

Tends to be in girls & on left

- like CDH

 

Associated with other congenital abnormalities

 

Aetiology

 

Failure of descent of arm bud

- arm bud appears in week 3 (level of C5 to T1)

- scapula develops in arm bud in week 5 (Opposite C5)

- descends over next 3/12

 

Usually by 3rd fetal month to level of T2 to T7

 

Clinical Features

 

Scapula small 

- scapula elevated

- superior angle rotated upwards & forwards

- shoulder musculature deficient

 

Limited shoulder abduction

- scapula joined to cervical spine by fibrous or bony bar

- Omovertebral bar

 

Usually presents as Neonate, but if mild presents later 

 

Associated Abnormalities

 

Scoliosis

Diastematomyelia 

Klippel-Feil Syndrome (usually bilateral)

Cervical ribs

Fused or absent Thoracic ribs

Thoracic Vertebral anomalies

Hypoplastic Humerus or Clavicle

 

X-ray 

 

Small high scapula

 

Omovertebral bone

- can see bone linking scapula and hyoid

 

Bilateral abduction xray reveals lack of ST motion

 

Operative Management

 

Indications

 

Cosmesis

Attempt to improve abduction range

 

Complications

 

Hypertrophic scar due to high strain on scar

Brachial Plexus Injury

 

Options

 

1.  Scapula Resection

 

Simplest procedures

- only cosmetic

- excision of prominent angle (excise scapula above spine)

- excision of Omovertebral bar

 

2.  Woodward Procedure

 

Concept

- principle concern is brachial plexus palsy

- best outcome in children 3 - 8

 

Technique

 

A.  Midline incision

- clavicular osteotomy to protect brachial plexus

- excise omovertebral bar

 

B.  Mobilise scapula caudally

 

C.  Detach Trapezius & Rhomboids from spinous process insertion

- reattach to supraspinous ligaments more inferiorly

- dynamic force inferiorly on scapula

 

3.  Green Procedure

 

Osteotomy clavicle first

- avoiding plexus injury

 

Scapula release

- released from medial border of scapula

- reattach after scapula reduced

 

 

Wormian Bones

Definition

 

Intra-sutural ossicles in lambdoidal, posterior sagittal and tempero-squamous sutures

- normal up to 6 months of age

- multiple areas of ossification skull cranial sutures

- early fusion can give papilloedema

 

Cremmin et al Skeletal Radiology 1982 8: 35-38

- size of greater than 6mm by 4mm

- mosaic rather than linear pattern

- more than 10 in number

 

DDx / Cretin PORK CHOPS

 

Cretanism

 

Pyknodysostosis

Osteogenesis Imperfecta

Rickets in healing phase

Kinky hair syndrome of Menke

 

Cliedocranial Dysplasia

Hypophosphatasia (also hypothyroidism)

Otopalatodigital syndrome

Primary Acroosteolysis, Pachydermoperiostosis

Syndromes and Chromosome disorder; esp 21

 

 

 

 

 

 

 

 

 

Osteochondroses

General

Definition

 

Involvement epiphysis or apophysis of immature skeleton

- heterogenous group of eponymous conditions

 

- usually occurs at time of greatest growth

 

Xray

 

Features of

- sclerosis

- fragmentation

- decrease in size of ossification centre

 

NHx

 

Some progress to OA

Some are self-healing

 

Pathology

 

Affected portion of bone has features of osteonecrosis

 

Thought to be due to recurrent minor trauma & avascularity

 

Three types

 

Crushing

 

Types

- Kohler's (4 year old boys)

- Freiberg's  (11 year old girls)

- Panner's (young child or teens)

- Kienbock's (20-30 years)

 

Pathology

 

Spontaneous necrosis of 

- ossific nucleus of long bone

- cuboidal bone of wrist or foot

 

Pathological changes of AVN

- bone death

- fragmentation

- collapse

- new bone formation

 

Pulling

 

Types

- Osgood-Schlatter

- Sever's

- Sinding-Larsen-Johansson

- Menalaus-Batten

 

Aetiology

 

Excessive traction by large tendon to unfused apophysis

- repetitive microtrauma

- no bone necrosis 

- apophysis becomes painful

- may be tenosynovitis

 

Splitting

 

OCD of Knee / Ankle / Hip

 

Uncertain aetiology but thought to be combination of microtrauma & hypovascularity

 

 

 

 

 

Kohler's Disease

DefinitionKohler's Disease

 

Osteonecrosis of the navicular

 

Epidemiology

 

Present at age 4-6 years

- same as Perthes disease

 

M:F 5:1

 

Bilateral in 20%

 

Aetiology

 

Repetitive trauma to maturing ossific nucleus

- avascular necrosis

 

Clinical Features

 

Pain & soft tissue swelling over navicular

 

X-ray

 

Flattening & sclerosis of navicular

 

NHx

- usually resolves within 18 - 36 months

 

Management

 

Aim to provide symptomatic relief

- rest from sport

- analgesia

- SL cast for 6-8 weeks if required

 

 

 

Panner's Disease

Definition

 

Crushing AVN of capitellum

- 4 - 10 years

- maximum 14 years

- usually boys, baseball pitchers

 

Aetiology

 

Valgus compression

- i.e. baseball pitchers

 

Two forms

 

1.  True Panner's / Crushing Osteochondritis

- young patient

 

2. OCD form in older boys

- older than 10

- loose bodies

- locking / catching symptoms

 

Clinical Features

 

Pain with throwing

- tender lateral aspect elbow

- FFD or lack of flexion

 

Xray

 

Panner's

- epiphysis condensed & fragmented

 

OCD

- localised area of AVN

 

MRI

 

Useful in grading OCD

 

Treatment

 

Panners

- usually reconstitutes without disability 

- immobilise 4 weeks, NSAIDS

- avoid throwing for period

- gradual strengthening and return

- good prognosis

 

OCD

- grade and treat appropriately

- pin in situ if able

- remove loose bodies / microfracture

- arthroscopy

- prognosis much worse especially in competitive athlete

 

See Elbow / Capitellar OCD

 

 

 

Sever's Disease

Definition

 

Calcaneal apophysitis / Pulling osteochondrosis

 

Epidemiology

 

Young boys

- peak age 10

- bilateral in 60%

 

Aetiology

 

Overuse injury of calcaneal apophysis

Repeated microfracture

 

History

 

Mechanical heel pain

Worse if barefoot

 

Examination

 

No redness or swelling

Tenderness on sides of tendon insertion

Restricted ankle dorsiflexion

 

Xray

 

Controversial whether normal

- increased fragmentation & density of apophysis

- may be the same on the other side

 

Primarily performed to exclude other conditions

- giant cell tumours

- UBC

 

Management

 

Algorithm

 

Restrict activities for 1-3 months

 

NSAIDs

- oral

- topical

 

Physio

- T achilles stretching / dorsiflexion exercises

 

Orthotics

- heel raises

- custom made heel cups & insoles

- short leg cast for 4/52 if symptoms severe

 

 

 

 

Rickets

Definition

 

Rickets

- paediatric disorder characterised by deformity and growth retardation

- secondary to defective mineralisation of the growth plate

 

Osteomalacia

- adult disorder

- defective mineralisation of osteoid

 

Physiology

 

Generation of Vit D

 

Vitamin D3 ingested and absorbed

- activated by sunlight

- need 10 - 15 minutes 3 x per week

 

25 hydroxylated in liver

 

1 alpha-hydroxylase in kidney

- active 1,25 vit D3

- calcitriol

- this is under PTH control

 

Action Vit D

 

Intestine

- increases Ca and PO4 absorption

 

Kidney

- decreases Ca and PO4 excretion

 

Bone

- increases Ca and PO4 release

 

Pathology

 

Physis

- hypertrophic zone widens

- provisional zone never forms

- no mineralisation of osteoid

 

Clinical

 

Osteopenia

 

Bowing

- usually develops during periods of rapid growth

- not seen in newborn

- see in toddlers

 

Fractures

- recurrent clavicle fractures

- stress fractures

 

Teeth

- delayed formation

- increased dental caries

 

Kyphoscoliosis

 

Short stature

- adults < 5 foot

 

Xray

 

Tibial and femoral bowing

 

Pathognomonic signs

- widened physeal plate

- cupping of the metaphysis

 

Blood Results

 

Ca2+ low

Phosphorus low

Alk Phos high

Vit D low

Electrolytes

 

Causes

 

1.  Vitamin D deficiency

 

Causes

- inadequate sun exposure

- low dietary intake

 

More common in dark skinned children

- exclusively breastfed

- live in northern cities with low sunlight

- vegetarian diet

 

Treatment

- daily vitamin D

- 30 minutes daily sunshine

 

2.  Congenital disease

 

A.  Vitamin D dependent rickets Type 1

- defect in 1 alpha hydroxylase

- converts 25 (OH) to 1,25 (OH)

- 1,25 biologically active

 

B.  Vitamin D independent rickets Type 2

- mutations in the vitamin D receptor

- more than 10 types

 

C. Familial X linked hypophosphataemic rickets

- AD, most common form

- vitamin D resistant rickets

- impaired tubular resorption phosphate

- treatment phosphate + calcitriol

 

3.  Vitamin D metabolism abnormalities

 

Causes

- renal failure

- phenytoin

 

 

Rotational Profile

External Tibial Torsion

Epidemiology

 

Presents in late childhood

 

Often unilateral

 

More often right side

 

NHx

 

Tends to increase with age

- rarely a problem until late childhood

- associated with PF instability & pain

 

Aetiology

 

Spina bifida / CP

 

Torsional Malalignment Syndrome

 

Miserable Malalignment Syndrome

- IFT with compensatory ETT

 

Management

 

Non-operative Management

 

Useless

- rarely indicated on functional grounds

- lever action of foot is not lost until the FPA > 60°

 

Operative Management

 

Indications

- osteotomy indicated if TFA > 40°

- certain cases of CTEV & NMD 

 

 

 

 

 

General Principles

Aetiology

 

1. Physiological

 

2. Non Physiological

- congenital

- acquired

 

Definition

 

Version

- normal rotation

 

Torsion

- rotation 2 SD <> mean

 

Femoral Version

- angular difference between transcervical & the transcondylar axis

 

Tibial Version

- angular difference between transcondylar axis of knee & transmalleolar axis

 

Normal Development

 

Lower limb bud develop during 4/52

- great toe points lateral

- during 7th week bud IR 

- brings hallux into midline

 

Limb continues to ER through intra-uterine and childhood

- femoral anteversion decreases

- tibial ER increases

 

Neonates have ER hip contracture that masks the femoral anteversion 

 

Natural History

 

In-toeing toddlers become outoeing adults

 

Femoral anteversion decreases with age

- 40° neonate

- 15° adult

 

Tibial ER increases with age

- 5° neonate

- 15° adult

 

Classification

 

Toeing In

- Internal Femoral Torsion IFT / Femoral anteversion FAV

- Internal Tibial Torsion ITT

- Metatarsus Adductus 

 

Toeing Out

-  Physiological

-  External Tibial Torsion ETT

-  Pronation / Abduction of the Feet

 

DDx

 

Intoeing

- increased FAV

- CP

- genu varum

- metatarsus adductus (residual CTEV)

 

Out-Toeing

 

SUFE

ETT

 

Presentations by Age

 

1st Year of Life

- intoeing usually metatarsus adductus

 

2nd Year of Life

- intoeing usually FAV

 

After 3rd Year of Life

- bilaterally intoeing FAV

- unilaterally intoeing ITT

- unilateraly outoeing ETT

 

Issues

 

1.  Is it in femur?

2.  Is it in tibia?

3.  Is it in foot?

 

History

 

Age of onset

Severity

Disability - i.e. clumsiness

Age first walked

Previous management

 

If walking age delayed consider CP

- FHx of In / Out Toeing

- sitting W position

 

Examination

 

Alignment

- genu varum

 

Gait

- squinting patella (increased FAV)

- patella anterior (ITT)

- out toeing

 

Spine / scoliosis

 

Staheli's Torsional Profile (6)

 

1.  Foot Progression Angle 

- assessed on gait

- usually 10° out (0°-30°) 

 

2.  Hip IR

- child prone

- usually < 65° 

- > 70° = FAV

 

3. Hip ER 

- usually 40° (20-60°)

- greater in young child

- note IR + ER should = 90°

 

4. Thigh- Foot Angle TFA

- child prone & knees flexed

- reconstruct foot

- usually 15° (0°-30°) ER

- measures tibial torsion

 

5. Transmalleolar Axis (TMA)

- prone & knees flexed

- usually 0 - 30° ER

 

6. Foot

- shape of foot

- Metatarsus adductus / everted foot affects foot progression angle

 

Xray

 

AP Pelvis

- acetabular version

- SUFE

 

Malligan Technique

- AP & lateral hip allows calculation of version 

- using tables by Magilligan

- converts measurements of neck length into an FAV angle

 

CT scan

 

Direct measurement of femoral & tibial version

 

Management

 

General Principles

 

1. Trying to control the sleeping, walking, or sitting of infants & children is impossible

 

2. Splints are of no benefit & interfere with child

 

3 Observational Management successful >99%, only 1 in 1000 need OT

 

4. OT correction effective but carries significant risk

 

5. OT only justified in the child with severe deformity that has failed to resolve with time 

- ITT < -10° 

- ETT > 40° 

- FAV > 50°

 

6. At least > 8 years old prior OT

 

 

 

 

 

 

Internal Femoral Torsion

Definition

 

Transverse plane rotation of the femoral neck axis anteriorly relative to the transcondylar axis

 

Epidemiology

 

F:M = 2:1

Bilateral, symmetrical

 

Aetiology 

 

Unknown

Familial

 

NHx 

 

Increases up to age of 5

- resolves by age of 8 

 

Resolves in 95%

- compensatory ETT may develop after 5 years

- little functional disability

 

>50% of patients with persistent femoral anteversion achieve normal gait

 

Doesn't predispose to OA

 

Presentation

 

Intoeing in early childhood

- starts 3 years old

- maximum 4-6 years old

 

Examination

 

Squinting patellae

 

Sit in W 

 

W sitting

 

Run like egg beaters & trip over

 

Prone Rotation test / Gages Trochanteric Angle

- find position where GT most prominent laterally

- angle tibia makes from vertical is FAV

 

Increased IR

- abnormal if IR > 70° 

- if severe, no ER possible 

 

CT scan

 

If surgical intervention deemed likely

 

DDx

 

If unilateral / progressive rule out DDH / CP

 

Non operative

 

No evidence for orthoses

 

Operative

 

Indications

 

Very severe functional gait disturbance

Cosmesis

 

Age > 10

 

Rotational criteria

- IR > 85° & ER <10° 

- measured Anteversion > 50°

 

Principles

 

Leave until at least > 8-10 years old because many resolve

Not required if 10° ER present

 

Subtrochanteric derotation osteotomy

 

Proximal femoral plate

Aim IR = ER on table

 

 

 

 

 

Internal Tibial Torsion

Definition

 

Angular difference between transcondylar axis of knee & transmalleolar axis

 

Aetiology

 

Though to be a packaging disorder

 

Epidemiology

 

Number 1 cause of intoeing in 2 year odl

- does not occur in preterm infants 

 

10% < 2 years ITT 

- 2/3 bilateral 

- 1/3 unilateral of these usually left side

 

NHx 

 

Normal 0 - 30o ER

 

Most cases of ITT resolve by 2 years of age

- TMA increases 0-5° from age 1 to 2 years

- few resolve > 8 years

- resolution not universal

- positive FHx association with poor prognosis

 

Associations

 

Associated with MT Adductus in 1/3

NMD / CP

 

DDx

 

NMD

Fracture

Tibia vara

 

Symptoms

 

Children tend to trip & appear clumsy

 

Examination

 

Patella normal position

- in-toeing

- thigh foot angle < 0o

- TMA < 0o

 

Management

 

Non-operative Management

 

Splints shown not to work

 

Operative Management

 

Complication rate of proximal tibial osteotomy very high

 

Supramalleolar Osteotomy 

 

Indications

1. TMA > 3 SD (< -10° or > 40°)

2. Age 10+ years

3. Severe disability

 

Technique

- anterolateral approach

- DCP plate

 

 

 

Skeletal Dysplasia

A Background

Definition Short Stature

 

< 1.25 m in adult

 

Types

 

1.  Proportionate dysplasia

- mucopolysaccharidoses

- Morquio's, Hurler's, Hunter's

 

2.  Dysproportionate dysplasia

- short trunk / SED

- short limb / achondroplasia & MED

 

Proportionate dysplasia / Symmetrical decrease in limb and trunk length 

 

Mucopolysaccharidoses

- Morquio's, Hurler's, Hunters

 

Cleidocranial Dysplasia 

Severe OI

Severe Osteopetrosis

Hypophosphatasia

Hypophosphataemia

 

Dysproportionate dysplasia

 

A.  Short trunk 

- SED

- Kniest's syndrome

- Metatropic dysplasia

 

B.  Short limb

- Achondroplasia / Pseudoachondroplasia

- MED

- Diastrophic dysplasia

 

Rhizomelic - proximal

Mesomelic - middle

Acromelic - distal

 

Specific Conditions

 

1.  Achondroplasia / Pseudochondroplasia

 

Dysproportionate dwarfism

- short limbs, normal trunk

 

Characteristics

- coxa vara, genu varum

- radial and tibial bowing

- radial head dislocation

- metaphyseal flaring with inverted V shaped distal femoral physis

- Square iliac wings

 

Problems

- spinal problems i.e. stenosis

- coxa vara / genu varum

 

2.  MED

 

Dysproportionate dwarfism

- short limbs, normal trunk

 

Characteristics

- irregular delayed ossification at multiple epiphyses

 

Problems

- coxa vara, genu valgum

 

3.  SED

 

Dysproportionate dwarfism

- short trunk

 

Characteristics

- similar to MED but involves spine

 

Problems

- coxa vara / genu valgum

- spine (kyphoscoliosis / platyspondyly / ondontoid hypoplasia /atlanto-axial instability)

 

4. Metaphyseal chondrodysplasia

 

Characteristics

- metaphyseal changes with normal epiphyses

- can look like rickets

 

Problems

- coxa vara / genu valgum

 

5.  Progressive Diaphyseal Dysplasia 

 

Engelmann Disease

 

Characteristics

- diffuse sclerotic thickening of cortex

 

6.  Dysplasia Epiphysealis Hemimelica 

 

Trevor's Disease

 

Characteristics

- epiphyseal osteochondroma

- affects one joint only

- often ankle

- often medial

 

7.  Mucopolysaccaridoses

 

Proportionate dwarfism with complex sugars in urine

 

Morquio's syndrome

- AR

- presents 18 months to 2 years with waddling gait

- keratan sulphate in urine

- normal intelligence

 

Hurler's

 

Hunter's

 

8.  Dysplasias associated with Benign Bone growths

 

Diaphyseal aclasia / multiple osteochondromas

Fibrous dysplasia

Ollier's

Maffucci's

 

Achondroplasia

Definition

 

Dysproportionate dwarfism

- short limbs and normal trunk

- rhizomelic

 

DDx

- physeal dysplasia (SED, MED)

 

Aetiology

 

FGF Receptor 3 

- point mutation

- decreases endochondral ossification

- normal intramembraneous ossification

- FGFR3 is found in all pre-bone cartilage & in CNS

- FGFR3 inhibits chondrocyte proliferation in the proliferative zone

- appears that in achondroplasia the receptor is overactive & inhibits the proliferative zone  

 

Inheritance

 

AD

- 85% from spontaneous mutation

 

Pathology

 

Defect in enchondral bone formation / Proliferative zone

- dalteration in normal chondrocyte maturation, hypertrophy & degeneration

- abnormal clustering of chondrocytes

 

Hypertrophic Zone 

- narrow & irregular cells of differing sizes

 

Clinical Presentation

 

At birth 

- short limbs & normal trunk

 

Lower Limbs

 

Tibia

- bowing

 

Knees

 

Genu varum 

- long fibula

- may need fibula epiphysiodesis

 

Inverted V shaped distal femoral epiphysis

- flared metaphysis with ball in socket epiphyseal/metaphyseal junction

 

Hips

 

FFD hips + increased lumbar lordosis

 

Coxa vara

- short femoral necks

- horizontal acetabular roof

 

Pelvis

 

Square iliac bones 

- " elephant ears"

- narrow siatic notch

 

Champagne-glass pelvic cavity

- the pelvis is wider than it is deep

 

Spine

 

Thoraco-lumbar

 

Achondroplasia Decreased Interpedicular DistanceAchondroplasia Increased Lumbar Lordosis Platyspondyly

 

Increased lumbar lordosis

 

Non-rigid TL kyphosis 

- usually resolves with ambulation

- due to hypotonia

 

Spinal canal stenosis

- decreased inter-pedicular distance 

- narrows from L1  down (normally increases)

- short pedicles

 

Platyspondyly

- bullet shape vertebrae 

- anterior inferior body beak T12- L2

- posterior scalloping of vertebral bodies

 

Achondroplasia MRI Spinal Stenosis.jpgAchondroplasia Short Pedicles

 

Cervical

 

Foramen magnum stenosis

- responsible for higher mortality in infants

- may have symptoms myelopathy

- often presents with apnea / snoring

- can cause sudden death

- MRI / sleep studies

 

Upper Limbs

 

Hand

 

Trident hand in 50%

- persistant space between middle & ring in extension

 

Stubby tubular bones 

- normal bone girth

 

Fingertips only reach to hips

- difficulty with hygiene 

 

Humerus

- posterior bowing / limitation of extension

 

Elbow

- cubitus varus

 

Forearm

- bowed ulna

- radial head dislocation

 

Head

 

Large skull with frontal bossing

 

Operative Management

 

Spinal Stenosis

 

Present later in life

- 50% or more of patients

- require multilevel laminectomy +/- fusion

 

Chondrodysplasia Punctata

 

Characteristics

 

Multiple punctate calcifications of dysplastic epiphyses 

 

Cause

 

Enzymatic defect in peroxisomes

- causes calcification

 

Types

 

Conradi-Hunnerman

 

Asymmetric limb shortening

- Vertebral anomalies & Scoliosis

- Epiphyseal stippling

 

Stippling gone by age  4 yrs

 

Long bones, pelvis & vertebrae

- Epiphyses may remain irregular like Epiphyseal Dysplasia

- Skull normal

 

Sheffield Form

 

Milder form 

 

Autosomal Recessive Form 

 

Lethal by age 1

 

 

 

Diaphyseal Dysplasia

Engelmann's Disease

 

Progressive diaphyseal dysplasia

- marked thickening of cortices of long bones

 

Present

 

Young child with gait disturbance and limb pain

 

Clinical

 

Bilateral symmetrical diaphyseal sclerosis of long bones

- Affects endosteal & periosteal new bone

- Metaphysis & epiphysis are normal

 

Skull base sclerotic

- can get blindness and deafness

 

 

 

Diastrophic Dysplasia

 

Diastrophic Dwarfism

- twisted dwarf

- disproportionate dwarfism

- very short limbs

- rhizomelic

- AR

 

Clinical Features

 

Skull

- Superimposed calcified pinnae

- swollen cauliflower ears

 

Spine

 

Cervical Kyphosis

- May be vertebral wedging

- may need immediate surgery

 

Thoracic Kyphoscoliosis 

- Progressive structural curve

 

Lumbar lordosis - 2° to flexion contractures

 

Interpedicular narrowing from L1 to L5 

 

Spina bifida occulta

 

Long Bones

 

Short & broad long bones

- Flaring of metaphyses

- Flattened & irregular epiphyses

 

Shortened ulna/ fibula

 

Hip

- Delayed appearance of capital femoral epiphyses

- Central saucer-shaped defect in capital femoral epiphysis

- Hip dislocation common

 

Hitchhiker thumb

- abducted over a short MC

 

Severe joint contractures 

- hip and knee requiring release

- Subluxed or dislocated Patella

 

CTEV

- hitchhiker great toe

 

Cleft palate

 

MED - Multiple Epiphyseal Dysplasia

Definition 

 

Disturbance of endochrondral ossification of epiphyses of tubular bones

- preservation of vertebrae (c.f. SED)

 

Characteristics

 

Dysproportionate dwarfism with normal trunk

- not as severe as achondroplasia

- usually 145 - 170 cm tall

 

Characterised by small flattened irregular epiphyses

- symmetrical

- leads to OA by 3-4th decade

 

Types

 

Limb length may be affected, not as severe as achondroplasia (usually 145 - 170cm tall)

 

Spectrum of involvement

 

Ribbing 

- mild form

- affect only the hips

 

Fairbank 

- more severe

- affect the epiphyses of all long bones

 

Aetiology

 

AD

- variable expression

- affects collagen IX / COMP

 

Pathology

 

Disturbance of enchondral ossification in epiphysis & physis 

- deficient proliferative region of physis

- rongue-like projections of cartilage into metaphysis

 

Radiology

 

Epiphyseal ossification

- delayed / smaller

- appear fragmented

- symmetrical involvement

 

Joint surfaces flattened & irregular

- Leads to premature osteoarthritis

 

Vertebral ring epiphysis maybe affected, only mildly

 

Clinical Feature

 

Varies in severity

- polyarticular or pauciarticular

- Some cases only couple of joints involved, others condition is widespread

 

Most commonly affects 

- hips / knees / ankles / wrists

 

Spine, skull & face - normal 

 

Mild shortness of stature

 

Normal intelligence

 

NHx

 

Present with stunted growth or joint pain  and progressive deformity

- problems walking in childhood

 

Premature OA in adulthood

 

Hip

 

Most frequently & severely involved

- Coxa vara

- small irregular & fragmented capital epiphyses

- progressive flattening & extrusion of  head

- poor head coverage

 

MEDMED Late

 

Premature OA common

 

Management

- valgus intertrochanteric osteotomy +/- shelf

 

DDx

 

1.  Perthe's

- asymmetrical

- has metaphyseal cysts

- MED affects acetabulum

 

2.  Cretinism

 

3.  Pseudoachondroplasia

 

4.  SED

 

Knee

 

Femoral condyles flattened & rectangular

Slanted tibial physis

Genu valgum common

 

Hands

 

Short stunted metacarpals

Digits shortened

 

 

 

 

Metaphyseal Chondrodysplasia

Type

 

Short limbed dwarfism, normal trunk and spine

 

Characteristics

 

Metaphyseal changes of tubular bones

- Normal epiphyses

- metaphysis adjacent to physis very broad and mildly scalloped

- resembles healing rickets

 

Pathology

 

Defect in proliferative and hypertrophic zones of physis

 

Types:

 

Jansens (Rare)

- most severe

- defect PTHrP (AD)

- extensive calcification

- retarded

- very short

- striking bulbous metaphyseal expansion long bones

 

Schmid's

- more common, less severe

- present later (AD)

- defect type X collagen

 

McKusick

- associated immune defects

 

Clinically

 

Hips

- coxa vara

 

Knees

- genu valgum

 

DDx

 

Rickets

- normal blood tests

 

Metaphyseal Dysplasia

AKA Pyle's Disease

 

No dwarfism (c.f. chondrodysplasia)

 

AR

 

Clinical Features

 

Genu valgum

 

X-ray

 

Sclerosing bone deformity

- Usually incidental finding on XR

 

Erlenmeyer Flask Deformity

 

DDx

- Gauchers

- metaphyseal chondrodysplasia

 

 

Nail Patella Syndrome

Background

 

Relatively common

 

Autosomal Dominant

 

Clinical Features

 

Hypoplastic / dystrophic nails

 

Absent or hypoplastic patellae

 

Radial head subluxed laterally/dislocated

 

Horns on post/lat aspect of iliac blades

 

Club Feet

 

Scoliosis

 

Other Associated Problems

 

Proteinuria (50%) and renal failure (5%)

Glaucoma

 

 

 

 

Spondyloepiphyseal Dysplasia

Type

 

Disproportionate dwarfism with a short trunk

 

Problems with dwarfism, spine, vision and hearing

 

Definition

 

Primary involvement of vertebrae & epiphyseal centres of limbs

 

Resulting short-trunk dwarfism

- Limb are short, but trunk is even shorter

 

(cf Metaphyseal Chondrodysplasia - Short limbed Dwarf)

 

Types

 

Congenita

- AD, usually spontaneous mutation

- more severe, early

 

Tarda

- X linked recessive

- late presentation

- spine and hips

 

Pathology

 

Defect Type II Collagen Gene

- Disorder of growth plate

- Abnormality of Proliferative zone - Microcystic areas

- Surrounded by ring of abnormal chondrocytes

 

Clinical Features

 

Short limbs

- Rhizomelic - short proximal segment

- Normal hands & legs

 

Shorter trunk 

- Neck almost absent

 

Hip FFD

- Increases lumbar lordosis

 

Genu valgum

 

Club foot

 

Adolescent kyphoscoliosis

 

Also

- Cleft palate

- Pectus carinatum & barrel-shaped chest

- Protruberant abdomen

 

DDx 

 

Morquio's disease

- But shortening in distal limb segments (acromelic)

- Urinalysis = Keratan Sulphate excretion

- hands and feet severely affected

 

Achondroplasia

- normal trunk

- abnormal face

- nil platyspondyly

- rhizomelia - short long bones

 

Medical Complications

 

Eye problems common

- Myopia

- Retinal detachment

 

Radiology

 

Epiphyses appear late (like MED)

 

Spine

 

Platyspondyly 

- Flatten or oval vertebral bodies

 

Schmorl's nodes - indentations of end plates

 

Irregularity of the vertebral bodies ring epiphyses

 

Odontoid hypoplasia

 

Thoracic kyphoscoliosis

- Usually progresses and should be braced early, may need fusion

 

Lumbar lordosis 

 

Neck

 

Odontoid hypoplasia 

- atlantoaxial subluxation

- Cord compression result in cervical myelopathy

 

Treat with posterior atlantoaxial or occipitoaxial fusion

 

Hip

 

Delayed ossification of capital femoral epiphysis

- Coxa vara - valgising IT osteotomy

- Varus may progress to discontinuity of femoral neck

- May be progressive dislocation

 

 

 

Trevor's Disease

 

Dysplasia Epiphysealis Hemimelica

 

So called because affects half of epiphysis

 

Pathology

 

Epiphyseal osteochondroma

- on histology

 

Aetiology

 

Spontaneous mutation

 

Presentation

 

Partial cartilaginous overgrowth of an epiphysis

- usually medial half

- Usually one joint

- Usually ankle (tarsus, carpus, knee, ankle)

 

Treatment

 

Partial excision of overgrowth

- do well but

- Recurrence common

- May need later osteotomies

 

 

 

Spine

Congenital Kyphosis

Definition

 

Kyphosis due to congenitally anomalous vertebrae

- characterized by severe angular deformity

- prominent gibbus at the apex of the curve

 

Classification

 

Type I - Failure of formation

 

Failure formation anteriorly + preservation of posterior elements

- hemivertebrae / wedge vertebrae

- most common

- produces the worst deformity

- NHx is one of relentless progression

- 7per year

- most likely to result in paraplegia

 

Congenital Kyphosis XrayCongenital Kyphosis CT

 

Type II - Failure of segmentation

 

Anterior unsegmented bar

- better prognosis

- produces more rounded kyphosis

- deformity progresses more slowly

- paraplegia is uncommon

 

Type III

 

Mixed pattern

 

Clinical Features

 

Severe deformity maybe obvious at birth

- less obvious deformities may not appear until later

- may be accentuated during adolescent growth spurt

 

Progression occurs to end of growth & often after growth complete

 

Mechanism

 

Due to

- growth differential

- erosion of vertebral body from mechanical pressure

 

Issues

 

Deformity can become very severe (Type I)

- breakdown of the overlying soft tissues

- compression of abdominal viscera

- impairment of pulmonary function

 

Paraplegia may occur (Type I)

 

Management

 

Non operative not effective

 

Work up

 

MRI / Neurosurgery

- exclude intraspinal pathology

 

Echocardiogram

- cardiac abnormality

 

Abdominal US

- renal abnormality

 

Operative

 

Type I

 

Ideal treatment is early detection & early posterior fusion

- best results if fusion by age 3

- can be done as early as 6/12

 

A.  < 5 years & curve < 50o

- posterior spinal fusion

 

B.  > 5 years and > 500

- anterior and posterior fusion

 

C.  Neurology

- anterior decompression first

 

Congenital Kyphosis CTCongenital Kyphosis Correction Lateral

 

Type II 

 

If early, can treat with anterior osteotomy of unsegmented bar

- i.e. epiphyseolysis

 

If late, requires posterior fusion

 

 

 

 

 

Neck

Atlanto-axial Rotational Instability

Pathology

 

Paediatric populations predisposed

- due to lateral mass anatomy and ligamentous laxity

- both alar ligaments and facet capsules must be torn to occur

 

Aetiology

 

Due to forced rotation and lateral tilt

Can be spontaneous

 

Presentation

 

Most patients have torticollis and limited rotation

 

Cock robin position 

- lateral flexion and rotation to other side

 

Fielding Classification

 

1. Rotatory fixation without anterior displacement

 

2. Rotatory fixation with anterior displacement 3 - 5 mm

 

3. Rotatory fixation with anterior displacement > 5mm

- indicates disruption of both facet joints and transverse ligament

 

4. Rotatory fixation with posterior displacement

 

Associations

 

Downs

RA

Klippel Feil

Morquio

SED

Achondroplasia

 

DDx

 

Torticollis

Atlantoaxial rotatory fixation

Ondontoid fracture

Os Ondontoid

 

Grisel's syndrome

 

Pyogenic atlanto-axial subluxation

- after upper respiratory tract infection

- inflammation induced ligamentous laxity

- can be post tonsillectomy

 

Present with painful torticollis and limited rotation

 

Xray

 

Widened atlanto - lateral mass interval

 

Dynamic CT

 

Head turned to left then to right

- demonstrate fixed subluxation

 

Management

 

Atlanto-axial instability

 

1.  Early presentation

- i.e. first day or two

- trial soft collar / analgesics

- see 1 week later

 

2.  Reduce any anterior displacement with halter traction

- add sequential weight

- check X-ray to ensure no C0/1 displacement

- valium and Phenergan

- if successful apply HTB

- flexion extension views out of HTB for residual instability

 

3.  Failure halter traction / Trial skeletal traction

 

4.  Failure skeletal traction / Open reduction and fusion

 

Residual instability

 

C1/2 Gallie fusion

 

Chronic

 

> 3/12

- consider fusion in situ

 

Grisel's

 

Usually will reduce spontaneously

 

First week

- NSAIDS and hard collar

 

Failure or > 1 week symptoms

- soft halter traction

 

> 4 weeks

- skeletal traction and HTB once reduced

 

 

Basilar Invagination

Definition

 

Cranial prominence of odontoid into region of foramen magnum

- upper cervical spine encroaches on brain stem

 

Aetiology

 

Congenital syndromes

- Klippel Feil Syndrome

- Achondroplasia

- OI

- Downs 

- Morquio

- SED

- NF

 

Congenital abnormalities

- atlas hypoplasia

- bifid posterior arch atlas

- occipitocervical synostosis

 

Acquired

- JRA

- Rickets

- RA

 

Clinical Features

 

Become symptomatic in third decade

- neurological compression 15%

 

Xray

 

McRae's line 

- line along foramen magnum 

- Basion to Opisthion

- dens above line = basilar invagination

 

MacGregor' s Line

- hard palate to inferior aspect occiput

- < 4.5mm above line

 

Ranawat Criterion

- distance between centre of C2 pedicle & transverse axis of C1

- men < 15mm

- women < 13mm

 

MRI 

 

Delineate pathology

 

Management

 

Consider surgery if symptomatic

 

Indications

 

1.  Severe pain

 

2.  Neurology

 

3.  Progression

- basilar invagination > 5mm

 

Technique

 

Surgery complex & fraught with complications

 

1.  Fusion

- Gallie C1/2 fusion if reducible

- occipitocervical fusion if not with C1 laminectomy

 

2. Excision of odontoid

 

 

 

 

Congenital Muscular Torticollis

Definition

 

Twisted / Wry neck secondary to fibromatosis in sternocleidomastoid

 

Epidemiology

 

Packaging defect

- commonest first born 

- 75% on right

 

Associations

 

CDH 20%

Metatarsus adductus 15%

Breech presentation

Klippel - Feil Syndrome

Arthrogryposis

 

Aetiology

 

Fibrosis of SCM on one side

Fails to grow & causes progressive deformity

 

Pathogenesis

 

Unknown

 

Theories

 

1. Ischaemia secondary to position in utero 

- compartment syndrome SCM

 

2. Birth injury with haemorrhage

 

Natural History

 

Many resolve spontaneously

 

However if untreated get permanent facial asymmetry

 

Clinically

 

Lump may be noticed in first few weeks of life 

- often disappears

 

Head tilted to one side so ear approaches shoulder

 

Head turned towards other shoulder

 

Associated facial asymmetry

 

DDx

 

Primary

 

Congenital fibrosis SCM

 

Congenital vertebral anomaly

- Klippel Feil

- os ondontoid

- C1-2 fusion

- unilateral C1 deficiency

- many others

 

Secondary

 

Trauma

- atlantoaxial rotatory subluxation

- # C1 /2

 

Grisel's syndrome

Ocular dysfunction

Infection / Discitis

SCM scar / tumour

HNP

 

X-ray 

 

To exclude congenital vertebral anomaly

- 17 cases of unilateral C1 deficiency with wry neck in literature

 

Indicated with failure non operative management

 

Management

 

Non operative

 

Stretching exercises

 

90% successful

 

Techniques

 

1.  Parents taught to carry child with their arm under flexed side of neck 

- stretches SCM whilst carrying

 

2.  Passive stretching exercises

- lateral head bend away from affected side

- head rotation towards affected side

- 90% success 

 

Operative

 

Indications

 

If persists past 1 year age chance of resolution very poor

- operate especially if > 30o limitation of movement

 

Timing

 

Ling et al Clin Orthop 1976

- 103 operations

- high rate scar tethering if released <1 year old

 

Options

 

1.  Distal release first

- if not successful proximal release also

 

2.  Distal and proximal release

- often at age 4-6

- expose both and mark with sling (more difficult to isolate after one end divided)

- complete release both

 

Technique

 

Distal release 

- 5cm transverse incision 1cm above medial end clavicle

- incise tendon sheath

- draw tendons out (NB sternal and clavicular heads)

- divide / Z plasty / excise 2 cm of both tendons 

- explore wound digitally for any fibrous bands and divide these

- if release incomplete perform proximal release

 

Proximal release

- incision immediately behind & below ear

- divide SCM transversely just distal to tip of mastoid process

- NB spinal accessory nerve at risk

 

Post operative

- manual stretching 3x /d starts at 1 week post-op

 

 

 

Congenital Odontoid Abnormalities

Ossification

 

Atlas

 

Three 1° Ossification centres

- two lateral masses with posterior arch in between

- anterior arch which appears at 1 year

- posterior arch fuses at 4 years

- anterior & posterior fuses to body at 7 years

 

Axis

 

Five 1° Ossification centres

- two neural arches / lateral masses

- two 1/2 dens

- body

 

Five 2° Centres

- Os Terminale (Appears 3, fuses 12)

- inferior ring apophysis

- 2 transverse processes

- 1 spinous process

 

Body of odontoid fuses by birth from two centres

- failure = Dens Bicornis

 

Dens separated from body by Dentocentral Synchondrosis

- present at birth

- fuses by 6 years

 

Tip of Odontoid not ossified at birth

- ossifies by age 3

- fuses by age 12

- Ossicum Terminale Persistens if doesn't fuse

 

Deformities

 

1. Os Odontoideum

- failure of fusion of base of odontoid

- normally fuses by age 6

- appears like Type II fracture

 

2. Odontoid Hypoplasia

- congenitally small odontoid

- asssociated with Morquio Syndrome & SED

- can get AAI 2° Hypoplasia 

 

3. Odontoid Aplasia

- absent odontoid

 

4. Os Terminale Persistens

- unfused terminal apophysis

- normally fuses by age 12

 

Os Ondontoideum

 

Theories

 

Congenital

 

Developmental

- ? os odontoideum is a traumatic event

 

Clinical Features

 

Usually asymptomatic

 

May present in adulthood with

- pain (Usually dull ache)

- neurologic symptoms

- Lhermitte sign / lightning pain

- long tract signs

- risk of sudden death

 

X-ray

 

Open-mouth view

 

Instability

- > 3.5 mm translation on FE views

- ADI > 5 mm

- SAC also important if < 14 mm

 

Surgical Indications

 

Non syndromic

 

1.  > 10 mm FE motion

2.  Painful neck with instability 

3.  Neurology

 

Prophylactic neck fusion is controversial

- if asymptomatic avoid contact sport

 

SED /  Morquio

 

ADI > 5mm = Fusion

 

Technique

 

Gallie Fusion

- involves using notched bone graft

- sitting it between the posterior arch of C1 and the spinous process and medial lamina arch of C2

- secured by sublaminar wires 

- this is not inherently rotationally stable

- usually requires halo immobilisation

 

 

 

 

 

 

 

Klippel Feil Syndrome

DefinitionKlippel Feil

 

Congenital synostosis of cervical vertebrae

- refers to patients with any element of cervical spine fusion

- ranges from 2 vertebrae to whole spine

 

Classic triad 

 

1. Short neck

2. Low posterior hairline

3. Stiff neck

 

Triad present in <50%

 

Aetiology

 

Failure of normal segmentation of mesodermal somites of neck

- during 3rd to 8th week

- may be AD inheritance

 

Clinical

 

Neck

 

Stiff especially lateral movements

- usually hypermobile at unaffected levels

 

Torticollis

 

Short neck

 

Head arises from shoulders

 

Often associated with webbing of neck

 

Low posterior hairline

- on level with shoulders

 

Problems

 

Degenerative OA

- caused by hypermobility of adjacent segments

- leads to instability & degenerative OA

- presents 3rd decade

 

VECTRAL

- associated abnormalities

 

Other congenital cervical spine abnormality

- occiptiocervical synostosis

- basilar impression

- odontoid anomalies

 

Scoliosis

- 60%

- congenital type

- associated respiratory problems

- need to consider neck problems when treating scoliosis

 

Sprengel's Deformity

- 30%

- same insult that causes KF halts descent of scapulae

 

Renal

- 35%

- renal failure often occurs early

 

Deafness

- 30%

- affects development of speech & language

 

Synkinesia

- 20%

- involve paired movements of hands

- abnormal pyramidal tracts

- usually resolves

 

Cardiac

- 15%

- various congenital defects

- need preoperative investigations / echo

 

Xray

 

Vertebral fusions

 

Klippel Feil Xray

 

Thoracic abnormalities

- scoliosis

- kyphosis

 

CT scan

 

Body fusion / block vertebrae

Hemivertebrae

Flattening & widening bodies

Hypoplasic discs

Cervical spina bifida

 

Klippel Feil CT SagittalKlippel Feil CT Coronal

 

Management

 

1.  Manage associated conditions

 

Torticollis

Scoliosis 

 

2.  Neck Deformity

 

Little can be done for neck deformity

 

Avoid contact sports as sudden death & neurology reported 

- worse if small mobile segment adjacent to long fused segment

 

 

 

Paediatric Back Pain

DDx

 

Spondylosis / spondylolisthesis

- most common cause

- adolescent playing sport

 

Scheuermann kyphosis
- 1/3 of patients

 

HNP - rare

Fracture

 

Apophyseal ring fracture

- traumatic fracture between vertebral body and cartilaginous ring

- apophysis displaced posteriorly with disc

- decompression if causes neurological symptoms

 

Discitis / Osteomyelitis

 

Tumour

- benign: OO / OB / ABC / Giant cell / EG

- malignant: Ewings / OS / Chordoma / Leukaemia

 

RA

Diastematomyelia

Juvenile Osteoporosis

 

Clinical

 

Pain character

- aspirin relief / night pain

- osteoid osteoma

 

Associated neurology & deformity

 

Systemic features / Red flag signs

- fever / weight Loss

 

X-ray

 

Standing PA & Lateral

 

CT

 

For spondylithesis

 

MRI

 

Low threshold

- may need GA

 

Bloods

 

WCC / ESR / CRP

RF, ANA, HLA B27

 

 

 

 

Paediatric Discitis

Aetiology

 

1.  Infection

- usually haematogenous 

- some direct spread from vertebral osteomyelitis

- usually frankly infective with marked vertebral body end plate changes

- elevated temperature & ESR

 

2.  Inflammatory 

- probable auto-immune picture with minimal bone changes

- no need for antibiotics

 

Anatomy 

 

Blood supply

- nucleus pulposis never has blood supply

- annulus fibrosis has blood supply until age 20

- primary infection possible in infants

 

Natural History

 

60% resolve

20% auto-fuse

20% chronic pain

 

Presentation

 

1-12 years / symptoms age dependent

 

0 - 3

- refusal to walk / weight bear / limp

- irritability

 

3 - 9

- abdominal pain

 

> 9

- back pain

 

Also

- stiff flexed spine 

- tight hamstrings & decreased SLR

- scoliosis

 

Microbiology

 

50% positive blood culture

66% positive CT guided biopsy

- usually not required

 

Types

- Staph 60-70%

- Streptococcus

- Gonococcus > 12 years

- E Coli in neonates

- Atypicals (TB, Brucellosis)

 

X-ray

 

Initial xray normal

 

Later

- loss of disc height

- end plate irregularity & sclerosis

- disc can regain height, but endplate changes remain

 

MRI

 

Child may need sedation / GA

 

DDx

 

Tumour

- leukaemia, metastasis (vertebral)

- EG (vertebra planar)

- OO, OB

 

Epidural abscess

Paraspinal abscess

SI joint septic arthritis

 

Management

 

Antibiotics

 

Controversial as whether to treat with antibiotics or not

- most authors agree that there is a bacterial component to the process

- most recover with or without antibiotics

 

Appropriate ABx (broad spectrum)

- bed rest

- brace

 

Usually rapid response

- CT guided biopsy if fails to resolve with above 

 

Results

 

Kayser et al Spine 2005

- 25 patients with spondylodiscitis

- most had long delays to treatment due to non specific symptoms

- inflammatory markers usually only mildly elevated

- 48% had evidence of vertebral body destruction

- 60% healed with disc narrowing, 40% with fusion

 

Garron et al J Paediatr Orthop 2002

- 35 needle aspirations of the disc

- 55% Staph

- 27% Kingella Kingae

 

 

 

 

 

 

 

Scoliosis

Congenital Scoliosis

Definition

 

Lateral curvature of the spine 2° to vertebral anomaly

- causes an imbalance in longitudinal spine growth

 

Epidemiology

 

True incidence unknown

 

F > M

 

Typically Thoracic

 

Inheritance

 

No association in twins / suggests not inherited

5% risk in family if complex multi-level

 

Aetiology

 

Occurs during mesenchymal development 4-8/52  

- unknown foetal insult

- high incidence other anomalies 

- VECTRAL"

 

V vertebral and spinal abnormalities

E endocrine

C cardiac

T tracheo-esophageal fistulas

R renal

R radial club hand

A anus imperforate/ urogenital (cloacae abn)

L limb Sprengel shoulder, club hand, CTEV

S single umbilical artery

 

Associations

 

Referrals

- neurosurgery

- cardiology

- urology

 

Spinal Dysraphism (25%)

- failure of neural tube closure

- diastematomyelia , syrinx, tethered cord, arnold chiari malformation, fibrous dural bands, intradural lipoma, SB

- clinically associated with hair patches, dimples, lipomas, tails

- all should have MRI especially presurgery

- can have rib abnormalities / fusions

- can have posterior element abnormalities

 

Genitourinary 25%

- 6% have obstructive uropathy

- horse shoe kidneys

- lifethreatening

- all have ultrasounds +/- urology opinion

 

Cardiac 10%

- all murmurs should be investigated

- ASD, VSD, PDA, Tetralogy Fallot

- all patients have echo prior to surgery

 

Limbs

- radial club hand

- congenital amputations

- cavovarus foot / CTEV

 

Klippel-Feil 25%

 

Classification 

 

International  Spine Society / MacEwen

- failure formation

- failure of segmentation

- mixed

- unclassifiable

 

Most common

- unilateral bar

- fully segmented hemivertebrae

- semi segmented hemivertebrae

 

Failure of Formation

 

A.  Partial / Wedge Vertebra (5%)

 

B.  Complete / Hemivertebra (30%)

 

Fully Segmented

- common

- open disc above & below

- greatest growth disturbance

 

Semi Segmented

- 1 growth plate (either above or below)

 

Non Segmented / Incarcerated

- no disc above or below

- minimal growth potential

 

Failure of segmentation

 

A.  Unilateral / Unilateral Unsegmented Bar (50%)

 

B.  Bilateral / Block Vertebra (5%)

 

Mixed

 

Combination both / Commonest

 

Unclassifiable (20%)

 

NHx

 

Anomaly present at birth but may not become evident until later life

 

Diagnosed < 3 or 9-14 years old

 

Associated with most rapid growth periods for the spine

- in utero

- from birth to 5 years

- puberty

 

Progression

 

Progression occurs if differential growth

- if more physis on one side 

- count number of physes

 

25% show no progression

 

50% progress rapidly

 

25% progress slowly

 

Prognostic Features

 

Type

 

Age

- worst prognosis with clinical deformity in first year of life

 

Location

- higher in the spine, less likely to progress

- upper thoracic curve best

- lumbar worst

 

Worst  10° / year

 

Unilateral unsegmented bar + fully segmented contralateral hemivertebrae

- over 14 years can get 140 - 180o curve

 

Intermediate 5˚

 

Unilateral unsegmented bar 

 

Fully segmented hemivertebra

- most common

- usually a problem

- 2 growth plates

- 2 consecutive hemivertebra a problem

 

Semisegmented hemivertebrae

- second most common

- single growth plate

- curve at maturity usually < 40o

 

Consecutive Hemivertebrae

 

Best < 2˚

 

Block vertebra best

- bilateral growth impairment

 

Non segmented / incarcerated hemivertebrae

- may produce slowly progressive curve

 

Unpredictable

 

Single hemivertebra unpredictable

- act as enlarging wedge

- often small with limited growth potential (incarcerated)

 

Xray

 

Lesion often difficult to interpret

 

See fused ribs

 

3D CT 

 

Excellent guide to anatomy

 

MRI

 

Should be performed before operative intervention

- assess for vertebral abnormality (40%)

- exclude Diastematomyelia / Tethered cord / Syrinx / Arnold Chiari Malformation

 

Other

 

Renal ultrasound

Echo

 

Management

 

Key is identification of curves likely to progress to prevent severe deforrmity

 

Non-operative

 

Bracing not indicated

 

Observe each 6/12

- look for progression

- examine for neurology

- continue until skeletal maturity

 

Operative

 

Indications

- progressive > 4-6o per year

- >40°

- high likelihood severe deformity i.e. unilateral unsegmented bar

- unbalanced child e.g. L5 hemivertebrae

 

Options

- fusions (posterior, anterior/posterior)

- hemi-epiphysiodesis

- hemivertebrae excision

- corrective osteotomy + instrumentation

- growing rods

- expansion thoracoplasty

 

Growth Inhibition

 

Each vertebrae contributes 1 cm in height in normal spine

 

Not true in abnormal spine

- delaying fusion actually makes patient shorter due to increasing deformity

- best to operate early

- unbalanced growth centres don't contribute to vertical height

 

Principle 

 

Fully segmented hemivertebrae

 

1.  Stop growth on affected side

- segments above & below fused unilaterally on side of hemivertebra

- hemi-epiphyseodesis performed anterior & posterior

- effectively produce contralateral unsegmented bar above & below

 

2.  Excision of hemivertebrae

- more common

- especially at lumbosacral junction

 

Unilateral bar

 

Stop growth on other side

- hemi-epiphyseodesis anterior / posterior on  convex side 

- < 5 years

- segment effectively converted to block vertebra

 

Other options

 

Growing Rods

 

For patient < 5

- long segment involved

 

Expansion Thoracoplasty

 

Fused ribs common

- excise fusion

- vertical expandable titanium rib

- allows chest expansion and normal development of ribs

 

 

Idiopathic Scoliosis

Adolescent Idiopathic Assessment

DefinitionScoliosis Thoracic Major

 

Lateral curvature of the spine with vertebral rotation

- defined as > 10o coronal plane deformity

- occurs at or near the onset of puberty 

- no cause is established

 

Planes of Deformity

 

Triplanar

- coronal / scoliosis

- sagittal / thoracic lordosis

- transverse / rotational

 

General Categories

 

Structural

 

Fixed lateral curvature with rotational deformity

- intrinsic anatomical change

 

1.  Idiopathic 75%

2.  Neuromuscular 10%

3.  Congenital 10%

4.  Other 5%

 

Non structural

 

Reversible, non rotational and disappears with sitting

- nil intrinsic anatomical change

 

Compensatory / Hysterical / Irritative / Postural / Sciatic

 

Incidence

 

< 10o - 2.5 %

> 30o 4 / 1000 

> 40o 1/1000

 

Gender

- little difference overall

- females more likely to have larger curves

- females more likely to progress

 

Classification

 

Scoliosis Research Society (SRS)

 

Infantile: 0-3 years onset 

 

Juvenile: 3-10 (Puberty)

 

Adolescent: 10 - Cessation of Growth (20 years)

 

Alternative

 

Early Onset - < 5 years 

- rare and severe

- male 2:1

- left sided

- if less than 1, 90% resolve

- >1, 20% resolve

- many other congenital anomalies

 

Late Onset  - > 5 years

- Adolescent Idiopathic

- females 6:1

- right thoracic

- nil associations

- FHx common

 

Aetiology

 

Structural Differences

 

Intervertebral Disc

- decreased GAG in Nucleus and increased collagen content 

 

Paravertebral Muscles 

- differences in muscle fibres on either side of curve 

- more type I fibres on the convex side of curve 

 

Ligaments and Tendons 

- PLL thickened 

 

Endocrine 

- patients with idiopathic scoliosis often taller 

- normal GH but altered Somatomedin levels - ? significance 

 

Vertebral Body

- structures on concave side hypoplastic 

- structures on convex side hyperplastic

- due to persistent asymmetrical loading

 

Postural Equilibrium

- abnormality in vestibular system in brainstem 

- scoliosis induced in bipedal rats with destruction of brainstem 

- not conclusive - ? effect rather than cause 

 

Neurotransmitter

 

Scoliosis produced when the pineal gland removed from chickens 

-  transmitter found to date - ? melatonin

 

Genetics 

 

Increased incidence in affected relatives 

 

Mother with scoliosis

- 10% chance for female child

 

Sister with scoliosis

- 20% chance for female child

 

Mother and father with scoliosis

- 80% chance for female child

 

Lordosis

 

Biomechanical initiator of the deformity

- thoracic lordosis normally lies in front of the normal axis of rotation

- causes the lumbar lordotic section to rotate in flexion 

- the tethering of the posterior elements (thickened PLL) also contributes to rotation in flexion

- explains the Crank Shaft Phenomena 

 

Adolescent Idiopathic Scoliosis 

 

Epidemiology

 

Prevalence dependant on the size of the curve 

 

As the curve increases in magnitude the female preponderance increases as well 

 

Overall is 3.6:1  F:M

 

Curve Patterns

 

There are five major curve patterns in decreasing order they are

 

Right Thoracic

Double major (Thoracic dominant)

Thoracolumbar 

Double major (Lumbar dominant)

Left Lumbar 

 

Progression 

 

Definition

 

Absolute increase in Cobb angle of 10o

- or 5o over two consecutive visits 

 

Remember the interobserver error of Cobb angles is +/- 4o

- can vary with the time of day (increases in the pm)

 

Factors related to progression

 

MR Sex MAP

 

Magnitude:  curve > 20o

Risser:  0 or 1

Sex:  Female

Menarche: premenarche

Age: < 12

Pattern:  Thoracic & double curves most

 

Growth Remaining

 

Menarche 

- 66% prior to and 33% after menarche 

- most growth is 1 year before and 1 year after menarche

- have on average 2 years growth left

- have passed PHV

 

PHV

- peak height velocity / most important factor

- adolescent growth spurt

- girls 8 cm / year

- boys 9.5 cm / year

- before menarche / at Risser 0 / open triradiate cartilage

- PHV generally over 2 years

 

Tanner sign 

I - Pre-pubertal 

II - Breast buds  - related to adolescent growth spurt

III- Pubic hair 

IV- Menarche 

 

Triradiate cartilage

- may be more sensitive in judging the maturity 

- Risser 0 + open triradiate cartilage indicates a lot of growth to go

- closes in the middle of the PHV

 

Risser sign 

 

Risser grade relies on ossification of the iliac apophysis from lateral to medial and is completed with maturity

- Grade 0 to 5 

- Grade 0 means no ossified apophysis present

- Grade 1 means appearance of apophysis laterally / after menarche

- Grade 5 is fusion of the apophyseal cap to ilium / little growth remaining / 14-16 Boys and 11-13 Girls

- can be difficult to distinguish between 0 and 5

 

Curve Pattern 

 

Double curves have higher incidence of progression than single curves

- single thoracic > single lumbar

- lumbar the least

 

Curve Progression Studies

 

1.  Lowenstein Study of Curve Progression

 

Looked at Risser sign + intial curve in regards to curve progression

 

Risser   0 - 1 2 - 5
Initial curve 0 - 19o 22% 2%
Initial curve 19 - 290 66% 22%

 

2.  Weinstein and Ponsetti 

 

Looked at the progression after maturity  / 30 year study

- curves less than 30o as rule DO NOT progress after maturity 

- 50 - 75o progressed most ( 1o/ year ) 

- this is the basis for surgery for curves 450 plus

 

Slowed over 100o with costopelvic impingement 

 

Findings

- mortality 2x expected

- high percentage disability pension

- none in heavy work

- nil increase incidence LBP

 

Natural History of Untreated Scoliosis

 

Back Pain 

 

Most studies suggest that the incidence of back pain is no higher than in general population 

 

Back pain seen in thoracolumbar or lumbar curves of > 45o

- particularly if large apical rotation or imbalance 

 

Pulmonary Function 

 

Affect on pulmonary function not seen until curves of 80o reached  

- restrictive pattern

- linear relationship between FVC and PaO2 and curve size 

- nil effect with curve < 60o

- 1/3 with curve 60-100o

- 1/2 with curve > 100o

 

Mortality

 

Nachemson 1968

- not increased until curve of 100o noted

 

Cor Pulmonale 

 

Seen at 40 and 50 years of age if curve > 80o

 

Assessment

 

History 

 

How detected 

Presence of progression 

Associated complaints 

- pain 

- neurological symptoms 

- respiratory symptoms 

 

Status of growth 

- growth spurt

- menarche

- changes in puberty 

 

Want to ensure is idiopathic

- normal delivery / normal milestones

- bladder troubles (NM)

- Marfinoid / OI / NF

 

Examination 

 

See the section on examination for detail

 

AP Film

 

Standing AP or PA films of whole spine including the iliac crests 

- PA has less radiation to ovaries and breasts 

- AP has less magnification 

 

1.  Neutral / end vertebrae

 

End vertebra is the last vertebra that tilts into the concavity of the curve

- when the end plates are parallel, the one furthest from the apex of the curve is the end vertebra

 

Scoliosis End Vertebrae

 

2.  Cobb Angle 

 

Detect the end vertebrae where the end plates are last to converge 

- line drawn along upper plate of the upper end vertebrae and lower plate of the lower end vertebrae 

- perpendiculars to these lines 

- intersection angle measured 

 

If double curve

- one vertebrae is upper end vertebrae for the lower curve 

- lower end vertebrae foe the upper curve 

 

Measurements all taken from same vertebrae in future 

 

Scoliosis Cobb Angle

 

3.  Identify Apical Vertebrae

 

In centre of curve

- furtherest from central sacral line

- not tilted / most horizontal

- maximum rotation

 

T10 above apical - Thoracic

T11 - L1 apical - Thoracolumbar

L2 down - Lumbar

 

4.  Stable Vertebrae

 

Central sacral line

- lowest vertebrae this bisects or

- line between 2 pedicles

- lowest vertebrae instrumented in surgery

 

Scoliosis Central Sacral Line

 

5.  Look at shoulders

 

Important in double thoracic major

- high structural thoracic curve

- if left shoulder high in right thoracic curve

- need to instrument to T2 to correct this

 

Lateral Films 

 

Standing 

- measure the kyphosis and lordosis via Cobb method

- important presurgery

- want to correct this intra-operatively

- usually need to recreate thoracic kyphosis

 

Lateral Bend Films 

 

Push prone

- supine with maximal voluntary bend 

- differentiates structural from compensatory curves 

 

MRI 

 

If suspect intraspinal pathology 

- Brain + 3 level spine / neurocentral

 

Indications for MRI

 

Scoliosis Left Lumbar Curve

 

Left sided 

Male 

Painful 

Rapidly progressive 

Neurological abnormality present 

 

Findings 

 

Right sided curve: 20% have pathology 

Left sided curve: 80% have pathology 

 

Assessment of Rotation 

 

Rib Hump / Scoliometer 

 

Adams forward bend test

< 5o tilt = < 30o rotation

> 7o tilt = > 30o rotation

 

Rotation of Pedicles

- indicates the structural curve

 

Classification

 

Lenke

 

3 areas of curve

- main thoracic: MT

- proximal thoracic: PT

- thoracolumbar / lumbar: TL/L

 

Assess

- curve location

- lumbar modifier

- thoracic sagittal profile

 

Type 1 Main Thoracic

- MT structural

- PT non structural

- TL/L non structural

 

Scoliosis Main Thoracic

 

Type 2 Double Thoracic

- MT and PT structural

- TL/L non structural

 

Type 3 Double Major

- MT and TL/L structural

- PT non structural

 

Type 4 Triple Major

- all 3 structural

 

Type 5 Thoracolumbar / Lumbar

- only TL/L

 

Scoliosis Lumbar

 

Type 6

- TL/L and MT structural

- TL > MT by more than 10o

 

King-Moe  Classification

Very poor inter observer reproducibility

 

Type I -  Lumbar Dominant Double T + L

- both the thoracic and lumbar curves cross the midline 

- lumbar curve larger and more rigid 

 

Type II - Thoracic  Dominant Double

- both the thoracic and lumbar curves cross the midline 

- thoracic curve larger and more rigid 

 

Type III -  Short Thoracic 

- thoracic curve 

- lumbar curve doesn't cross the midline 

- lumbar curve not structural

 

Type IV - Long thoracic 

- long thoracic curve extends to lumbar spine 

- L5 over the sacrum 

- L4 tilted into the curve (stable vertebrae)

 

Type V -  Double structural thoracic 

- double thoracic curve with L upper, R lower

- tilting of T1 into the upper curve / elevation of L first rib

- cervical extension

- compensatory lumbar curve with upper curve structural

 

 

 

Adolescent Idiopathic Management

Observation

 

Curves < 20o observation only at 3-6 month intervals depending on growth rate 

 

Non Operative Management / Bracing 

 

Never brace curves if patient Risser 4 or 5 

 

Indications

 

1.  Risser 0-2 (growth potential)

 

2.  Curve >30o adolescent

 

3.  Curve >25o with progression (5o in six months)

 

4.  < 10 years old

- very young with high progression potential

- high risk crankshaft if operate

 

5.  Willingness to comply

 

Guidelines

 

Angle                      High Growth Potential                Lowth Growth Potential

<20°                                   observe                               observe or DC

20°-30°                               observe/brace                     observe

30°-45°                    brace                       observe

>45°                                   surgical                              surgical / observe

 

Effect

 

Will control curve only

- end result is initial curve + 5o

 

Brace should be customised to patients curve 

- designed to prevent progression NOT to achieve correction 

- generally see a moderate amount of correction when using the brace

- then slow steady progression of curve back to original magnitude during weaning 

- best curves to brace are those < 40o

 

Bracing complications

 

Failure to prevent progression

Skin irritation

Pressure areas

Abdominal discomfort, eating habit disruption

Cast syndrome - SMA / duodenal obstruction

Psychological

 

Milwaukee Brace  / CTLSO

 

Best for curves with apex above T8 

- three point fixation technique 

- less efficacious for curves > 40o

 

Consists of 

- well moulded pelvic piece above the iliac crests (most important)

- two posterior uprights and one anterior upright 

- neck piece with plastic throat mould anteriorly and two occipital pads posteriorly         

- thoracic pad placed over the apex of convexity of curve 

- lumbar pad over TP between lowest rib and iliac crest on concave side 

- active correction by muscle contraction pulling body away from pads

 

Protocol

 

23 out of 24 hours a day

- result dependant on time in brace

 

Need to check regularly and readjust after 1-2 weeks 

- Xray on 6 month basis 

- if progresses > 45o then surgery

 

Aim for 30-50% correction in first 6 months 

- if not achieved consider surgery 

 

Weaning

 

Once skeletal maturity / Risser 4 / full height 

 

Wean 

- 20 hours for 4 months 

- 16 hours for 4 months 

- 12 hours for 4 months 

- night time only for 4 months 

 

TLSO (Under arm or Boston Brace)

 

If apex < T8 

 

Higher compliance 

May not be as efficacious in holding correction 

Made from cast

 

Operative Management

 

Indications 

 

1.  Immature / Risser 1 /2

- Cobb > 40o with documented progression

- peak height velocity

- will progress 1o per month

- need to stabilise early

 

2.  Mature

- T > 45 - 50o

- TL or L > 30o with marked rotation

- double major > 50o

- significant coronal imbalance

- cosmetic deformity

- failure bracing

 

This curve will progress slowly

- patient has time to make up mind

 

Goals

 

Solid arthrodesis that prevents progression

Balanced spine

Correction of deformity

Prevent respiratory compromise

 

Options

 

1.  Most curves 

- posterior instrumented fusion

 

2.  Lumbar curves

- anterior instrumented fusion

 

3.  Large curves > 70o / young patients

- anterior and posterior surgery

 

Principle

 

Fuse the structural curve with minimum segments

- to stable vertebra

- minimise the levels (preserve motion segments)

- avoid to L5 and above T1 (may increase pain)

- if fuse to L5, only 1 motion segment left, risk LBP

 

Correct curve in sagittal and coronal planes

 

Best to wait til 10 - 12 years to avoid crankshaft

 

Structural Curve

 

1.  Largest curve

2.  One to which trunk shifted

3.  Least correction on AP lateral bending Xray

4.  Pedicles rotated

 

Posterior Instrumented fusion

 

Multisegmental Hook and Pedicle screw systems

- allows for correction via Compression / Rotation / Distraction

 

Crankshaft phenomenon

 

Concept

- seen in young child with high growth potential

- pre PHV surgery or with open triradiates

- pivot on posterior fusion

- vertebral bodies and discs bulge towards convexity

 

Problem

- get loss of correction, increase in rotation, recurrence of rib hump

 

At risk

- Risser 0

- girls < 10

- boys < 13

 

Specific Surgery

 

Lenke Type 1

- main thoracic

- posterior stabilisation

- usually limit to T4 as shoulders equal

 

Scoliosis Fusion Long Thoracic

 

Lenke Type 2

- double thoracic / MT and PT

- need to instrument to T2

- equalise shoulders

 

Scoliosis Fusion Double Thoracic

 

Lenke Type 3

- double major / MT and TL/L

- long posterior instrumented fusion

 

Lenke Type 4

- triple major

- very long posterior instrumented fusion

 

Lenke Type 5

- thoracolumbar / lumbar curve

- can fuse short curve this through bed of T9 / T10 rib

- otherwise posterior instrumented fusion

 

Scoliosis Lumbar Curve Fusion

 

Lenke Type 6

- TL > MT structural

- long posterior instrumented fusion

 

Technique Posterior Instrumented Fusion

 

Pre-operative

 

Consent

Cell saver

- accumulate large blood loss

- often large exposure

Xmatch blood

2 x milled femoral head allograft 

Spinal monitoring / SSEP's

- needles scalp / hands / feet

- begin pre-op once asleep as baseline

IDC

Pedicle screws / TP hooks / rods available

Post op ICU bed especially neuromuscular

 

Position

 

4 Poster Bed

Protect eyes, knees, elbows

No pressure on abdomen / reduce venous bleeding

 

Dissection

 

Posterior approach

- betadine pack buttocks

- midline incision

- divide thoracolumbar fascia midline

- split apophysis with knife (if present)

- subperiosteal elevation strap muscles

- use diathermy, cobb

- sequentially pack with rolled up packs to control bleeding

 

Lumbar spine

- expose facet joints and transverse processes

- don't go between transverse processes laterally as nerve roots here

- pedicle screws inserted bilaterally bottom 3 pedicles

 

Thoracic

- TP hooks above

- pedicle hooks below

- compress

 

2 x rods prebent in sagittal plane

- correct coronal malignement and rotation as able

- may use sublaminar wires if large long curve

- midsection of curve in concavity

- tie over rod and tighten to correct

 

Decorticate lamina, add bone graft along each side

 

Closure

 

Technique Anterior Fusion

 

Indications

 

Large lumbar curve in young patients 

- skeletally immature patient to achieve growth arrest and prevent crankshaft

 

Any lumbar curve to decrease fusion length

- this is debatable

 

Large / rigid curve to achieve mobility 

- severe curves >70o

- supplement posterior fusion

 

Advantages

 

Fewer levels instrumented 

Better correction of rotation

Large surface for fusion

Fusion under compression

Use rib as bone graft

 

Disadvantages 

 

Requires anterior approach

Does not produce lumbar lordosis 

Respiratory problems (need chest drain)

Need to divide segmental vessels

 

Technique

 

Supine, rolled

- curved right sided approach

- remove 9th rib (save for bone graft)

- through bed of rib

- identify peritoneum, stay outside

- take down diaphragmatic crura

- divide segmental vessels, remove discs

- unilateral screws and rod

- repair diaphragm, close over ICC

 

Endoscopic Anterior Instrumentation

 

Advantages

- reduced blood loss and pain

- better scars and cosmesis

 

Disadvantages

- technically difficult

- respiratory problems / deflate lung

 

Growing rods

 

Indications

 

Growing children / open triradiate cartilage

- avoid fusion / crankshaft phenomen

- biannual surgery

- high complication rate 50%

- hook dislocation

- rod breakage

 

Costoplasty / Thoracoplasty

 

Technique

 

Partial excision of 5 or 6 ribs from the TP to posterior axillary line 

 

Advantage

 

Corrects the rib hump 

Cosmetic procedure

Good source of graft 

Does not affect the post op morbidity or pulmonary function

 

Complications G. Coe SRS Report 2006

 

Early

 

Neurological

 

0.32% in posterior corrections (SRS) in adolescents 

- 2% in adults 

- highest in congenital curves

 

Prevention

- SSEP's monitoring in all idiopathic and congenital curves 

- wake - up test in suitable patients (difficult in children)

 

SSEP's

- stimulate in legs, readings in cortex

- avoid inhalation anaesthetics

- time delay as must average amplitudes and reduce background noise

- issue if lose > 50% amplitudes

 

If lose SSEP's

- avoid hypotension

- transfuse Hb if low

- check electrodes

- wake up test

- give steroids

- reverse correction

- remove instrumentation

 

Infection 1.35%

 

Prophylaxis warranted 

Late chronic infection with Proponiobacterium acnes 

 

Respiratory 1.6%

- PTX

- atelectasis

 

PE 0.02%

 

Death 0.03%

 

Ileus - very common

 

Blood Loss 

 

Avoided with

- autologous blood 

- cell savers 

- hypotensive anaesthesia

- autotransfusion

- often blood loss that contributes to neurological compromise 

 

Incorrect fusion levels / wrong level surgery

 

SIADH secretion 

- decrease UO night of surgery

- steady improvement 2-3/7

 

Late

 

Pseudoarthrosis

- 1% overall

- instrument failure 

 

Crank shaft Phenomena 

 

In rapidly growing child after posterior fusion

- spine will rotate as the bodies grow anteriorly

- thus if child with significant growth then add anterior discectomies and fusion

 

Other solutions

- posterior growing rods

- anterior staples / guided growth

 

Sagittal malalignment 

- loss of lumbar lordosis

- flat back / loss of thoracic kyphosis

 

Back Pain 

 

Related to fusion below L4 and loss of lumbar lordosis 

 

Levels and back pain

- L5 - 80%, L4 - 60%

- L3 - 40%, L2 - 20%

 

Late infection - low virulence organism

 

Results

 

Gothenburg Sweden 1968

- 23 year follow-up post fusion with instrumentation

- preop Cobb 62°; postop 33°; last followup 37°

- same series had 127 patient braced

- prebrace 33°, best brace position 30°, last followup 38°

 

 

 

Infantile Idiopathic

Epidemiology

 

< 4 years by definition

 

M : F

 

75% left thoracic

 

Actually very uncommon 

- likely most patients once had spinal dysraphism (Arnold-chiari / syrinx / tethered cord)

- reduced by prenatal folate

 

Progression

 

Age < 1

- 90 % spontaneously regress

- very important

- 10% progress to severe deformities

 

Age > 1

- 80% progress

- more likely to cause cardio / respiratory compromise

- alveoli not developed til age 8

- lungs need room to develop

- high associated with other abnormalities

 

Progression Risk

 

1.  Mehta Angle / Rib-vertebral angle difference / RVAD

 

Difference in angle at which rib meets spine at Apex of curve on either side on AP x-ray

-  > 20° likely to progress 

 

Phase one and phase two

- phase 2 : rib head overlaps and angle cannot be measured

- risk of progression high

- indicative of rotation

 

2.  Degree of Curve

 

Likely to progress if curve > 25°

 

CT

 

Exclude congenital scoliosis

 

MRI

 

Mandatory

- 20% incidence intraspinal pathology

- syrinx / tethered cord / diastematomyelia / ACM

 

Management

 

Non operative

 

Indications

 

Curve < 25o and RVAD < 20o

- resolve spontaneously

- no need for treatment

 

Curve > 25o and RVAD > 20o

- brace

 

Bracing

 

Serial cast < 1 year

 

Brace > 1 year 

- Milwaukee brace can be curative

- younger age group more likely to tolerate this brace than adolescents

- brace must be worn until curve maximally & permanently corrected

- infantile growth spurt continues till ~ age 4-5

 

Operative

 

Indications

 

Curves > 35o

Progressive curves 5o in 6/12 

 

Issue

 

Patient < 10 years or before PHV

- high risk crankshaft effect 

- fusions must be anterior and posterior

 

Options

 

1.  Posterior growing rods

- obtain correction whilst maintaining truncal growth 

- subcutaneous Harrington rod / Growth Rods / Luque rods

- spine exposed at ends for hook insertion with submuscular or subcutaneous rod insertion

- requires surgical adjustment every year (can be done up to 5 times)

- surgery each time adds to scar and increases risk of problems including infection and cut out

- posterior fusion at later age 

 

2.  Hemiepiphyseodesis

- fusion of convex side of apical vertebrae

- is difficult to obtain half disc fusion

- may be used supplementary to growing rods

 

3.  Staples / guided growth hemiepiphyseodesis

- can be done endosopically

 

4.  Posterior instrumented fusion

- not indicated in young children as causes crankshaft effect due to anterior growth continuing

- can be safely performed at age 12 (girls) or 14 (boys)

 

5.  Anterior & Posterior instrumented fusion

- avoids crankshaft effect

- inhibits truncal growth

 

 

 

Scoliosis Examination

Aims of Examination

 

1.  Identify cause

- Marfan's / Neurofibromatosis / Skeletal Dysplasia

 

2.  Balance & body asymmetry

 

3.  Exclude LLD as cause

- correct with blocks or sit patient

 

4.  Forward flexion / Adams forward bending

- look for rotation / rib hump

 

5.  Assess flexibility if considering surgery

 

Typical curve

- the right shoulder is raised

- the right scapula is prominent

- the loin creases are asymmetrical

- the pelvis is level

- there is flattening of the normal thoracic kyphosis

- there is a normal lumbar lordosis

- on forward bending, there is a (mild/moderate/severe) (well rounded/angular) rib hump and a mild left lumbar fullness

 

Front

 

Skin

- cafe-au-lait spots / axillary freckling / neurofibromas

 

Eyes

- Lisch nodule (NF)

- blue sclera (OI)

- cloudy cornea (mucopolysaccharidases)

- dislocated lens (Marfan's)

- optic glioma

 

Mouth

- abnormal teeth (OI)

- high-arched palate (Marfan's)

 

Trunk

- pectus carinatum or excavatum (Marfan's)

 

Limbs

- hemihypertrophy

- dolichostenomelia (long limbs)

- arachnodactyly (thumb in palm)

- clubfoot - often first sign of dysraphism

- cavovarus foot

 

LLD

 

Side

 

Thoracic kyphosis - reduced / hypokyphotic

 

Lumbar lordosis - exaggerated, normal or reduced

 

Protruberant abdomen

 

Back

 

Curve 

- right or left

 

Balanced / Unbalanced 

- alignment of C7 over gluteal cleft (ask for plumb bob)

 

Shoulder height

 

Scapular symmetry

 

Loin creases / lumbar fullness

 

Pelvis 

- level or not (pant line or PSIS)

 

Spinal dysraphism

- hyperpigmentation / hairy patch / dimple / lipoma / tail

 

Leg length 

- if abnormal use blocks & reassess curve

 

Scoliosis can cause apparent LLD

- true curves rotate into their convexity

 

A long leg will elevate the hemipelvis / convexity will be to opposite side

- fixed left scoliosis will give apparent left leg shortening

 

True LLD -> Apparent scoliosis

True scoliosis -> apparent LLD

 

Adam's test 

 

Hands together & bend forwards to touch floor

- mild / moderate / severe rib hump

- well rounded or angular

- satisfactory unroll

 

Meaure rib hump with scoliometer

 

Gait / STRAWS

 

Short

Trendelenburg

Rigid

Antalgic

Weak

Spastic

 

Supine

 

SLR

- hamstring tightness

 

Neurological

- reflexes UL / LL / Abdominal / Babinski

- sensation

- power UL / LL

 

Scoliosis + °Abdominal reflexes & °Axillary sensation 

- syrinx till proven otherwise

 

Abdominal reflexes disappear during teens

 

Prone

 

Correctable

 

Collagen

 

Ligamentous laxity / Wynne Davies criteria

 

Concealed

- if forgotten 

- natal cleft / mouth / eyes / axillae

 

Cephalad joints

- ROM if deformity

 

Circulation

 

Xray

 

"This is a PA spine radiograph of a __ old skeletally mature / immature Risser __ male/ female with scoliosis"

 

"There is a R/L typical/atypical curve thoracic/lumbar curve ±  a R/L T/TL/L lower curve"

 

"The spine is/isn't balanced, the pelvis is/isn't level & the curve has a rotational component"

 

"The curve appears to be Idiopathic / Congenital / NF / NM

 

Don't mention which is 1°/ 2° or postural or structural

 

Neuromuscular Scoliosis

Epidemiology

 

Prevalence of 25-100%

 

Ambulation

- walking decreases incidence

 

Age 

- worse with young onset

 

Aetiology

 

Exact biomechanical explanation of origin & progression of curves unclear

 

Associated with

- weakness 

- poor muscle control

- no proprioception

 

Classification

 

1. Neuropathic

 

A. UMN

- CP 

- Friedrich's

- cord tumour / trauma

- CMT

 

B. LMN

- Polio

- Spinal muscle atrophy

- Spina Bifida

 

2. Myopathic

- Arthrogryposis

- Muscular Dystrophy

- Myotonia Dystrophica

 

NHx compared with Idiopathic 

 

Onset younger

More rapid progression

Progression after skeletal maturity / throughout life

Longer curve

Cervical involvement

Pelvic obliquity

Pulmonary complications common

 

Curve

 

Long C shaped curve

- compensatory curve uncommon but possible

 

Curve associated with

- pelvic obliquity

- hip contractures / dislocation

- cervical involvement

 

Other problems

 

Lung disease common (decreased FVC, LRTI) 

Poor nutrition 

Pressure areas

CRF in Spina bifida

 

Management

 

Aims

 

Maintain cardiorespiratory function

Promote mobility & ambulation

Preserve sitting balance

Improve cosmesis

 

Principles

 

Brace till 12 years then fuse

 

Earlier if

- lose control with rapid progression

- large curve

 

Nonoperative

 

Indications

 

Small curves < 30°

Large curves in patients with very poor prognosis

 

Orthoses

 

Best overall initial treatment

- rate of progression slowed

- allows further spinal growth before definitive treatment

 

TLSO

- 2 piece custom moulded

- used to control & correct scoliosis or kyphosis in growing years

- preferable to Milwaukee brace in NM conditions

- easier to make and wear with reduced incidence of press sores

- worn during day while child is upright

- off at night

 

Seating

- very important

- aims to give trunk & spine control

- able to correct postural curves

- no effect on structural curves

- significantly decreases nursing & handling time

 

1. Tumbleform Seat

- moulded seat for infants

- 3 sizes

 

2. Commercial Chairs

- pelvic support, abduction pillow, thoracic support, head support

 

3. Moulded Sitting Support Orthosis

- AKA SSO

- custom fitted support made from patient mould

- provides maximum sitting support with padded supports & belts

 

Surgery

 

Neuromuscular Scoliosis Posterior Fusion

 

Aim

 

Solid arthrodesis of balanced spine over level pelvis

 

Requirements

 

Long, solid fusion from upper thoracic to lower lumbar

Rigid instrumentation & massive bone grafting

 

Issues

 

Increased bleeding

Osteopenic bone / Poor hold

Fusion to pelvis

High pseudarthrosis rate

Poor medical state

 

Ambulation

- fusion may eliminate ability to ambulate

- trunk movement may be important for ambulation

- obviously lose any spinal movement with flexion

- should test in brace first

- may need to limit extent of surgery

 

Preoperative

 

General medical workup

- cardiopulmonary status

- especially Friedreich's & Duchenne MD

- FVC1, FEV, ABG's

 

Book ICU bed

 

Approach

 

Posterior

- most common especially in CP

- moderate curve

 

Staged Anterior / Posterior

- severe kyphosis / severe rigid scoliosis (> 45°)

- posterior fixation compromised (SB)

- < 9 years to stop crankshaft deformity

 

Technique

 

Require

- spinal cord monitoring if indicated

- cell saver

- post op ICU bed or HDU

- IDC

 

Instrumented fusion

- proximally from T3 or T4 to prevent later subsequent deformities

- caudally to pelvis if >15° pelvic obliquity

- to L4 or L5 if balanced

- often need sublaminar wires for large C shaped curve

- place in midcurve to pull spine to the rods

- large fusions require allograft

 

Blood Loss

- issue because extensive approach / small  blood volume / osteopenic bone bleeds+++

- addressed with meticulous haemostasis / hypotensive anesthesia / haemodilution / cell saver

 

Complications

 

Pulmonary complications

- most common cause of death

- nearly all CP patients get pneumonia post op 

 

Neurological injury

- especially if correcting curve > 90°

- spinal monitoring

- if damaged when wake up, remove metalwork

 

SIADH

- careful fluid balance 

 

Infection

- bacteraemia from remote sites especially  UTI

- increased incidence of wound infection

 

Loss of ability to ambulate

- due to loss of trunk flexion and extension

 

Cerebral Palsy

 

Most common neuromuscular cause of scoliosis in western world

 

Principles

 

Professor Bruce McPhee, Royal Brisbane Hospital

- key is that they all continue to progress past maturity

- says as general rule all need operation so can pick your timing

- try not to fuse too early as get very short trunk

- no longer goes to pelvis 

- feels that stopping at L5 is adequate and pelvis is not worth the morbidity

- insists CP patients have their operation at 50o

- if families decline he won't operative at 70-80o because then they need 2 stage

- high associated morbidity

- follow up every 6 or 12 months

 

Incidence

 

10% in ambulatory patients

70% spastic quadraplegics

 

Lonstein Classification

 

Group 1 curves (A&B) have level pelvis

- double thoracic curve

- little pelvic obliquity

- fuse as for idiopathic

 

Group 2 curves (C&D) have pelvic obliquity

- large lumbar or thoracolumbar curve

- marked pelvic obliquity

- fuse long (T4) to pelvis

 

Algorithm

 

Observation

- curves up to 30˚ in the growing child

- curves up to 50˚ in skeletally mature

 

Orthoses

- often only postpones arthrodesis until puberty

- seating most common form of non-operative treatment instituted

- TLSO effective in ambulatory patient

 

Surgery

 

Fusion to sacrum essential with pelvic obliquity

- Galveston technique (iliac wing rod fixation)

- pedicle screws only viable in S1, not S2

 

Spina bifida

 

Incidence

 

>60% of patients with myelomeningocoele develop scoliosis

 

Principles

 

The higher the level the more severe

- T12 - 100%

- L1 - 90%

- L2 - 80%

- L3 - 70%

- L4 - 60%

- L5 - 25%

- S1 - 10% 

 

40% incidence of spinal dysraphism

- may cause scoliosis progression

- hydrocephalus, syringomyelia, tethered cord, Arnold Chiari

 

20% have congenital component

 

Most require surgery

 

Why fuse wheelchair bound Spina Bifida children?

 

Physiological

RFT's

Feeding

- often use peg

- tends to disappear into skin folds

- hygiene & technical difficulties

Acid reflux when hunched over

Only tolerate small feeds

 

Physical

Pressure areas

Ability to nurse

Takes arm off chair so can do ADL's

Allows stable sitting

Pelvic obliquity

Allows coverage of hips

 

Psychosocial

Self-esteem both patient & carer

Allows better interaction with environment

 

Poor prognostic signs

 

Early age of onset

Asymmetrical motor paralysis

Presence of spasticity

10% - 20% have congenital scoliosis

 

Algorithm

 

Curve 20-40˚

- TLSO

- but bracing complicated by pressure sores

 

Curve > 40˚

- anterior and posterior fusion

- need anterior as posterior elements deficient

- pelvis may be deficient

- attempt to preserve lumbosacral segments to aid wheelchair transfers

 

Duchenne's muscular dystrophy

 

Incidence

 

Usually lose ability to walk during age 9-12 years

- due to progressive weakness

- 80 - 100% develop severe collapsing scoliosis

 

May progress rapidly (2-3˚/ month)

- can progress to 100o

- 10o per year once in chair

 

Issues

 

Affects ability to sit

 

Scoliosis is life threatening

- progressive respiratory compromise

- due to respiratory muscle weakness and curve

- with curve > 35˚, vital capacity 40% of predicted

- further compromises respiratory problems

- death occurs in late teens to early twenties

- may be delayed by curve correction

 

Bracing

 

Orthotic use little success

- almost all progress

- slows progression (15˚ / year comparted with 30˚ / year)

 

Surgery Indications

 

Curve greater than 30o

FVC < 30% predicted

Life expectancy > 2 years

 

Surgery

 

Only posterior

- anterior compromises respiratory function too much

 

Spinal muscular atrophy

 

Most patients develop scolioses

- onset by age 7

- usually progressive

- consider surgical treatment when curve reaches 40o

- large C shaped curves

- T2 to sacrum

 

Friedreich's ataxia

 

All have scoliosis

- 50% have hyperkyphosis

- curve patterns resemble idiopathic rather than neuromuscular

- curves appear later

- late teens or early 20's

- consider surgical treatment when curve reaches 40o

 

Paraplegia and quadriplegia

 

Incidence

 

Age related with risk of spinal deformity

- 90% if cord injury preadolescent

- due to loss of sensory / proprioceptive / motor functions supporting the spinal column

 

Posttraumatic angular deformities related to crush fractures

 

 

 

Other Causes Scoliosis

 

 

Achondroplasia

Background

 

Among the skeletal dysplasias, spinal deformity is seen most commonly in patients with achondroplasia. 

 

Issues

 

1.  Thoracolumbar kyphosis

 

Occurs in almost all patients prior to walking

- non rigid, likely due to hypotonia

- resolves with ambulation

 

Bracing may help to prevent clinically significant kyphosis

- persists in approximately 15% to 20% of patients

 

2.  Spinal Stenosis

 

Pathology

- Short pedicles

- narrow interpedicular distance 

- Stenosis may be present at any level from the foramen magnum down

- most commonly L1-S1

 

Symptoms

 

Over 50% of patients with achondroplasia have back pain

- other symptoms may include leg pain, claudication, paresthesias, weakness, and paraplegia. 

- Bowel and bladder difficulties may occur with stenosis in the lower thoracic spine

 

Symptoms usually occur in early adulthood and are slowly progressive

- Occasionally, symptoms may arise in childhood

 

Surgical decompression

 

Wide, multilevel laminectomies

- Disc removal usually is not indicated 

- unless radiculopathy is present

- Early surgical intervention leads to a better outcome

 

Postlaminectomy instability may occur

- fusion probably should be added in cases of multilevel decompression

 

3.  TL kyphosis + stenosis

 

Anterior decompression and strut grafting should be added

 

Irradiation Induced

Mechanism

 

Vertebral bodies grow axially by endochondral ossification. 

- radiation can inhibit this

 

Incidence

 

Spinal deformity is the most common side effect of abdominal irradiation

- Children irradiated at age 2 years or younger are most affected

 

Study of Wilm's tumour patients

- 7 x incidence scoliosis

 

Reported 10 to 100%

 

May get LLD

- femoral head

- ilac crests

 

Effects

 

Irradiation of the entire vertebra

- produces a symmetric hypoplastic vertebral body

- without significant malalignment

 

Irradiation of a portion of the body

- causes segmental loss of growth 

- resultant scoliotic deformities

 

Soft-tissue fibrosis and contracture may be a cause of spinal curvature

 

Management

 

Milwaukee brace treatment 

- indicated in a young child with a flexible curve caused by irradiation

 

Early posterior fusion and instrumentation is recommended

- Repeat bone grafting may be necessary

- need longer duration of postoperative immobilization 

- several authors recommend a duration of up to 1 year

 

 

Marfan's

Marfan's Syndrome

 

Epidemiology

 

Spinal involvement occurs in approximately 75% of patients with Marfan's syndrome

- scoliosis is the most common spinal deformity

- the cervical spine is almost always normal

 

Those who develop scoliosis do so by 9 years of age

- one half of the patients develop it by age 6 years

 

Pathology

 

Curve patterns resemble idiopathic scoliosis

- frequent progression during adolescence

 

Despite the patients' generalized ligamentous laxity, many of the curves are quite rigid

 

Thoracic lordosis

- second most common spinal deformity

- associated with loss of normal lumbar lordosis

 

Progression

 

These curves also tend to be painful, progressive, and often cause respiratory problems

 

Curve progression rates average 7 - 10o per year

- with the most rapid increase occurring during the early adolescent period

 

Management

 

Non operative

 

Brace treatment

 

Controversial

- most patients fail brace treatment

- orthotics may be used as a holding device for patients who eventually will undergo fusion

 

Operative

 

Technique

 

Posterior spinal instrumentation and fusion 

- mainstay of treatment

- a high rate of pseudarthrosis can be expected

- may lower with addition of an anterior fusion, abundant bone grafting, and postoperative immobilization

 

Complications

 

Pseudoarthrosis

 

Cardiac

- high incidence of malformations

- need good pre op work up

 

Superior mesenteric artery syndrome

- high incidence if put in jacket

 

 

 

 

 

 

 

NF

Epidemiology

 

2% scoliosis patients have NF

 

10 - 30 % NF patients have spinal deformity

- most common skeletal manifestation

- mostly non dystrophic variety

 

Types

 

A.  Non dystrophic

- vertebral wedging, angulation and rotation

- very similar to idiopathic scoliosis

- right thoracic

 

B.  Dystrophic

 

Cause

- may be due to intraspinal lesions such as tumours, meningoceles and dural ectasia

- can simply be bony dysplasia

 

Characterised by

- short segments, sharp angulation with severe apical rotation

- scalloping posterior margins

- widening of the spinal canal

- enlargement of neural foramina

- widened interpedicular distance

- thinned defective pedicles and lamina

- paraspinal mass

- rotation of ribs - 'pencilling'

 

Other problems

 

Cervical spine kyphosis

 

Atlantoaxial dislocation 

- has been reported in five patients

- flexion and extension views are important in assessing cervical instability

- needed pre-op

 

Kyphosis

- marked acute posterior angulation

- can develop myelopathy and even paralysis

 

Intraspinal Tumours

 

Dural Ectasia

- circumferential dilatation of dural sac

- contains CSF and brown material

- erodes osseous structures

- very thin lamina

- can cause great angular deformity

- may have multiple dumbbell appearances

 

Dumbbell lesion

- single neurofibroma

 

Meningocoeles / pseudomeningocoeles

 

Management

 

Non Operative Management

 

Brace treatment

 

Not been effective in dysplastic curves

Can use early in non dysplastic

 

Operative Management

 

Non dystrophic

 

Issues

 

Recommend posterior fusion for progressive

- lower threshold for surgery than in idiopathic

- potential for progression much higher

 

Surgery as for idiopathic

 

Idiopathic

 

Brace 20 - 35o

Surgery > 35o

 

Dystrophic

 

Issues

 

A.  All dystrophic curves need MRI to assess intraspinal lesion

 

B.  All need C spine screening before surgery

- these patients also often have cervical spine abnormalities and instability

 

C.  Incidence of pseudoarthroses ranges from 6% to 38% with isolated posterior fusion

- recomment addition of anterior fusion for dysplastic scoliosis is therefore recommended

- anterior release then posterior fusion

 

Indications

 

Anterior release and fusion followed by posterior fusion if > 50o

 

Kyphoscoliosis

 

Posterior fusion alone is usually insufficient in patients with kyphosis

 

Decompression of the cord with laminectomy 

- contraindicated because the lesion is usually anterior

- removal of the posterior elements predisposes the patient to further postlaminectomy kyphosis.

- need decompression and fusion

 

> 50o

- brace early

- need anterior and posterior fusion

- may even need brace post surgery

- if have myelopathy symptoms may also need decompression

 

Lordoscoliosis

- in dystrophic

 

 

 

 

 

 

Osteogenesis Imperfecta

Incidence

 

Spinal deformities are found in 20% to 80% of patients with OI

 

Risk factors

 

Severe disease with nonambulatory status

 

Progression

 

Scoliosis in OI may progress after skeletal maturity 

- may be related to weakened osteoporotic bone 

 

Management

 

Non operative

 

Bracing

 

Poor results

- risk of rib fractures

 

Operative

 

Posterior segmental instrumentation / arthrodesis 

 

A progressive curve that has reached 35 - 40o should be fused

- regardless of the patient's age

- significant correction cannot be expected

 

Augmentation with segmental sublaminar wiring

- to improve purchase in the osteoporotic bone

 

OI Scoliosis

 

 

Scoliosis Classification

Definition

 

Lateral curvature of the spine with coronal plane deformity > 10°

 

Types 

 

1.   Structual

 

Passively non-correctible deformity of a spinal segment 

- vertebral rotation at the apex

 

2.   Nonstructural (CHIPS)

 

Secondary or compensatory scoliosis

- no rotational component

- fully passively correctable to the midline

 

Compensatory

Hysterical

Irritative

Postural

Sciatic

 

Compensatory

- leg length discrepancy

- hip flexion contracture

 

Hysterical

- teenage females

- no rotation or wedging on XR

 

Irritative

- painful condition of spine

- fracture, tumour, infection

 

Postural

- childhood

- slight correctable curve

- disappears on recumbency

 

Sciatic

- irritation of nerve root by HNP or tumour

- secondary to paravertebral muscle spasm

 

Structural

 

Idiopathic  75%

 

Commences before skeletal maturity

- diagnosis of exclusion

 

No features to categorise it as 

- Congenital

- Neuromuscular

- Other 

 

Types

- Infantile < 3

- Juvenile 3 - 10

- Adolescent 10+

 

Neuromuscular 10%

 

Neuropathic

- UMN:  CP, FA, trauma

- LMN:  Polio, SMA, Spina Bifida 

 

Myopathic 

- Muscular Dystrophy

- Myotonica

 

Congenital (10%)

 

Failure of Formation

- wedge vertebra

- hemivertebra

 

Failure of Segmentation

- unsegmented bar

- block vertebra

 

Mixed

Complex

 

Other (5%) NAOMI

 

Neurofibromatosis

 

Skeletal dysplasias

- Achondroplasia

- SED

- Mucopolysaccharidoses

- Diastrophic Dwarfism

 

Osteogenesis Imperfecta

 

Collagen disorders

- Marfan's

- Ehlers Danlos

 

Traumatic - fractures, surgical

 

Infective - vertebral osteomyelitis

 

Irradiation

 

Metabolic

- rickets, juvenile osteoporosis, osteogenesis imperfecta

 

Tumour - osteoid osteoma

 

Screening

 

Incidence of curve >30° is 3/1000 in USA

 

Screening controversial

- cost vs benefit

- increases health costs by 20% vs late fusion for severe disease only

- if believe that bracing works, then should believe in screening before Peak Height Velocity to decrease severe scoloiosis

- if don't believe in bracing, treat scoliosis as it comes later

- it is debatable whether condition is common enough to merit screening

 

WHO 5 Features of Screening Programme

1. Condition should be important problem

2. NHx should be known

3. An acceptable screening test

4. Acceptable treatment available

5. Programme should be cost effective

 

Screening Methods

 

1. Adam's Test

- bend forward with arms free

- Interobserver error

- no threshold value

 

2. Inclinometer / most common

- assess angular rib hump

- 7° threshold

- will miss 12% 20° curve, but decreases referral rate to 3%

- less sensitive

- more specific

 

 

 

Spinal Dysraphism

Diastomatomyelia

Definition

 

Fibrous / cartilage / bony bar creating a longitudinal cleft in the cord

 

Site

 

Mainly in lumbar spine / can occur in thoracic

 

Symptoms

 

Can lead to cord tethering with associated neurological defects

 

Signs

 

Hyperreflexia / clonus / asymmetric abdominal reflexes

 

Xray

 

Inter-pedicular widening / bony bar / spina bifida occulta

 

MRI

 

May see 2 hemicords

- each within a single dural sac

- each within their own dural sac

 

Conus Medullaris often extends below L2

 

Management

 

Asymptomatic

 

Observe

 

Neurology

 

Should be resected

 

 

Spina Bifida General Principles

Definition

 

1. Spinal dysraphism

 

Failure of the neural tube to close (i.e. spina bifida + failure of neural tube development)

 

May see one of 5 telltale skin defects 

- tail

- skin dimpling 

- sacral pit 

- hair tuft 

- lipoma 

 

Myelodysplasia: Any developmental defect of the spinal cord 

 

2. Spina Bifida Cystica

 

Vertebral laminae absent with prolapse of neural elements 

 

A.  Myeloschisis / Myelocoele

- neural plate material spread out over the surface (most severe form)

 

B.  Myelomeningocoele

- prolapse of cord & dura in defect (commonest)

 

C.  Meningocele

- prolapse of dura only

- usually no neurological deficit 

 

3. Spina Bifida Occulta

 

Congenital spinal disorder where the two halves of posterior arches fail to fuse leading to bony abnormality

- defect is hidden 

- may have diastematomyelia 

- seen in 5-10% of radiographs in normal population 

- not really related to spina bifida cystica and probably not prevented by folate

 

Epidemiology

 

1 / 1000 live births 

- varies with geographic location 

- Ireland, England, Wales - 5/1000

- Australia & USA 1/1000

 

1:20 with one affected sibling 

1:10 with second affected sibling

 

Girls > boys 

 

Aetiology

 

Multifactorial nature 

- polygenic inheritance with teratogenic enviromental factors 

 

Decreased RBC folate associated with increased risk 

- avoid folate antagonists

- folate taken prior to conception reduces rate of spina bifida by 70%

- need to take before first month of pregnancy

- 0.4 mg per day normally

- 4mg per day if have a previous child with SBC

 

Pathogenesis

 

Early problem 26th gestational day (24-28)

 

Two theories

A. Failure of neural tube to close  

B.  Initially closed tube which reopens due increased intraluminal pressure

 

The latter theory is favoured & explains other defects such as diastematomyelia etc

 

Prenatal Screening

 

Should be able to detect 90% fetus with an exposed neural defect

- small skin covered defects may be missed

 

Maternal alphafetoprotein increased 

- test at 16-20 weeks

- detects 90% of spina bifida

- will justify other investigations

- amniocentesis (spontaneous abortion risk 1:200)

 

Ultrasound

- vaginal at 10-12 weeks will identify anencephaly & some spina bifida

- standard 16-18 weeks identify anencephaly & 80% spina bifida

 

Management of early detection

- deliver by C section to avoid contamination by vaginal flora

- secondary infection which then can lead to loss of level

 

NHx

 

Survival

- without repair nearly 100% will die

- especially in pre-antibiotic day

- increase survival to 50% at 25 years

- antibiotics / selective closure / shunting

 

Neurology

- not static & there is often a decline during childhood especially first 4 years

- 60% have neurological deficiency in upper limb

- this is due to syrinx / Arnold Chiari / hydrocephalus

 

Function

- only 30% are functionally independent as adults

- only 30% will have full or part-time jobs

- 65% will have normal intelligence

 

Wheelchair

- 100% if L1 or L2 or higher

- 80% if L3, L4, L5

 

Modified Asher & Olson Classification

 

Lowest level at which Grade 3 power is available i.e. antigravity power

 

Thoracic: no grade 3 strength in lower leg muscles

L2: hip flexion

L3:      knee extension

L4:      knee flexion

L5:      ankle dorsiflexion

Sacral: ankle plantarflexion

 

i.e. L4 and below are most likely to walk

 

Clinical Features

 

1. Hydrocephalus 

 

Incidence 90% 

- of these 80% will require shunt

- inserted at time of the defect closure

 

IQ normal if no hydrocephalus 

 

Must constantly be on lookout for blocked shunt 

- deteriorating neurological function

- bulging fontanelle

- poor muscle function

 

2. Hydromyelia / Syringomelia / Syrinx 

 

Incidence 50%

- related to hydrocephalus 

- fourth ventricle communicates with the central canal of cord 

- increased hydrocephalus pushes fluid into cord 

 

Leads to

- increased lower limb paralysis & back pain

- progressive scoliosis

- weak upper extremities with increased tone

 

Usually settles with V-P shunt 

 

May need drainage prior to spinal procedure 

 

3. Arnold - Chiari Malformation: (Type II in 90%)

 

Displacement of medulla into cervical canal

- cerebellar tonsils herniated below foramen magnum on MRI

 

Symptoms   

- periodic apnea 

- stridor 

- weak / absent cry

- nystagmus 

- upper extremity spasm & weakness 

 

May resolve with shunt / if not then surgically decompress 

 

4. Tethered Cord    

 

Attachment of cord to meningocoele sac 

- prevents normal upward migration of the cord during growth

- almost universal to some degree

 

Only small number have symptoms

- buttock & posterior thigh pain 

- increased spasticity & weakness in lower extremities 

- progressive scoliosis the classic presentation is progressive 

- cavus foot deformity in young child 

- rapid deterioration in quadriceps power in teenager

 

Surgical release will halt progression but not restore function

 

5. Urinary Complications

 

One of major causes of morbidity & mortality in childhood from renal failure

 

Causes

- incontinence 

- hydronephrosis 

- recurrent UTI

 

Management

- intermittent catheterisation 

- bladder augmentation

 

6. Skin anaesthesia

 

Pressure sores major problem with casts

- hip spicas should always include feet

 

7. Latex Allergy

 

IgE mediated

 

Should always provide latex free operating environment 

- assume allergy and prevent development

 

Can screen with RAST (radioallergosorbent test) / skin test / IgE assay

- IgE assay most sensitive & specific

 

8. Fractures

 

Classic exam scenario / clinical photo

- supracondylar femoral fracture is commonest

 

Often present late 

- picture of infection (redness, swelling, warmth)

 

Treat with removable splints for short time as get very stiff

 

9. Upper Extremity Function

 

60% have abnormality of UL function

- usually weakness with increased tone

 

Risk factors

- high level

- multiple shunt revisions (ie cerebral damage from hydrocephalus)

- syrinx etc

 

10.  Orthopaedic Deformity 

 

Causes

- muscle imbalance 2° to UMN or LMN lesion

- intrauterine posture

- habitual posture after birth

- congenital malformation (eg club foot)

- arthrogryposis

 

Problems

- scoliosis

- hip FFD / Dislocation

- Knee hyperextension / flexion contracture / valgus

- teratological CTEV 

- cavus foot

- charcot foot

- ulceration

 

Orthopaedic

 

Goals

 

1.  Ambulation

- level dependent

 

2. Hips

- reduce hips if unilateral / low level / walk

- different to CP as not painful

 

3. Foot 

- keep shoeable & plantigrade 

- even if in wheelchair

- avoids ulcers and amputation

 

4.  Spine

- 80% develop scoliosis

 

5.  Prevent pressure areas

 

Always manage in spina bifida clinic

 

Muscle charting 

- should start 24 hours after birth

 

1/3 Complete LMN & loss of sensation & bowel control below affected level 

 

1/3 Complete lesion at some level but distal segment of cord preserved 

- mixed picture of intact DTR & spasticity 

 

1/3 Incomplete & some movement & sensation preserved 

 

Levels

 

L1 Level

 

Muscles

- iliopsoas grade 2 or better / some weak hip flexion

 

Position

- Hip flexed, abducted & ER

 

Ambulation

- HKAFO - hip knee ankle foot orthosis

- RGO - reciprocating gait orthosis, when one hip flexes the other extends

- adults usually wheelchair bound 

 

L2 Level

 

Muscles

- iliopsoas, sartorius & adductors grade 3 or better

- hip flexion & some adduction

 

Position

- hip flexed and adduction 

- FFD of hip may develop 

 

Ambulation

- most ambulate as children in HKAFO

- FFD may need to be corrected first 

- adults usually choose wheelchair 

 

L3 Level

 

Muscles

- quadriceps grade 3

 

Position

- hip flexed & adducted

- knee extended 

- Hip subluxation & dislocation common due to unopposed hip adduction & flexion 

 

Ambulation

- usually household ambulators 

- grade 4 quadriceps - 80% community ambulators / 98% household ambulators 

- grade 3 quadriceps -  need KAFO to walk & 88% in wheelchair 

- patients with quads & good abductors can ambulate without aids 

 

L4 Level  

 

Muscles

- medial hamstring grade 3

- Tibialis anterior 3 or better

- quadriceps grade 5

 

Position

- hip flexed & adducted

- knee extended

- ankle in varus due to T anterior

 

Ambulation

- walk in AFO 

 

Surgery often required to maintain hip & knee extension / foot plantigrade

 

L5 Level

 

Muscles

- lateral hamstrings >=3

- G medius >=2

- T posterior >=3

 

Position

- calcaneovalgus or calcaneus foot 

- need T anterior to T Achilles transfer age 3 to prevents foot drop gait

 

Ambulation

- 95% community ambulators throughout life 

 

Sacral Level 

 

Muscles

- Gastroc/Soleus >/=2 

- G medius >/=3

- G maximus >/=3

 

Position

- clawing of toes & cavus foot from intrinsic minus

 

Ambulation

- usually brace free but may need special shoes 

- ie arch supports

 

At high risk of heel ulcers due to foot imbalance / calcaneus / nil S1 protective sensation

 

 

 

 

Spina Bifida Levels

Thoracic

 

Function

- no hip flexion

- no voluntary leg movement

 

Effect

- no ambulation

- hip flexed and ER

- feet equinovarus

 

L1

 

Function

- grade 2 psoas

 

Effect

- no ambulation

- hip adducted and flexed

- feet equinovarus

 

L2

 

Function

- grade 3 psoas

 

Effect

- hip adducted / flexed

- knee flexed

- feet equinovarus

 

L3

 

Function

- grade 3 quads

 

Effect

- hip adducted and flexed / highest risk hip dislocation

- household ambulator only with KAFO

- knee recurvatum / no hamstrings

- foot equinovarus

 

L4

 

Function

- > grade 3 quads

- medial hamstrings

- tibialis anterior grade 3

- AFO

 

Effect

- hip adducted / flexed

- reasonable ambulation with AFO

- knee extended

- foot cavovarus

 

L5

 

Function

- lateral hamstrings

- grade 3 abductors

 

Effect

- hip flexed but not adducted

- community ambulator in AFO

- calcaneocavus foot

 

S1

 

Function

- grade 3 power T achilles

 

Effect

- claw toes, poor sensation

- still get calcaneus as weak T Achilles

 

 

 

 

Spina Bifida Management

Closure of defect

 

Selection of patients for closure of the defect is controversial 

- if not treated most die of meningitis

- remainder have early closure - 50%

- closure of defect within 24 hours with VP shunt insertion

- early closure & shunting survival is 50% at 25 years

 

Most centres avoid urgent operation if

- level above L1 

- severe deformity 

- marked hydrocephalus 

 

Principles Orthopedic Management / Menelaus

 

1. Always manage in Spina Bifida clinic 

2. Select surgery appropriate to future demands 

3. Perform minimal surgery 

4. Condense management 

5. Correct muscle imbalance 

6. Consider absent sensation / bone fragility / infection 

7. Minimise immobilisation time to prevent bone loss & pathological fractures

8. Promote walking to

- allow normal bone development 

- prevent contractures

- psychological advantages

9. Surgery is soft tissue releases and tendon transfers 

10. All surgery at one sitting / SEML

11. Wait until 12 months

- spinal level & deformity evident & assessable

- most shunt & closure problems sorted 

 

Potential walkers are likely to need more sophisticated surgery

 

Ambulation Requirements

- extended hips & knees 

- plantigrade feet 

- straight spine

- strong quadriceps as general rule

- strong abductors / if no hip abductors usually need crutches or stick)

 

Sitting Requirements

- flexed knees 

- feet that are shoeable & placeable in wheelchair 

- straight spine - releases the hands for other activities other than truncal support

 

Hips

 

Concepts

- hip dislocation does not alter level of mobility only neurosegmental level does

- a painless dislocated hip is preferable to a stiff, painful, reduced hip

- reduction is dealt with as for DDH hip

 

Indications / Menelaus

 

1.  Non walkers

- no quadriceps & bilateral - never 

- no quadriceps & unilateral - sometimes 

- usually doesn't affect ability to sit in wheelchair

 

2.  Walkers

- quadriceps and bilateral - sometimes 

- strong quadriceps & unilateral - always 

 

Management

 

1.  Sharrard procedure 

- soft tissue procedure to prevent and reduce hip dislocation

- transfer psoas to GT / adductor tenotomy

- limited benefit and can compromise walking

- iliopsoas is the major driver of walking

 

2.  Girdlestone's procedure

- rarely done in spina bifida as not painful

- indicated for painful, arthritic subluxed or dislocated hips

- combine with valgising osteotomy / Schanz

 

3.  Osteotomy age 1 year 

- varus shortening osteotomy of femur 

- pelvic osteotomy if acetabular dysplasia present

 

Knees

 

Concept

 

Walker (L3 or better)

- aim for extended knee that is braceable 

- extended knee is stable position 

 

Non walker

- need knee flexion for wheelchair use

 

Extension Contracture 

 

May interfere with walking 

- serial casting best 

- if fails then V-Y quads lengthening 

 

Flexion Contracture

 

Hamstrings are usually not tight

- more common with CP

- check popliteal angle in HE/HF

 

Options

 

1.  Posterior capsulotomy and gastrocnemius releases 

 

2.  Anterior hemiepiphysiodesis with Stephens 8 plates

- treat FFD with hyperextension

 

3.  Extension distal femoral osteotomy

- near skeletal maturity with large FFD

 

Ankles & Feet

 

Concepts

 

1. Majority have deformity

- 50% equinovarus / CTEV like 

- 20% calcaneus / L5 level

- 20% normal 

- 10% planovalgus, equinovalgus, cavus & claw toes 

 

2. Aim for braceable plantigrade foot 

- almost all require a brace 

 

3.  Multiple deformites seen

- very difficult to predict the deformity

- not just due to the muscle imbalance

- can be abnormal secondary to hydrocephalus, tethered cord etc

- may be a mixture of upper and lower cord signs

- due to lesions at various levels / cerebral from ICP / high or low spinal lesions

 

Equinovarus / CTEV like

 

Varies from flexible to very rigid 

 

Splintage & casting initially 

- is not the same as CTEV

- the tendons etc are not just tight

- there is a muscle imbalance

 

All require OT / PMR at 6 - 12 months

- high risk recurrence

- may be better to resect 1 - 2 cm of tendon

 

Recurrence

- talectomy 

- triple arthrodesis near skeletal maturity

 

Calcaneus

 

L5 level most common 

 

Causes heel ulcers

 

Surgery at 3 years

1.  Divide the EDL, EHL  +/- anterior AKJ capsule release

2.  Transfer Tibialis Anterior to heel if normal & no tendo achilles function 

 

>6 years

- deformity osseous

- posterior displacement osteotomy calcaneum

 

>10 years

- triple arthrodesis for severe deformity

 

Planovalgus

 

Usually less problem than varus 

- can be managed often with AFO

- may occurs in Ankle or Subtalar joint

 

X-ray

- Cobey view

- assesses alignment of AKJ & STJ in weight bearing

- can see where malalignment is

 

Lateral tilt of ankle in mortise 

- TA to fibular tenodesis - in young child causes overgrowth of fibula due to increased blood supply

- 8 plates medially/ guided growth if sufficient growth remaining  (< 6 years of age)

- supramalleolar osteotomy with medial closing wedge if teenager

 

Subtalar joint

- Grice arthrodesis

- calcaneal siding osteotomy

- lateral calcaneal lengthening

- triple arthrodesis 

 

Cavus

 

Pressure effects major problem 

 

Options

- plantar release / metatarsal osteotomies / calcaneal osteotomy  / Jones procedure / claw toe management

- if close to maturity & significant deformity then look at Triple Arthrodesis 

 

CVT

 

< 2% of children with spina bifida

- manage as per all CVT

 

External Rotation of Tibia

 

Commonly associated with valgus ankle 

 

Requires supramalleolar osteotomy > 8 years of age if severe

 

Scoliosis

 

Most common skeletal abnormality 80%

- more common in high lesions 

 

Aetiology

 

1. Congenital

- congenital spinal deformity 

 

2. Neuromuscular

- paralysis with nstability of posterior elements 

 

3. Neurological

- hydrosyringomyelia / malfunction of VP shunt

- tethered cord 

 

Orthotics

- temporary measure to delay fusion to allow trunk height development 

- pressure sore problems 

 

Surgery

 

Indications for intervention

- failure of orthotic management to maintain curves <45°

- severely affected children will have to support trunk with upper limbs

- makes walking & sitting very difficult

 

Anterior release & fusion 

- posterior elements usually very deficient & not suitable for bone graft

- posterior approach may be difficult exposure due to previous sacral repair & skin flap

 

Kyphosis

 

Specific problem in spine in spina bifida

 

Issues

- difficulty sitting in wheelchair 

- ulceration over kyphos 

- breathing difficulties 

 

Management

- excision of kyphosis & osteotomy of spine 

- excision of distal cord (roll up procedure)

- may need extensive anterior release & full length fusion to pelvis (loss of correction is common)

 

Lordosis

 

Usually corrected by FFD / hip correction

 

 

 

Spinal Dysraphism

Definition

 

Spina Bifida is a congenital disorder in which the 2 halves of posterior vertebral arch fail to fuse

 

Dysraphism is maldevelopment of neural tube and skin

 

Cutaneous signs

 

Tuft of hair

Pigmented naevus

Scarred area

Post midline dimple / sinus

Lipoma

Tail

 

Associations

        

Cord tethering

Diastematomyelia

Cysts or lipoma of cord

Hydrocephalus

Arnold Chiari Malformation

- distal tethering may cause herniation of brain stem / cerebellum obstructing CSF flow

 

Epidemiology

 

10-30% of population

- 25% no cutaneous stigmata

- clinically significant dysraphism is rare

 

Embryology

 

Germ cell problem

- can get deformity in all three germ cell lines

 

1.  Somatic Ectoderm 

- cutaneous dysplasia

- hairy patch, naevus, dermal sinus

 

2.  Mesoderm

- vertebral dysplasia

- split in spinous process and laminal defects

 

3.  Neuroectodermal

- neural tube

- myelodysplasia, intramedullary and extramedullary growths associated with dysraphia

 

Commonest forms

 

Diastematomyelia

- spinal cord / filum terminale / both

- split sagittally by bony or fibrocartilaginous septum

 

Lumbosacral lipoma

 

Meningocele manque 

- loop of nerve root or trunk becomes adherent to dura

- then returns to cord or cauda close to origin

 

Arachnoid / Dermoid cyst

 

Tethered Cord

- tight filum terminale leading to tethered cord

- conus at level of coccyx in fetus

- upper border of L3 at birth

- upper border L2 by 5 years

 

Hydromyelia

 

Clinical Presentation

 

Vast majority asymptomatic

 

Any age from birth to maturity with

- short, wasted leg

- small foot

- cavovarus deformity

- paralytic valgus foot deformity

- trophic ulceration

 

DDx

 

Hemihypertrophy or hemiatrophy if small limb

CMT

CP 

 

Ultrasound 

 

Investigation of choice < 6 /12 presenting with midline lumbar dermal anomalies

 

X-ray

 

>6/12  

- varying degrees of spina bifida

- vertebral anomalies

 

MRI 

 

Management

 

Principles

 

Recognise the condition and arrest any further neurological deterioration

- MRI spine

- If find tethered cord etc needs neurosurgical opinion on whether release of cord worthwhile

 

Address foot deformity along typical lines once neurological deformity stabilised

 

 

 

 

 

Syringomyelia

Definition

 

Progressive and  chronic disorder associated with cord cavitation & gliosis

 

Effects

 

Muscle wasting

Dissociate anaesthesia

Scoliosis

Neuropathic arthropathy

 

Epidemiology

 

M > F

No familial tendency

 

Aetiology

 

1. Idiopathic

 

2. Hindbrain Herniation / Arnold- Chiari Malformation

- abnormal pressure & circulation of CSF

 

3. Post-Traumatic

- occurs in 3%

- ? due to subarachnoid block 2° to adhesive arachnoiditis

- more common in complete tetraplegics

- delayed presentation of deterioration

- 3/12 - 30 years

 

4. Arachnoiditis

5. Intramedullary Tumour

6. Extrinsic Compression With central cord necrosis

7. Necrotic Myelitis

 

Arnoldi-Chiari Malformation 

 

Congenital abnormality of Cerebellar Tonsils 

- herniation of medullary tonsils through foramen magnum into cervical canal

- block CSF flow through foramen Lushka & Malgagne blocked

- often need AV shunt

 

Type I  

- communicating

 

Arnold Chiari Type 1 MRI

 

Type II  

- non communicating

- usually larger and associated with spina bifida

 

Arnold Chiari Malformation with syrinx

 

Pathology

 

Cord damage

- anterior dissection of cavity with interruption of decussating spinothalamic fibres

- effects pain & temperature 

- extension into lateral corticospinal tracts 

 

Most common in lower C Spine

- may progressively extend up to medulla & down to lumbar 

- fluid-filled tubular cavity

- separate from but usually communicating with central canal

 

Spastic Paraparesis

- involvement of 2° order neurones

 

Pain with causalgia

 

Consequences

 

Dissociative sensory loss

Spastic paraparesis

Neuropathic joints especially GHJ

Scoliosis as syrinx is irritative

- scoliosis not due to muscle imbalance as neurology most commonly normal

 

Histology

 

Cystic space

- lined by thick layer of glial tissue

- gliosis with tendency to infiltrate white matter

 

Associations

 

Craniovertebral anomalies

Platybasia

Hydrocephalus

Myelomeningocoele

 

Cause of deterioration in Spina Bifida

 

Shunt malformation

Arnold Chiari

Cord Tether

Diastomatomyelia 

Neuroenteric cyst

Syrinx

 

Clinical Features

 

Classic Triad

 

1.  Dissociate sensory loss in cape distribution

- loss of pain and temperature

- preservation of light touch (dorsal columns) 

- arms and trunk with cervical

- legs with lumbar

 

2.  Muscle wasting / weakness with diminished reflexes 

- in UL due to direct pressure on anterior horn cells

 

3.  Atypical high thoracic kyphoscoliosis

 

Site of syrinx

 

1.  Cervico-Thoracic

- dissociate sensory loss in arms & trunk

- weakness & wasting of arms

- absent abdominal reflexes

 

Syrinx Thoracic MRI SagittalSyrinx Thoracic MRI Axial

 

2.  Lumbar

- dissociate lumbosacral sensory loss

- weakness & wasting of legs & pelvic girdle

- diminished reflexes / Babinski negative

- impaired bowel & bladder function

 

3.  Cervico-Medullary 

- AKA syringobulbia

- palatal & vocal paralysis / dysarthria

- nystagmus / dizziness / tongue weakness

 

Cervicomedullary Syrinx 1Cervicomedullary Syrinx 2Cervicomedullary Syrinx 3

 

X-ray

 

Spine

Base of skull

 

MRI

 

Most sensitive investigation

- main differential is oedema

- need T1 image

 

Look for obstructions

- ACM

- spinal cord tumours

 

DDx

 

Amyotrophic Lateral Sclerosis / Motor Neurone Disease

MS

Cervical Myelopathy

Thoracic Outlet Syndrome

Klippel-Feil Syndrome

Cord Tumour

Brainstem Infarct

 

Operative Management

 

Indications

 

Rapid progression of neurology

Short History

Respiratory compromise

Pseudobulbar Palsy

Pre-spinal corrective surgery

 

Technique

 

Shunt

- Syringo-Cisternal

- Syringo-Peritoneal

 

Suboccipital decompression & C1-2 laminectomy

 

Orthopaedic Implications

 

Neuropathic Joints

 

Syrinx is most common cause of UL charcot joint

- shoulder

- elbow

- hot swollen joint may be first presentation of syrinx

 

DDx

- infection

- peripheral neuropathy - DM / Leprosy / Tabes dorsalis

 

Scoliosis

 

Scoliosis is usually 1° Presentation of syrinx

- curve is idiopathic type

- often left sided thoracic

- corresponds with level of syrinx

 

Males > Females

- 25% if < 10 year old

- 20% males with scoliosis have syrinx compared with 2% of girls

 

Neurological deficit usually subtle or absent

- axillary sensation absent

- absent abdominal reflexes

 

Treatment

- must correct syrinx first

- malignant progression if syrinx untreated

- cyst rupture during scoliosis surgery may be fatal

- progresses like idiopathic once syrinx treated

 

 

 

Tethered Cord

Definition

 

Conus medullaris below L2 / tethered

- unable to move freely with movement / growth

 

Aetiology

 

Thickened filum terminale (> 2mm at L5/S1)

Intradural lipoma

Diastomatomyelia

 

Surgery to close myelomeningocoele

- almost all will have signs of tethered cord at site of surgery

 

Issue

 

Stretches and damages the cord as child grows

- neurology

- pain

- difficulty walking

- foot deformity

 

Rapid progression of scoliosis

 

Clinical

 

80 - 90% of children will have cutaneous manifestation

 

MRI

 

Management

 

Open surgical release

 

Indication

- child

- neurological symptoms

- progressive scoliosis

 

 

 

 

 

 

 

 

 

Spinal Fractures

Cervical spine Fractures

 

Incidence

 

Rare

- < 1% children's fractures

 

< 7

- upper cervical

- craniocervical junction

 

> 7

- lower C spine predominate

 

Immobilisation

 

Very big heads

- will flex neck on spinal board

- need bump under T spine or

- cut out for head

 

Clinical

 

Beware distracting injuries

- cannot clear C spine clinically

 

Clearance

- normal mental state

- no distracting injuries

- not intoxicated

- able to adequately communicate

- no neck pain or tenderness

- Full ROM

 

X-rays

 

Issues

 

Soft tissue swelling

- unreliable in crying child

 

Paeds C spine

- not well cleared by X ray

- much of cervical spine cartilaginous

- CT invaluable

 

Normal findings

 

C2/3 pseudosubluxation

- up to 4 mm

- common / seen in 40% < 8 years old

- reduced in extension

- Swischuk's line:  posterior arch C1 - C3, C2 shoulde be within 1.5 mm

 

ADI > 3 mm in 20% children

 

Vertebral bodies classically wedged

 

CT

 

Fast

- may not need sedation

 

Essential for C0-2 if intubated

- include in any child having CT head

 

Will pick up vast majority of unstable fractures

 

MRI

 

Show disruption of endplate / disc junction

Ligamentous injuries

SCIWORA

 

SCIWORA

 

Spinal cord injury without radiographic abnormality

- C spine very flexible

- traction injury with normal X-ray

- usually upper C spine < 8

 

Immature C spine can stretch 5 cm without fracture

- spinal cord ruptures with 5 mm traction

- it is less elastic and tethered

 

Must be aware of possibility especially with GCS <3

 

Management of obtunded patient

- unable to clear C spine

- Aspen collar will cause pressure areas / increase ICP

- MRI within 12 hours

 

Upper C spine Fractures

 

Aetiology

 

< 8

- mobile neck

- ligamentous laxity

- shallow facets

- big head

 

Usually falls and MVA

 

Ossification

 

Axis

- 3 primary

- body and two arches

- fuse age 7

 

Atlas

- 4 primary

- body, 2 arches, dens

- dens fuses age 6

- summit ossification appears 3-6, fuses 12

 

Problems

 

1.  Os ondontoid

 

Thought to be related to previous trauma

- can give C1 / 2 instability

 

2.  C0/1 dislocation

 

Terrible injury

- quadriplegia

- can be fatal

 

C0-C1

- Basion axial / Basion Dens interval

- each less than 12 mm

 

Powers ratio

- tip of basion to posterior arch (BP)

- tip of opisthon to anterior arch (AO)

- BP / AO

- > 1 anterior dislocation

- < 1 posterior dislocation

 

3.  Ondontoid Fractures

 

Pathology

- occurs at the synchondrosis

- intact anterior periosteal sleeve

 

Mechanism

- MVA deceleration injury

 

Clinical

- neurological defects rare

 

Xray

- anterior displacement

 

Management

- reduce with extension and application HTB

- 50% apposition required

- non union rare

 

Lower C spine

 

Anatomy

 

Neurocentral synchondroses fuse 3-6

Bodies wedge shaped until become square at 7

Superior / inferior cartilage end plates attached to disc

 

Pathology

 

Fractures occur between cartilaginous end plate and vertebral body

- between hypertrophic and calcified zones

 

Thoracic spine fracture

 

Uncommon

- protected by rib cage

 

Cause

- MVA, falls

- osteopenia ( OI, chemo, leukaemia)

 

Fracture / dislocations / Chance fractures

 

High energy

- usually TL junction

- lap belt injuries

- high association with intra-abdominal injuries

 

Apophyseal ring injuries

 

 

 

 

Tibial Bowing

Posteromedial Bowing

Clinical picture

 

At birth

- short tibia

- calcaneovalgus foot

- foot often lies with dorsum against anterior leg

- most bow in distal 1/2

 

Aetiology

 

Probably caused by intrauterine fracture or malposition

 

Associations

 

Calcaneus foot

- triceps surae weakness

- extension contracture of ankle

- anisomelia

 

Long term

 

1.  LLD may persist

- remains proportional 

- tend to have static LLD 2cm 

 

Treatment

- epiphysiodesis other side

 

2.  Bowing 

- usually completely resolves 

- varus 6° may persist

- resolution starts at 1 year

 

Treatment

- consider osteotomy if at 3 years if progressive or severe deformity / bow not corrected

 

 

 

 

 

 

 

 

Tibial Bowing DDx

Four forms

 

Different consequences predictable by direction of bow

 

1.  Lateral bowing

- normal variant during first year of life

- usually resolves spontaneously

 

2.  Posteromedial Bowing

- benign

- packaging defect

 

3.  Anteromedial Bowing

- fibular hemimelia

 

4. Anterolateral Bowing

- tibial pseudoarthrosis

- tibial hemimelia

 

 

 

Tibial Pseudoarthrosis

Definition

 

Anterolateral bowing

 

True bone dysplasia

- hamartomatous area in the tibia

- non union or potential non union through pathological fracture in this region

 

The defect in the bone is filled with mature, fibrous connective tissue

- not really a pseudoarthrosis

- by definition, a pseudarthrosis has a cleft lined by fibrocartilage containing fluid and bounded by a capsule

- the name is so firmly embedded in the literature that it is retained

 

Epidemiology

 

Rare

- 1:200 000

 

M = F

 

50% have NF type 1

- 1-3% of NF have CPT

 

Aetiology

 

Cause unknown

 

Theories include

- intrauterine fracture

- localised vascular abnormality

 

Pathology

 

Hamartomatous cuff present at site of lesion site

Even with NF, no clear histological evidence that fibrous tissue is NF

 

Crawford Classification

 

Type I: Non-dysplastic

- anterolateral bowing

- thickened sclerotic cortex

 

Type II: Dysplastic

 

IIA Anterolateral bowing

- wide medullary canal

- failure of tubularisation

 

IIB Cyst +/- Fracture

 

IIC Frank pseudarthrosis

- atrophied ends

 

Boyd

 

Type 1:  Congenital anterior bowing

- defect present in tibia on x-ray

- rare

 

Type II:  Congenital anterior bowing with hourglass constriction of tibia

- tapered, rounded & sclerotic

- medullary cavity non continuous on xray

- spontaneous fracture or 2° minor trauma usually < 2 years

- most common & worst prognosis

 

Type III:  Fracture develops at site of bone cyst

 

Type IV:  Sclerotic bony segment

- segment may produce complete or partial obliteration of medullary canal

- no narrowing of tibia

- fracture develops like stress fracture

 

Type VCongenitally dysplastic tibia

- mild bowing ± pseudarthrosis 

 

Type VIIntraosseous NF or Schwannoma

- ± Pseudarthrosis

- Very rare

 

NHx

 

Neurofibromatosis

- virtually all will fracture by 2 years

- therefore reasonable to prophylactically treat involved segment if has NF

 

Management Algorithm

 

No Fracture / Type I or IIA 

 

Corrective osteotomy contra-indicated

- wll not heal

- must excise hamartoma

 

Brace all

- KAFO in walking  child

- PTB in older child until skeletal maturity

 

Prophylactic surgery

- NF 

- discuss excision + graft + stabilisation

 

Fracture / Type II B&C 

 

Principle

- won't heal non operatively

- treatment is surgical

- excise segment, graft & rod  / frame

- try to avoid until > 2 

 

Surgery timing

- controversial

- delay: shorter underdeveloped leg

- older patient: increased union rate

- brace till large enough or not controlled by AFO

 

Difficult surgery

- union difficult to achieve

- high refracture and recurrence rate

- increased with residual malalignment / provides an ongoing stress riser 

- failure of OT with graft resorption associated with amputation in 50%

 

Prognosis

- varies by type

- worse with tapering and sclerosis

 

Ankle

 

Valgus deformity common

- sloping tibial epiphysis or fibular pseudoarthrosis

- can treat with guided growth

 

Surgical Options

 

1.  IM Rod

 

Several options 

- Sheffield / Fassier-Duval growing rod / Rush pin / Steinman pins

- use Steinmann pin, Rush nail or IM nail depending on size of patient & medullary canal

 

Technique

- anterior incision 

- excise hamartoma and sclerotic bone

- balance between bony resection & minimising LLD

- iliac bone graft

- stabilise with intra-medullary rod extending from calcaneum across STJ / AKJ into proximal tibial metaphysis

- with growth, rod migrates proximally & releases AKJ

- must graft & rod any co-existing fibular pseudarthrosis

 

Post operatively

- prolonged immobilisation

- spica cast for 6/12

- once united PTB till skeletal maturity

- rods left until skeletal maturity to avoid fracture

 

Results

 

Vascularised Free Fibular Graft

 

Technique

- pseudarthrosis segment resected

- contralateral fibula as graft

- can use ipsilateral fibula if no pseudoarthrosis

- stability with external fixation

 

Complication

- valgus deformity of normal AKJ 2° overgrowth of distal tibial epiphysis

- distal fibula acts as tether 

- treat with tibia / fibula synostosis 

- avoid by using ipsilateral fibula

- only possible if fibula not involved

 

Ilizarov Technique

 

Technique

- pseudarthrosis resected

 

Bone transport

- 3-level ring fixator applied

- corticotomy of proximal metaphysis performed

- middle tibial segment moved distally 

- metaphyseal lengthening 

- pseudarthrosis compression / docking

 

Indication

- child > 5

 

Disadvantage

- high rate of refracture after removal of frame

 

Electrical & US Stimulation

 

Independent use has unknown benefit 

- combine with graft / rod

 

Amputation

 

"Should not be the operation of first resort or last resort"

- severe lesions with poor prognosis

- make early decision

 

 

 

Trauma

Amputations

Indications

 

1. Congenital Limb Deformity

- 60%

- rare for UL

- PFFD / Tibial Hemimelia

 

2. Tumours 

 

3. Trauma 

- 26%

 

Considerations

 

Growth

 

Diaphyseal amputation removes one of physes

- progessive LLD

 

Aims

 

1. Preserve physis via disarticulation

2. Preserve as much bone length as possible

 

Overgrowth

 

Terminal overgrowth of stump

- occurs when appositional growth of bone exceeds that of surrounding soft tissues

- may result in penetration of bone through skin

- occurs in 10%

- most common in humerus, fibula, tibia & femur

 

Treatment

1. Stump revision with adequate resection of bone + myoplasty

2. Autogenous osteochondral stump capping 

 

Phantom Limb sensation

 

Always occurs in children

- < 10 years old settles rapidly

- not painful usually

 

Prosthetics

 

Should coincide with normal motor skill development

 

Upper limb

 

Age 4-6 months

- when sitting balance required

- passive terminal device

 

Age 2-3 years

- add active cable control with voluntary terminal opening device

- when starts object manipulating with terminal device

 

Lower Limb

 

Age 8-12 months

- terminal device

- for AKA, add knee device when child walking well

 

Age 5-6 years

- more elaborate devices

- gait retraining

 

 

 

 

 

 

Anatomy

Definition

 

Buckle or Torus fracture

- metaphyseal-diaphyseal junction

- compression fracture

- compression side has undergone plastic deformation

 

Greenstick Fracture

- diaphyseal injury

- failure tension side

- plastic deformity compression side

 

Greenstick Fracture APGreenstick Fracture Lateral

 

Plastic Deformity

- microscopic failure

- forearm and fibula

 

Ossification

 

Primary ossification centres

- diaphyseal

 

Secondary ossification centres

- epiphyseal

 

Physeal Anatomy

 

Reserve Zone

- few cells, high matrix

- cells from Groove of Ranvier

- cells quiescent

 

Proliferative Zone

- cellular proliferation / mitosis

- columns of cells

- longitudinal growth

- metaphyseal side

 

Hypertrophic zone

 

A.  Maturation

- enlarged, swollen, vacuolated

 

B.  Degeneration

- increase Alk Phos

- increases phosphate for calcification

 

C. Provisional calcification

- ECM calcify

- cell death

 

Perichondral Ring of La Croix & Groove of Ranvier

 

Surround the physis circumferentially at its periphery

 

Groove - resting and proliferating cells

 

Ring - cartilage cells that move towards the metaphysis and become contigous with periosteum

 

Salter Harris Classification

 

I.  Whole epiphysis is separated from shaft

 

II. Fracture through metaphysis and physis

 

III. Separation of part of the  epiphysis

- fracture through physis and epiphysis

 

IV. Fracture passes vertically through epiphysis / physis / metaphysis

 

V. Crushing of part or all of the epiphysis

 

Physeal fractures

 

Thought to occur primarily through hypertrophic zone

 

Reserve zone injury

- increased growth arrest with proximal tibia and distal femur

- because of undulating course of physis

- more likely damage to germinal or resting layers

 

Management

 

Do not remanipulate physeal injuries > 7-10 days

- risk injury

- exception type 3/4 

- in these anatomical reduction more important

 

Metaphyseal / diaphyseal

- can MUA up to 3 weeks after

 

 

 

 

Child Abuse / Non Accidental Injury

Definition

 

Any act or failure to act that

- results in or potentially results in harm / death / physical / emotional / sexual abuse

- by a parent or caretaker who is responsible for the child

 

4 types

 

Neglect 

Physical (punch / kick / bite / burn / shake)

Sexual 

Emotional

 

Risk factors

 

Low socioeconomic x25 risk

Unemployed

Single parent

Substance abuse

First child

Premature

Unplanned

Stepchildren

Handicapped

Parents were abused

 

Birth parents more likely to abuse

 

50-80% substance abuse

 

Epidemiology

 

Abuse second most common cause of death in infants 1-6/12

- SIDS number 1

 

85% death from child abuse < 5 years

 

10% trauma < 3 years from child abuse

 

1962 landmark paper from USA / Kempe

- battered child syndrome

- poor hygiene

- poor nutrition / failure to thrive

- ST / bone injuries

- subdural haematomas

 

History

 

Delay in presenting

 

History vague, lacking in detail, contradictory

- mechanism of injury insufficient to explain injuries

- history of a fall

 

Characteristics of child

- less than 3 years old

- poor household environment / drug / physical abuse

- overly aggressive or passive

- behavioral problems

- handicapped child

- stepchild

- premature child

- subnormal growth

 

Non orthopaedic findings

 

Skin

- bruises (buttocks, perineum and genitalia, trunk, back of head and legs)

- multiple bruises in various stages of healing

- burns (pattern may reflect mechanism of burn)

 

Head and CNS

- skull fracture (multiple, skull base, crossing suture lines, depressed fractures)

- subdural hematoma, subarachnoid hemorrhage

- retinal hemorrhage, hyphema, retinal detachment

- cognitive disabilities

 

Chest, abdomen, and pelvis

- rib fractures (posterior, multiple), sternal fractures

- pneumothorax, hemothorax

- rupture of organ (liver, spleen, or pancreas / bowel or bladder rupture)

- intramural bowel hematoma

- kidney contusion, retroperitoneal hemorrhage

- sexual abuse

 

Orthopaedic

 

Musculoskeletal system

- multiple fractures

- fractures in various stages of healing

- metaphyseal corner fracture (pathognomonic)

- femoral fracture in child < 1 (more likely than not)

- humeral shaft fracture child < 3 (almost always)

- vertebral compression fractures, spinous process avulsion

- scapular fracture

- epiphyseal separation

 

Imaging

 

Any obvious injuries

 

Skeletal survey (< 5 years age)

- AP bilateral arms

- AP bilateral forearms

- AP bilateral hands

- AP bilateral thighs

- AP bilateral lower legs

- AP bilateral feet

- AP and lateral axial skeleton and trunk

- AP and lateral Skull

 

Bone Scan

 

Costly / difficult to evaluate / lacks specificity / radiation exposure

 

DDx

 

Accidental injury

OI

Rickets

Leukaemia

Congenital syphilis

 

Caffey's disease

- infantile cortical hyperostosis

- < 5 months, fever, pain

- typically mandible

- xrays show hyperostosis

- get periostitis

 

Management

 

Failure to Diagnose

 

30-50% recurrence of abuse

5-10% mortality risk

 

Reporting is mandatory

 

Team approach

 

Paediatrician / social worker / psychologist

General surgeon / Neurosurgeon / Orthopodedic Surgeon / Ophthalmologist / Dermatologist

 

Notify / discuss with

- primary care provider for background history

- police

- legal counsel

- social services

- child protective services

 

Hospital admission to treat acute injuries and remove child from dangerous environment

 

Carefully document all records

Statement regarding level of certainty of abuse

 

Legal consent still required to treat child or release information from chart

Court custody may be needed if family members not co-operative

 

 

 

 

 

Lower Limb Fractures

Ankle Fracture

Ossification

 

Distal tibia

- appears by 2 years

- closed at maturity

- asymmetrical closure

- central initially, then posteromedial to anterolateral

- accounts for Tillaux and Triplanar fractures

 

Distal fibula

- appears by 2 years

- closes 2 years after distal tibia

 

Medial Malleolar Extension

- begins age 7

- closes age 10

 

Types of Fractures

 

SH I

SH II

SH III - medial malleolus / Tillaux

SH IV

Triplanar

SH V

 

Salter Harris I

 

SH 1 Distal Fibula

 

Incidence

- 15% of distal tibial physeal fractures

 

Mechanism

- through zone hypertrophy

- distal fibular most common

 

Management

- operative if require realignment

 

Salter Harris II                                                

 

Paediatric Distal Tibia Salter Harris 2Distal Tibial SH2 AP

 

Epidemiology

 

40% of distal tibial physeal fractures

 

Pathology

 

Thurston-Holland fragment

- large metaphyseal component

 

Non operative Management

 

Acceptable distal tibial alignment

- short leg case 6 weeks

 

Operative Management

 

Unacceptable alignment

- attempt closed reduction

- sometimes anterior periosteum can block reduction

- anterior approach / remove periosteum / reduce fracture

- if unstable screws into Thurston Holland fracture

- if fragment too small need fixation across medial malleolus into metaphysis

 

Late presentation 7-10 days

- may be best left

- prevent damage physis

- later osteotomy if needed

 

Complications

 

LLD

- complete growth arrest

 

Angular deformity

- partial growth arrest

- uneven Harris growth lines

 

Salter Harris III / Medial Malleolus

 

 

Issues

 

2 problems

- articular disruption

- possible physeal bar

 

Management

 

ORIF any > 2mm step

- physeal cannulated screws

 

Complications

 

Joint incongruity

Growth arrest

 

Salter Harris III / Tillaux

 

Tillaux Fracture CT Coronal Tillaux Fracture CT Sagittal

 

Definition

- SHIII of anterolateral distal tibia

- epiphyseal avulsion of AITFL

- supination / ER injury

 

Incidence

- usually near skeletal maturity

- transitional fracture

- as distal tibial physis is closing

- anterolateral fragment is last to close

 

Tillaux Fracture ORIF

 

Management

 

ORIF

- reduce articular step / prevent physeal bar

- anterolateral incision to reduce

- either anterolateral 4 mm cannulated screw or

- place screw percutaneously from medial side

 

Salter Harris IV

 

 

Incidence

- 25%

- usually associated with triplanar

 

Complications

- bony bar

- articular step

 

Management

 

ORIF > 2 mm step

- restore articular surface

- reduce risk of bar

 

Triplanar Fracture

 

Definition

- fracture in coronal, sagittal and transverse planes

- 2, 3 or 4 part

 

Varieties

 

2 part

- can be medial or lateral based of site of main distal fragment

- typically anterolateral epiphysis attached to posterior metaphysis

- anteromedial intact distal tibia

 

3 part

- additional separate anterolateral epiphysis / Tillaux

 

4 part

- additional separate metaphyseal fragment

 

Extra-articular

- fracture extends into medial malleolus

 

Xray

 

Type III / Tillaux on AP

Type II on lateral

 

CT

 

Type III on coronal

Type II on sagittal

3 point star on axial

 

Management

 

ORIF

- > 2mm displacement

- usually anterolateral approach to reduce

- epiphyseal medial-lateral screw

- metaphyseal AP screw

 

Complications

 

Growth arrest 10%

 

Salter Harris V

 

Usually late diagnosis

Growth arrest / LLD

 

 

 

Distal Femoral Physeal Injuries

Problem

 

1.  Undulating growth plate / higher rate of growth plate injury

- growth arrest / LLD

- angular deformity

- need to be warned

- require close and careful follow up especially in first 2 years

 

2.  Can be unstable / malunion and shortening very problematic in this area

 

Xray

 

Salter Harris II

 

Distal Femoral Fracture SH2 APDistal Femoral Fracture SH2 Lateral

 

Salter Harris II

 

SH2 Distal Femoral Fracture APSH2 Distal Femoral Fracture Lateral

 

Management

 

Undisplaced

 

Extension plaster 6 weeks

 

Displaced

 

MUA +/- ORIF

 

Low threshold to ORIF to maintain position

- already have high risk of growth arrest / LLD / angular deformity

- don't wish to deal with malunion / loss of position as well

 

MUA

 

Block to reduction

- often medial sided periosteum

- may need small medial subvastus / anteromedial approach

 

Options

 

1.  Physeal sparing metaphyseal screw in SHII

- good option if Thurston-Holland fragment large enough

 

2.  Smooth transphyseal large K wires / Steinman pin

- SHI

- SHII with small Thurston-Holland fragment

 

Complications

 

Complete growth arrest common

 

Monitor 6 monthly

- plot short and long leg lengths on Mosely chart

- distal femur contributes 9 mm / year

 

Manage LLD as per predicted difference

- usually contralateral femoral epiphysiodesis if < 5m

- may need femoral lengthening / ISKD on maturity if > 5 mm

 

Partial growth arrest / angular deformity

 

Moniter closely and investigate any possible growth arrest

 

CT / MRI

- assess percentage of bony bridge

 

Bony bridge < 50%

- excision and fat graft

- manage angular deformity with 8 plates / osteotomy

 

Bony bridge > 50%

- hemi-epiphysiodesis

- may need correction of LLD and angular deformity

- opening wedge femoral osteotomy

 

 

 

Femoral Fractures

Paediatric Femoral Fracture AP

Aetiology

 

Varies per age group

 

Trauma 60%

 

Non Accidental Injury 15 - 30%

- suspect if non walker / < 1 year

- walking status single best indicator of risk

 

OI / Pathological 10 %

 

Epidemiology

 

Bimodal distribution

- peaks at 2 and 12 years

 

Management

 

AAOS 2015 Guidelines

http://www.aaos.org/research/guidelines/PDFF_ReIssue.pdf

 

 

Algorithm

 

0 - 6 months

- Pavlik harness

- spica

 

6 months - 4 years

- spica

 

5 years - 11 years

- Length stable - flexible nails

- Length unstable - plate (bridge or open)

 

> 12 years

- Length Stable - lateral entry nail or TENS (<50kg)

- Length unstable - plate

 

15+

- IMN (lateral or piriformis entry)

 

Polytrauma/ Soft tissue (any age)

Consider external fixator

 

0 - 6 months

 

Aetiology

- difficult delivery

- OI

- NAI

 

Management

 

Pavlik harness

- very small baby

Spica

 

Good outcomes in both

https://www.ncbi.nlm.nih.gov/pubmed/15308892

 

More skin complications in spica

 

6 months - 4 years

 

Paediatric Femur Fracture

 

Algorithm

- < 2 cm short / hip spica

- > 2 cm traction then hip spica

 

Emerging evidence that pre-spica traction unnecessary

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2686814/

 

Traction

 

Gallows / Split Russell (90/90)

- child under 2 years and 25 pounds

- maximum 5 pounds traction

- can get NV compromise

 

Application Hip spica

 

Contra-indication

- abdo injury

- head injury (high spasticity)

 

Technique

- under GA, on spica table (posterior thorax on post)

- apply stocking net over body and legs

- pressure pads over ASIS

- wool applied

- must have roll of wool over abdomen to give space to breath

- 90/90 sitting spica (knees 90o, hip 90o), hips 30o abduction, 15o ER

- 1.5 legs: to knee on contralateral side, include ankle on ipsilateral side

- control rotation

 

Check position under II

- accept 2 cm shortening

- 15o varus / valgus

- 20o flexion / extension

- 10o rotation

 

Apply long leg, mould, check II

- soft cast first, then fibreglass, leave area for peroneal care

- may need broomstick to reinforce between legs for strength

- aim to be able to sit child in car seat

 

Post op

- weekly x-ray for 2 weeks

- usually for 6 - 8 weeks

 

Paediatric Femur Fracture Hip SpicaPaediatric Femur Fracture 1 monthPaediatric Femur Fracture 2 monthPaediatric Femur Fracture 6 monthsPaediatric Femur Fracture 1 year

 

5 - 11 years

 

Management

 

Thomas splint until definitive management

 

Definitive management

- Length stable - flexible nails

- Length unstable - plate

 

Thomas Splint / Balanced Traction

 

Femoral Fracture Thomas Splint

 

Technique

- analgesia +/- sedation

- Thomas splint with 2 fingerbreadths diameter clearance around thigh

- cushioning for leg to sit in Thomas splint

- traction bed

- application skin traction to leg, tied to end of Thomas splint, traction by twisting paddlepop stick

- weight off end of Thomas over pulley at end of bed

- this pulls splint away from ischium and peroneum

- rope taken over 2 pulleys at top of bed with weight suspended to elevate Thomas splint

- weight is tied to bed at top to prevent falling onto leg

- splint must be checked 2 x day to prevent pressure areas, needs regular oiling

 

Can be definitive management

- 6/52 - 8/52 immobilisation

- skin / respiratory / cost issues

 

Acceptable alignment

- 10o varus / valgus

- 15o flexion / extension

- 15 mm shortening

 

Flexible nails

 

Indications

- Length stable i.e simple transverse, short oblique and midshaft

- maximum weight up to 50 kg / 12 years old

 

Contraindication

Higher rate of complications with:

Length unstable

Weight > 50 kg

 

 

Pediatric Femoral Fracture SpiralPediatric Femoral Fracture Comminuted

 

Risks of flexible nails

- 17% complication rate

- LLD; Malunion; Infection ; Peroneal nn palsy

- https://www.ncbi.nlm.nih.gov/pubmed/16791069

 

Technique

- use wires 40% of diameter of diaphyseal medullary canal

- 2, 3, 4 mm nails

- i.e. if canal 10 mm wide, use 2 x 4 mm

- recommend using 2 wires same diameter to avoid rotational instability

- 3 point bend in wires to get 3 point fixation

- aim for bend at fracture site

- the more unstable pattern the more bend should apply

- medial and lateral insertion, proximal to distal femoral physis

- oblique entry with awl

- medial entry wire will pass into femoral neck

- lateral entry wire will pass into GT

- if difficulty passing make small incision to guide across reduced fracture site

- cut off, tap in slightly further, leave 1.5 cm out so can retrieve

 

Post op

- early mobilisation with crutches

- TWB

 

Removal & Follow-up

- when fracture united

- about 6 weeks

- need to remove early to avoid injury from protruding ends on soft tissue

- follow up for 2 years

- check leg lengths

- average overgrowth 1 cm if less than 10 years old

 

Plate

 

Indication

 

Length unstable fractures -

spiral / comminuted / very distal or proximal

 

 

Concept introduced by Sink et al

https://www.ncbi.nlm.nih.gov/pubmed/16199934

 

Higher rate of complications /reoperation when TENS used

 

Options

1) Open Plating

Paediatric Femoral Fracture Plate

 

2) Bridge Plating

 

Technique

- supine or traction table

- incision 5cm proximal or distal (depending # site)

- blunt dissection to periosteal layer

- run bristow submuscularly

- prebend 4.5mm plate using intraop xrays as template

(higher rate complications with small frag)

- place plate submuscularly

- use stab incisions to place 3 screws above /below fracture

- sufficient spread

 

Postop

- TWB / PWB for 6/52 until union

- Removal at 6/12

 

Results

- trend towards lower rates of reoperation and less blood loss than open

https://www.ncbi.nlm.nih.gov/pubmed/23752149

 

External Fixation

 

Indication

open wounds as temporary stabiliser

 

Complications

- pin site infection

- malunion

- refracture post removal

https://www.ncbi.nlm.nih.gov/pubmed/10823615

 

12 years and over

 

Issues

- less potential to remodel (especially > 10)

- usually too heavy for flexible nails

 

Acceptable alignment

- varus / valgus 5o

- flexion / extension 10o

- shortening 1 cm

 

 

Options

 

1. Flexible nails

- < 50 kg

 

2. Plate

 

Advantage

- very safe option

- avoid growth plates

 

Disadvantage

- incidence of refracture with plate removal

 

3.  Antegrade Lateral Entry Femoral Nail

- increasing use in older heavier patients  

- entry point distal and lateral to tip of GT

- AVN rates - piriformis (2%) Vs Trochanteric (1.4%) Vs Lateral trochanter (0%)

https://www.ncbi.nlm.nih.gov/pubmed/21572274

 

 

4.  External Fixation

- declining use due to concern with complications

e.g. malunion / refracture

 

> 15 years old

 

Standard antegrade nail 

- safe

- no risk of AVN

Neck of Femur Fractures

Epidemiology

 

11-18 years

 

Aetiology

 

Usually severe trauma

 

Ossification

 

Head 5-6 months (1)

GT 2-5 years    (5)

LT 9-13 years  (9)

 

Delbert Classification

 

Type 1

- transepiphyseal separation / fracture

- rarest

- associated with hip dislocation

- AVN 100%

 

Type 2

- transcervical fracture

- commonest 50%

- AVN 50%

 

Type 3

- basicervical

- second most common 30%

- AVN 30%

 

Type 4

- intertrochanteric fracture

- AVN 10%

 

Management

 

Type I 

 

Undisplaced

 

Rare situation

- spica

- watch carefully for displacement

 

Issue

- would like to stabilise

- any fixation must cross physis

 

Displaced

 

Emergency

 

Anatomical reduction

- single closed attempt

- open / Watson Jones approach

 

Fixation

- < 3 years K wires

- > 3 years cannulated screws crossing physis

 

Spica post op < 10 years old

 

Type II 

 

Undisplaced

 

Hip spica

- need to watch carefull

 

Displaced

 

Anatomical reduction

- closed + capsulotomy

- open

 

Fixation

- < 3 years K wires crossing physis

- > 3 cannulated screws crossing physis

 

Spica post op < 10 years

 

Type III

 

Undisplaced

 

Hip spica v ORIF

- displacement a risk

- can leave fixation short of physis

 

Displaced 

 

Closed reduction

- < 3 years K wires

- > 3 cannulated screws / pediatic hip screw

 

Spica post op < 10 years

 

Type IV 

 

Undisplaced

 

Hip spica v ORIF

- displacement a risk

- can leave fixation short of physis

 

Paediatric Intertrochanteric Hip FracturePaediatric Intertrochanteric NOF ORIF

 

Displaced

 

Closed reduction

- < 3 K wires

- > 3 pediatric hip screw

 

Spica post op < 10 years

 

Complications

 

AVN

 

Urgent decompression of hematoma

- no real evidence

 

Options

- NWB

- bisphosphonates

- hinged articulated distracting external fixators

 

Non-union ~13%

 

Increased with non operative management

 

Treat with valgising osteotomy +/- BG

 

Growth Arrest / LLD 

 

Contra-lateral distal femoral epiphysiodesis

 

Coxa Vara

 

Valgising subtrochanteric osteotomy

 

 

 

 

 

Paediatric Hip Dislocations

Incidence

 

Very rare

 

Associated Injuries

 

SH type 1 femoral epiphysis most common

 

Paediatric Hip Dislocation

 

Management

 

Closed Reduction

 

Assess concentric reduction

Assess stability

Apply spica / traction for 2 weeks

Imaging to ensure no fracture fragments

 

Open Reduction

 

Indication

- displaced femoral epiphysis

 

Technique

- anterior approach / digastric osteotomy

- reduce eiphysis and neck

- long screw crossing physis

 

Results

 

Nirmal et al Arch Orthop Trauma Surg 2009

- 5 cases, 2 with redislocation

- recommended extreme caution post operatively in children < 10 years

 

 

 

 

Patella Fracture

Ossification

 

3 - 5 years old

 

Management

 

Undisplaced

- manage in plaster in extension

 

Displaced > 3 mm

- ORIF

 

Patella sleeve fracture

- usually small inferior fragment seen on xray

- patella high riding on xray / alta

- is actually large cartilaginous fragment avulsed with retinaculum

- can be osteochondral

- patient unable to SLR

- confirm on MRI

- requires ORIF

 

 

 

Pelvic Fractures

Anatomy

 

Bones more elastic and malleable

- absorb much more energy

 

Very thick periosteum

- can be periosteal sleeve fracture

 

Ossification

 

Triradiate cartilage fuses 13-16

 

Iliac / Ilium / ASIS apophysis

- appear as teenager

- fuse a couple of years later

- can confuse with fracture

 

Classification Key & Conwell 1951

 

1. No break in continuity of pelvic ring

A. Avulsion fractures

        1. ASIS

        2. AIIS

        3. Ischial Tuberosity

B. Fracture of pubis or ileum

C. Fractured wing of ileum

D. Fracture sacrum or coccyx

 

2. Single break in ring

A. Fracture of 2 ipsilateral pubic rami

B. Fracture near or subluxation of symphysis pubis

C. Fracture near or subluxation of SIJ

 

3. Double break in ring

A. Double vertical fractures or dislocation of pubis (straddle fracture)

B. Double vertical fractures or dislocation (Malgaigne fracture)

C. Severe multiple fractures

 

4. Fracture of acetabulum

A. Small fragment associated with dislocation of hip

B. Linear fracture associated with non-displaced pelvic fracture

C. Linear fracture associated with hip joint instability

D. Fracture secondary to central dislocation

 

Torode Classification

 

1.  Avulsion fracture

2.  Pelvic wing

3.  Stable pelvic fracture

4.  Unstable pelvic fracture

 

Associated Injuries Rang 1983

 

Local

 

Haematuria 30%

Urological / bladder 10%

Abdominal injury 11%

Perineal or gluteal lacerations 7%

 

Vascular injuries much more rare than in adults

 

Distant

 

Head 61%

Chest 9%

Upper extremity fracture 17%

Lower Extremity fracture 17%

 

Mortality rate  8%

 

Death usually not a direct result of pelvic fracture

- rather is due to associated injuries i.e. head injury

 

Examination

 

EMST / ATLS

 

Vaginal and rectal examination

 

Neurological and vascular examination

 

Management

 

Avulsion Fractures

 

Tensor fascia lata, sartorius, RF, Psoas, Hamstrings

- rarely require treatment

 

Pubic fractures

 

Exclude genito-urinary injury

 

Unilateral Fractures

- stable 

- mobilise with crutches

- weight bear as tolerated

- usually 3-4 weeks

 

Bilateral Fractures

 

Child

- if associated with posterior ring or sacral fracture

- potentially unstable

- usually doesn't need ORIF

- heals quickly with bed rest

 

Teenager 

- treat as Adult 

- ORIF where appropriate

 

Pubic Symphysis Diastasis

 

Assess

- urological injury

- posterior ring injury

 

Treatment

- heals with periosteal sleeve

- if wide should close with external fixator

- if remains widened child walks with ER deformity

 

Acetabular Fractures

 

Triradiate fractures

- uncommon

- usually from extension of adjacent rami and iliac fracture

- usually stable

 

Complication

- child < 10

- early closure triradiate cartilage

- acetabular dysplasia

 

Management

 

Tri-radiate fracture

- skeletal traction

- CT

- if severely displaced fragment ORIF with smooth pins

 

Physeal bar across triradiate cartilage

- follow up all displaced & non displaced

- consider bony bidge excision and fat graft

 

Vertical Shear Fractures

 

Unstable

- associated visceral injuries

- blood loss is substantial and should be replaced

- is rare for child to die of blood loss from pelvis compared with adults

 

Management

- 6 weeks of skeletal traction

- rarely need external fixator

 

Complication

- LLD usually < 2 cm

- contralateral hemi-epiphysiodesis

 

 

 

 

Tibial Fractures

Displaced Tibial Shaft Fractures

 

Acceptable reduction

- varus / valgus < 5o

- anterior / posterior < 5o

- rotation 5o

- shortening 10 mm

 

Poor remodelling potential

- valgus 

- apex posterior angulation  / recurvatum

- rotational alignment does not remodel

- shortening / in 2-10 year old average overgrowth is only 5mm

 

Technique

 

GA / II

- leg hanging over edge of bed

- gravity assists reduction

- apply short leg cast

- check II

 

Apply long leg cast in 45° knee flexion

- helps control unstable fractures

- prevents early weight bearing

 

Plantar flexion ankle

- mild plantar flexion for first 2-3 weeks to prevent posterior angulation

- 20° plantar flexion for middle & distal third

- 10° plantar flexion for proximal third

 

Need to observe position for first 3 weeks

 

Time in cast

- neonates 2-3 weeks

- juveniles 4-6 weeks

- adolescents 8-12 weeks

 

Toddler's Fracture

 

Toddlers Fracture Tibia

 

Definition

 

Undisplaced oblique fracture of distal tibia

- usually innocuous injury

 

DDx

 

Infection

- diagnosis aided by bone scan

- shows diffuse uptake throughout the tibia in fracture

- infection will tend to produce focal increased uptake

 

Management

 

Heal rapidly 

- can be treated in short leg weight bearing cast for 4 weeks

 

Open Fractures

 

Treated along the same principles as adult compound fractures

 

Buckley et al (1994)

- 42 cases

- average time to union 5 months (range 2-21)

- 4 patients had > 1 cm overgrowth

- 3 infections (7%) all resolved

 

Antibiotics

- first generation cephalosporin for Grade I & II

- add Gentamicin for Grade III injuries

- add Penicillin for farm yard & lawn mower injuries 

 

Compartment Syndrome 

- as for adults

- measure compartment pressures in ventilated or severely head injured children

 

Fixation

- Grade I  - reduced +/- percutaneous pinning, long leg cast once wound closed

- Grade II & III - external fixation

 

Amputation

- indications for primary amputations not clear in children 

- accepted indication for primary amputation is open tibia

- + avascular leg with posterior tibial nerve injury and insensate foot

- MESS Score useful predictor of amputation

 

Proximal Metaphyseal Tibial Fracture / Cozen's Fracture

 

Paediatric Proximal Tibial Fracture

 

Issue

- may develop long term valgus alignment

- due to medial epiphyseal overgrowth / periosteum medially

 

Management

 

Any displacement

- MUA

- extension long leg cast with varus mould

 

Management of valgus

 

A.  < 10 years with < 15o

- will usually remodel

 

B.  > 10 years with > 15o

- consider operative intervention

- guided growth plates

 

 

 

 

Tibial Tubercle Fractures

Epidemiology

 

Adolescent boys

 

Ossification

 

Proximal tibia / primary ossification centre

 

Tibial tuberosity / secondary ossification centre

- eventually merges with primary ossification centre

 

Ogden Classification

 

Type I - Tibial tuberosity ossification only

 

Tibial Tubercle Avulsion Type 1

 

Type II - Extends into proximal tibial ossification centre / non articular

 

Type II - Extends into articular surface of proximal tibial ossification centre

 

Management

 

Beware

 

Compartment syndrome

 

Popliteal artery injury

 

Associated extensor mechanism disruption

- suggest ultrasound

 

Undisplaced

 

Cast in extension for 6 - 8 weeks

 

MUA

 

Indicated in type I and II +/- type III

 

Extend knee

- often require sedation + analgesia

- assess reduction in cast

 

Operative

 

Indications

- type I & II displaced > 5 mm

- residual recurvatum

- type III / intra-articular extension

 

Technique

- open reduction + AP cancellous screws through tibial tuberosity

 

Results

 

Mosier et al J Paediatr Orthop 2004

- 19 fractures

- majority from athletics / mainly basketball

- 4 reduced and immobilised, remainder required ORIF

- 3 patients had associated extensor mechanism disruption

- 2 patella tendon avulsions and 1 quadriceps tendon

 

Complications

 

Compartment syndrome

Recurvatum

Remodelling

Poor Remodelling

 

Midshaft angulation

Older > 11 years

Large angulation > 20-30o

Rotational deformity

Intra-articular deformity

Angulation perpendicular to plane of joint motion

 

Good remodelling

 

> 2 years growth remaining

Fractures near end of bone

Angulation in plane of joint motion

 

 

 

Upper Limb Fracture

Distal Radial Fractures

IncidenceDistal Radius Fracture

 

Metaphyseal > physeal / SH2

 

Aetiology

 

FOOSH

 

Operative Management

 

Indications

 

Visible deformity

> 20o angulation

 

Options

 

MUA

MUA + K wire stabilisation

 

Indications K wire

 

Remanipulation of metaphyseal fracture

Instability

Associated with supracondylar fracture

 

Technique

 

Avoid physis if able in metaphyseal fracture

- dorsal Kapaji technique

- can use this technique laterally

 

Mercer Rang Remodelling Rules

 

Best if

- young age (won't remodel much if > 11 years)

- short distance from fracture to physis

- direction of angulation in plane of motion of joint

 

Won't correct rotation

 

Rang distal radius remodelling

- < 5      - 25-35° 

- 4 - 9    - 20-25

- 10-12   - < 15°

 

Rotational malalignment 

- best way to determine proximally is radial tuberosity 

- prominent towards the ulna with supination and disappears with pronation

- at same time radial styloid distal will be prominent with supination

 

Remanipulation

 

Never for physeal injuries

Up to 3/52 for metaphyseal injuries

 

Complications

 

Growth arrest

 

SHII DR

- around 1%

- need to warn parents to represent if child develops angular deformity

 

Do not MUA or repeat MUA after 1 week

- only reinjure growth plate

- will remodel extremely well

- if severe, simply plan for osteotomy later if required

 

Physeal injuries distal ulna

- high incidence of growth arrest

 

Metaphyseal Radius

- incidence growth arrest also 1%

 

Distal Radius Growth Arrest Original InjuryDistal Radius Growth Arrest K wire

 

Distal Radius Growth ArrestDistal Radius Growth Arrest

 

 

 

 

Elbow

Centres of Ossification

Age of appearance in girls

 

C (capitellum)               1

R (radiua)                     3

I  (internal epicondyle)   5

T  (trochlea)                  7

O  (olecranon)               9

E  (external epicondyle) 11

 

Boys: 2 years later in every centre except capitellum

 

Closure

 

14 - 15 in girls

15 - 18 in boys

Distal Humeral Physeal Separation

Pathology

 

Children < 6

- entire distal humerus physis is displaced

 

Xray

 

Distal physis not ossified < 1 year

- may be a difficult diagnosis

 

Radius and ulna lose normal association with distal humerus

- appears like an elbow dislocation

- disruption of radio-capitellar line

 

Diagnosis

 

MRI

Arthrogram

 

Management

 

MUA + cross K wires

 

 

 

Lateral Condyle Fractures

Epidemiology

 

Average age 6 years

 

20% distal humeral fracture

- second most common elbow fracture after supracondylar

 

Mechanism

 

Pull Off 

- more common 

- fracture begins posterolateral metaphysis

- LCL, ECRL & ECRB attached to fragment

 

Push off

- varus force to extended EJ

 

Classification

 

Anatomical

 

Milch I 

- SH IV through capitello-trochlear groove

- lateral wall of trochlear still attached to shaft

- elbow stable

 

Push off

- fracture line crosses ossification centre capitellum

- tend to physis arrest

- tend to be stable

 

Milch II

- SH II into trochlear notch

- lateral wall of trochlear goes with distal fragment

 

Pull Off

- tend to be unstable

- may be difficult to interpret on Xray

 

Displacement ~ Wadsworth

 

Stage I -   Minimally displaced with articular surface intact

Stage II -  Displaced

Stage III - Fragment rotated

 

Clinical Features

 

Age 6 

History fall

Lateral pain

Swelling

 

X-ray

 

Undisplaced

- typically metaphyseal flake

- looks minimally displaced on AP and lateral

- perform an internal oblique x-ray to exclude displacement

 

Displaced

- obvious

 

CT / MRI

 

Management

 

Non operative

 

Indication

 

Undisplaced 

- rare

- check with internal oblique xray

- splint in neutral

- watch with serial xray +++

 

Operative

 

Surgical indications

 

1.  Unstable

- Milch 2

 

2.  Displaced

- > 2 mm

 

ORIF

 

1.  Closed reduction

 

Not usually done

- fracture is intra-articular

- want to reduce under direct vision

 

Technique

- reduce by extension / pronation / varus

- pronation uses flexor mass to pull lateral condyle forward

 

2.  Open reduction

 

Lateral approach distal humerus

- between BR / ECRL and triceps

- distally can extend limited amount between ECRL and ECRB

- don't dissect posteriorly to protect blood supply

- don't need to dissect distal fragment

- anterior homan to elevate anterior capsule

- can see across distal joint line and perform anatomical reduction under vision

- one K wire horizontal across fracture into trochlea

- one K wire up lateral column

- bury K wires as need to be in for 6 weeks

 

Post op

- very real risk of non union

- elbow in POP for 6 weeks

- don't remove K wires until obvious union

- do so usually in OT

 

Late Presentation

 

Presentation after 2-3 weeks

 

Option

 

1. Reduction and ORIF

- documented risk AVN / Physeal Arrest

- exact incidence / risk is unknown

- best option if fragment is displaced and mobile

- may do up to 6 weeks to 6 months

- controversial

 

2.  Bone graft and screw in situ

- later osteotomy as required

 

Complications

 

Generally do poorly compared to supracondylar fractures

 

Reasons

- intra-articular fracture

- transphyseal fracture

- inherently unstable

- tenuous blood supply (AVN)

- synovial fluid in fracture site (non union)

- often need ORIF - more scarring

 

1.  Non-Union

 

Cause

- poor vascularity

- synovial fluid

- muscle pull

 

Prevention

- leave wires and immobilisation for 6/52

 

Diagnosis

 

Xray

- plain film oblique view

- collar like projection of posterolateral metaphysis attached to physis that hasn't united

- delayed union if present at 5/52

- non-union if present at 3/12

 

CT

 

Presentation

- pain

- loss of ROM

- cubitus valgus

- tardy ulna nerve palsy

 

Treatment

 

Controversial

- high rate of failure of attempted fixation 

 

1.  Non displaced non union

- POP til 3/12 mark

- then graft metaphyseal non-union + screw

 

2.  Displaced non-union

A.  Reduce / graft / screw

- though to be acceptable if < 6 months and fragment mobile

B.  Graft in situ & screw

- later osteotomy for malunion

 

2.  Cubitus Varus

 

2° overgrowth lateral condyle

- common problem

- little cosmetic or functional problem

 

3.   Malunion

 

Milch II malunion

- varising & lateral translation osteotomy

 

Milch I malunion

- varising osteotomy

 

4.  Fishtail Deformity

 

Causes

- 2° Trochlear AVN

- central growth arrest

 

Issue

- usually of little consequence / no need for treatment

- can lead to OA

 

Trochlea AVN

 

5.  Cubitus Valgus and Tardy Ulnar Nerve Palsy

 

Average 22 years later

- from AVN / nonunion / physeal arrest / malunion

- 47% associated with lateral condylar fracture

 

Management

- anterior transposition nerve

- varus osteotomy will not help nerve

 

6. MO

 

 

 

 

Little League Elbow

Definition

 

Medial epicondyle physeal injury

 

Aetiology

 

Overthrowing / valgus overload

 

Pitching

 

Pathology

 

Medial epicondylar epicondylitis / apophysitis

- traction / fragmentation

 

Medial epicondylar separation

 

UCL injury

 

Capitellum osteochondritis dissecans

 

Management

 

Rest

Limit pitch counts

 

 

 

Medial Epicondyle / Condyle Fracture

Medial Epicondyle

 

Ossification

 

Apophysis appears around 7 years

Ossifies age 16 years

 

Normal apophysis may be some distance from shaft

- rarely may be fragmented

 

Aetiology

 

Elbow Dislocation Medial Epicondyle Fracture

 

Dislocation

Valgus deformity

 

Operative Indications

 

Absolute

- entrapped in joint

- ulnar nerve injury

- elbow dislocation

 

Controversy

 

Medial Epicondyle Fracture

 

Displacement > 5mm 

- good outcomes reported up to 10 mm

- clinically have some valgus instability

- non significant to most patients

- may be significant in the throwing athlete

 

Farsetti JBJS Am 2001

- 42 patients with displacment > 5 mm

- average age 12 years

- half treated in long arm cast, half ORIF

- no difference in outcome

- poor outcome with fragment excision and ligament reattachment

- in nonoperative group all but two had nonunion radiographically

 

Entrapped in joint

 

Aetiology

- secondary to dislocation which has self reduced

 

Issue

 

< 5 with dislocation

- medial epicondyle not visible

- may be entrapped in joint

- suspect if decreased ROM / pain / non congruent reduction

- can try arthrogram

- may nee open exploration of joint

 

Management

 

Can attempt MUA

- valgus and extend wrist to tighten flexor origin

- rarely works

 

Usually require open reduction + K wire

 

ORIF

 

Technique

 

Position

- usually supine with arm table

- can place patient prone which makes fragment very easy to reduce

 

Medial incision

- find and protect ulna nerve

 

Reduce fragment anatomically

- ORIF with K wires or screw

 

Medial Condyle Fracture

 

Epidemiology

 

Rare

- can be difficult to diagnose

- trochlea may not be ossified

- looks like medial epicondyle injury

 

Management

 

ORIF if displaced > 2 mm

- intra-articular fracture

- may require MRI

 

Complications

 

Nonunion / cubitus varus

Trochlea AVN

Medial overgrowth / cubitus valgus

 

Monteggia

Paediatric Monteggia APPaediatric Monteggia Lateral

 

Definition

 

Fracture / plastic malformation of proximal ulna with dislocation of radial head

 

Xray

 

Radio-capitellar line disrupted

 

Bado Classification

 

By position Radial Head

- APLF

 

Type I 60%

 

Monteggia Fracture Anterior

 

Radial head anterior

- associated with PIN palsy

- immobilize in flexion and supination

- remember in pronation tend to sublux both DRUJ & PRUJ

 

Type II 15%

 

Radial head posterior

- associated with ulna nerve palsy

- immobilize in extension and pronation

- commonest in adults

 

Type III 20%

 

Radial head lateral

- immobilize in flexion and supination

 

Type IV 5%

 

Anterior with radial shaft fracture

 

Management

 

Algorithm

 

MUA Anterior Monteggia

- reduce ulna

- traction with posterior pressure on radial head

- maintain in supination

 

Unable to reduce radial head

A.  Ensure ulna fracture out to length / stabilise with K wire

B.  Plastic deformity of ulna / may need to perform osteotomy to reduce radial head

C.  May require open approach to radial head with relocation / repair annular ligament

D.  Radial head remains unstable- stabilise with strip of triceps fascia

 

Late presentation

 

Open reduction

- principles as above

- may need osteotomy or lengthening

 

Complications

 

PIN palsy

- from original injury

- will usually recover

 

HO

- late operation

 

Radio-ulna synostosis

- fractures at same level

 

 

Pulled Elbow

Definition

 

Radial head subluxation

 

Incidence

 

Average 2 - 3 years

- rare > 7

- annular ligament has better attachment after 5

- common, but exact incidence unknown

 

MOI

 

Axial traction with extended elbow 

- annular ligament subluxes off radial head

- more common with ligament laxity

 

History

 

History axial traction

Snap sometimes heard

 

Examination

 

Limb in extension & pronation 

- pain on supination

 

Pseudoparalysis

 

Tender over radial head

 

X-Ray

 

Usually normal

- mild increase radio-capitellar line > 3mm

 

US 

 

May show increase radio-capitellar distance between elbows

- > 3 mm difference suggestive of subluxation

 

Management

 

Concept

 

Pronation tends to sublux both DRUJ & PRUJ

Hence stabilise in Supination

 

Technique

 

Supination + Flexion 

 

Mild analgesia

- warn parents

- painful cry then relief

 

Feel for snap 

- classically immediate pain relief 

- child will use arm immediately

 

If subluxed for > 12/24 often 2° synovitis has developed

- pain relief not dramatic 

- may not have snap

- place in sling & review next day

- will often self reduce

 

Complications 

 

1.  Unreduced subluxations 

 

No series reports any long-term sequelae in which the subluxation is not recognized & reduced

Almost all the subluxations reduce spontaneously

 

2.  Recurrent subluxations 

 

5% to 39%

- usually respond to the same MUA as the initial injury

- ceases after 5 years of age

 

3.  Irreducible subluxations 

 

Open reduction is rarely necessary

- delineate from radial head dislocation which need reduction

- MRI if unreduced after 2/52

 

 

 

 

 

 

Radial Neck Fracture

MechanismPaediatric Radial Neck Fracture

 

FOOSH

- valgus injury

- don't get radial head fracture as is mostly cartilaginous

 

Types

 

SH 1 or 2

 

Associated Injuries

 

MCL injury

Olecranon / Medial epicondyle fracture

 

Non operative

 

Indications

 

Normal valgus neck is 15o

 

< 30o angulation acceptable / will remodel

 

> 30o - risk radiocapitellar OA

 

Operative Options

 

MUA

- rotate arm to maximum displacement

- use thumb to reduce

 

K-wire joy stick

- pronate arm to protect PIN

- insert into fracture site

- try to lever fracture into position

- then drive into opposite cortex

 

ORIF

- may need open reduction

- then stabilise with K wires

 

 

Supracondylar Fracture

MechanismSupracondylar Grade 3 Compound

 

FOOSH

 

Epidemiology

 

Age 6 as maximum ligamentous laxity

- < 4 - physeal separation

- > 8 - dislocation

 

L > R

M > F

 

Pathoanatomy

 

Supracondylar region in 6 year old is thin

- maximally at olecranon fossa (2-3 mm)

- poor lateral & medial columns 

 

Children also tend to hyperextend at the elbows due to increased ligamentous laxity

 

With increasing hyperextension, the anterior periosteum hinge is torn

 

Clinical Features

 

S Shaped arm which is very swollen

 

Pucker sign - anterior spike impales Brachialis to skin

 

Nerve examination

 

1.  Median nerve

- sensation

 

2.  AIN

- make circle with index and thumb

- check FPL, FDP index

- most common injury

 

3.  PIN

- thumb up

 

4.  Ulna nerve

- cross fingers / spread fingers / sensation

 

5.  Radial nerve

- decreased sensation first dorsal webspace

 

Classifications

 

Flexion type 2%

 

Supracondylar Flexion Type

 

Extension type 98%

 

Gartland Classification Extension Fractures

 

1.  Undisplaced

- anterior and posterior cortices intact

 

2.  Displaced with intact posterior cortex

 

Supracondylar Grade 2

 

3.  Displaced, nil cortical contact, posteromedial or posterolateral

 

Supracondylar Grade 3

 

Modified by Wilkins

 

Type 2B Rotated

 

Type 3 A Posteromedial

 

Commonest

- distal fragment goes posteromedial

- cause cubitus varus

- injures radial nerve which is tethered over lateral spike on proximal fragment

- manage by pronation which tightens medial periosteal hinge

 

Type 3 B Posterolateral

 

Distal fragment goes posterolateral

- causes cubitus valgus

- tend to ER

- injuries median nerve & brachial artery

- these get tethered over medial spike on proximal fragment

- manage by supination which tightens lateral periosteal hinge 

 

Lateral Xray

 

Position for good lateral

- thumb vertical

- hand above elbow

 

Anterior angulation

- shaft - condylar axis

- normally 40o

 

Lines

 

Anterior humeral line 

- should pass through capitellum

 

Radio-capitellar line

- pass through capitellum

 

Teardrop 

- shadow above the capitellum

- anterior dense line: posterior margin of the coronoid fossa

- posterior dense line: anterior margin of the olecranon fossa

- inferior portion: ossification center of the capitellum

 

AP X-ray

 

Baumann's Angle

 

Technique

- angle between longitudinal axis humerus and capitellar physis

- should equal uninjured side

- 75o normal

 

Cubitus varus

- varus malposition  > 81° 

 

Cubitus valgus

- < 70o

 

Management

 

Algorithm

 

Type 1

 

Cast / collar and cuff

- flex up to splint with triceps

- 3/52

 

Type 2A

 

Non operative

- anterior humeral line contacts anterior capitellum

- no rotation

- flex elbow

- remodels in plane of joint movement

- exact amount acceptable is unknown

 

Type 2B / 3

- MUA + K wire

 

Timing of surgery

 

Mehlman JBJS Am 83A 2001

- compared < 8 hours to > 8 hours

- no increased need to open

- no increased nerve injury

- no increased compartment syndrome

- no change in outcome

 

Closed reduction in OT

 

Technique

 

1.  In line traction at 30o

- 2 minutes to overcome soft tissues

 

2.  Reduce translation / rotation / varus and valgus

- adjust carrying angle / Baumann's angle

- avoid medial impaction / cubitus varus

 

3.  Flexion pushing olecranon forward with thumb

 

4.  Hand position

- posteromedial displacement / pronation in flexion

- posterolateral displacement / supination in flexion

 

Bony landmarks

 

In extension

- medial / lateral epicondyle and olecranon all in a line

 

In flexion

- medial / lateral epicondyle with olecranon from equilateral triangle

 

Assess reduction in flexion

 

Cannot K wire without adequate reduction first

- check medial and lateral columns with oblique views

- reduction of teardrop on lateral

 

Inherently unstable if not reduced

- will slip into varus or valgus

 

K wires

 

1.  Crossed K wires 1.6mm

 

Concept

- wires must cross above the level of the fracture

- engage 2 cortices

- assess stability

 

Supracondylar fracture cross K wires

 

Medial K wire 1.6mm

- open approach

- identify and protect nerve

- extend elbow past 90o

- in some children the ulna nerve subluxes anteriorly in flexion

- entry point anterior aspect epicondyle

- K wire directly onto bone

- explore after K wire to make sure not in nerve

 

Lateral K wire inserted percutaneously

 

Extend elbow and check carrying angle

Assess stability

Check pulse and perfusion

Usually leave K wires protruding for easy removal at 3 weeks

 

2.  2 x lateral K wires

 

Advantage

- no risk to ulna nerve

 

Disadvantage

- if not correctly done, can be unstable

 

Technique

- 2 x lateral 1.6 mm K wires

- must engage medial and lateral columns

 

Surgical Issues

 

Irreducible

Absent Pulse

Excessive swelling / skin abrasions

 

Irreducible 

 

Problems

- brachialis might be trapped

- brachial artery might be in fracture

 

Management

 

Sterile tourniquet

 

Lateral approach and open reduction

- avoid posterior dissection to preserve blood supply

 

Pulseless supracondyalr

 

1.  On presentation

- urgent closed reduction + K wire

- position at 90o

- ensure pulse returns and hand pink

- observe if not pulse but hand pink

- close monitoring / ensure cast not too tight / minimal flexion

 

2. No pulse & white after reduction

 

Scenarios

A. Pulse present premanipulation / pulse lost and hand white post reduction

- likely brachial artery entrapped in fracture

- anterior lazy S approach

- expose artery

- open reduction and K wire

 

B.  No pulse before or after closed reduction

- artery either in spasm or has intimal tear

- open artery exploration

- antispasmodics trialled

- if this fails require vascular surgeon

- intra-operative angiogram may be required to diagnose thrombus / intimal tear

- thrombectomy / microvascular repair

 

Lazy S anterior approach

- transverse component just proximal to transverse crease

- proximal limb medial over brachial artery

- distal extension Henry approach

 

3.  No pulse & pink after reduction

 

Controversial

- observe for 24 hours

- arm kept warm

 

After 24 hours

- colour duplex ± MRI angiogram 

- vascular consult

- up to 70% will have a vascular injury

- risk is late Volkmann's contracture

 

Results

 

White et al J Paediatr Orthop 2010

- systemic review of perfused pulseless supracondylar fractures

- 70 - 80% had evidence of brachial artery injury

 

Noaman et al Microsurgery 2006

- 89 / 120 absent pulse returned after closed reduction

- remaining 31 explored

- traumatic aneurysm in 17 with complete transection in 8

- excision and repair / reverse vein graft primary treatments in these groups

- thrombosis and partial laceration in remainder

- these treated with thrombectomy or repair

 

Too swollen / skin abraded 

 

1.  Skin Traction

- arm straight

- weight over pulley

- body weight counter traction

 

2.  Dunlop traction

- screw in olecranon

- bent arm traction

- difficult to control rotation

 

Complications

 

Nerve Injury 

 

Incidence

- 5 - 19%

- AIN is most commonly injured

 

Indications for exploration

- post reduction nerve injury with non anatomical reduction

- open injury

- °EMG recovery at 3/12

 

Arterial Injuries 

 

Compartment Syndrome

 

Volkmann's Contractures

 

Incidence

- 1%

- usually affects FDP and FPL

 

Transfers

- BR to FPL

- ECRL to FDP

 

Malunion 

 

Issues

 

Sagittal plane deformity will remodel

Coronal plane deformity & rotation will not

 

Cubitus Varus  

- most common deformity

- mainly cosmetic problem

- can also be due to medial growth arrest

- correct with closing wedge osteotomy after skeletal maturity

 

Cubitus Valgus  

- uncommon

- may cause tardy ulna nerve palsy

- manage by transposing nerve

 

Cubitus valgus

 

Stiffness 

 

MO uncommon 

- may be secondary to remanipulation > 1/52 / later presentation

 

Stiffness usually minimal

- takes up to 1 year to regain full motion

- especially extension

 

AVN Trochlear 

 

Uncommon 

 

Produces fishtail deformity

- deepening of trocheal groove

- DDx: trochlear AVN / central growth arrest / non reduced intercondylar fracture

- can be associated with early degenerative changes

- could consider epiphysiodesis of remaining growth plates, but usually well tolerated

 

Paediatric Elbow Trochlea Avascular Necrosis

 

 

 

 

Forearm Fractures

Ossification

 

Primary

- 8 / 52 gestation radius & ulna

 

Secondary

- distal radius age 1

- distal ulna age 5

 

Non Operative Management

 

Unacceptable Position

 

< 10:  > 15o malalignment

> 10:  > 10o malalignment

 

Postreduction Positionin / Rule of Thirds 

 

1.  Fracture proximal to insertion of Pronator teres

 

Proximal fragment supinated by biceps

- supinate the forearm

- match proximal fragment 

 

2. Fracture in the middle 1/3  

 

Midposition / neutral

- biceps / pronator teres balanced

 

3. Fracture in the distal 1/3

 

Proximal fragment pronated by Teres

- pronation is the position of choice

 

Outcome

 

Usually lose more pronation than supination

- pronation can be compensated for with shoulder abduction

- supination loss less easy to compensate for

 

Operative Management

 

Options for displaced fractures

 

1.  MUA

 

Indications

- young patients < 10

- greenstick fractures

 

2.  Compression Plating

 

Gold standard

- careful protection of PIN proximally

 

Advantage

- rigid, anatomical fixation

 

Disadvantage

- increased scarring

- risk of complications with plate removal

- infection / nerve injury / refracture

 

3.  TENS Nails

 

Advantage

- smaller scars with insertion

- easier to remove than plates

- reduced risk of refracture post plate removal

 

Disadvantage

- non anatomical reduction c.f. plates

- theoretical risk of loss supination / pronation

- ? avoid > 11 - 12

- associated with tendon rupture due to prominence of nails at insertion sites

 

TENS nails technique

 

Distal radial entry

- avoid physis / proximal to physis

- skin incision

- blunt dissection to protect SRN

- entry with awl obliquely

- pass nail (preop measure diameter)

- may need small open reduction of radius

- ensures anatomical reduction

- pass nail

- leave slightly short, cut, then insert further

- need to leave some nail protruding to aid removal

 

Proximal ulna radial sided entry

- avoid ulna nerve

- avoid olecranon apophysis

- similar technique

- often don't need to open if do radius first

 

Remove nails

- once out of plaster - 6 weeks

- avoid tendon rupture distally

 

 

 

 

Proximal Humeral Fractures

Types

 

Age 0 - 5

- Salter Harris I

 

Age 5 - 11

- Salter Harris II

 

III / IV rare

 

Issues

 

Great remodelling potential

- 80% growth of humerus from proximal physis

 

Shoulder ROM compensatory

 

Age

- even older adolescents do well

 

Growth Plate Closure

 

Females 14 - 17

Males 16 - 18

 

Neer-Horwitz Classification

 

Type I

- < 5 mm displacement

 

Type II

- < 1/3rd shaft width

 

 Paediatric Shoulder Fracture SH 2

 

Type III

- 1/3 - 2/3rd shaft width

 

Type IV

- > 2/3rd shaft width

 

Paediatric SNOH Off Ended

 

Management

 

Nonoperative Management

 

Issue

 

Amount of remodelling potential

- patient greater than 11 may risk shortening and loss of abduction

 

Case:  16 year old boy

 

Pediatric Proximal Humeral Fracture Remodelling PrePediatric Proximal Humeral Fracture Remodelling Post

 

Case:  18 year old boy

 

Pediatric Proximal Humeral Fracture 2 Pediatric Proximal Humeral Fracture Remodelling 2

 

Guidelines

 

Age                           Acceptable alignment

 

< 5                            Up to 70° angulation, 100% displacement

5-12                          Up to 40–70° angulation

< 12                          Up to 40° angulation, 50% displacement

 

Operative Management

 

Options

 

1.  MUA

- displacement rates up to 80%

 

Paediatric SNOH Pre MUAPaediatric Shoulder Post MUA

 

2.  MUA + percutaneous pinning

- K wires from metaphysis into head

- +/- K wire from GT down into metaphysis

- axillary nerve at risk

 

Paediatric Shoulder K wiresPaediatric Shoulder K wire Lateral

 

3.  MUA + TENS

 

Technique

- 2 x drill holes in lateral supracondylar ridge

- pass 2 x TENS

- standard 0.4 of diameter of bone

- usually 2.5 mm

- radial nerve at risk

 

Rajan et al J Child Orthop 2008

- 14 cases

- average age 12.5

- good results

 

Proximal Humerus TENS

 

4.  Open reduction

- failure of closed reduction

- biceps / periosteum may become entrapped

- deltopectoral approach

- protect blood supply / ascending branch medial circumflex humeral

 

 

 

 

Upper Limb

Amniotic Bands

Definition

 

Deep skin crease encircling digit

 

Incidence

 

1: 15 000

 

Aetiology

 

? Due to amniotic adhesions formed after haemorrhage in the distal rays

 

40% associated with other congenital hand abnormality

 

Streeter's syndrome

- hand and feet amniotic bands

 

Patterson Classification

 

1.  Simple Ring

- transversely around limb

 

2.  Deep Ring

- deformity, associated with lymphoedema of distal part

 

3.  Acrosyndactyly

- fingers formed, then bands form constriction

- proximal fingers open, distal closed

 

3.  Intrauterine amputations

- usually distal

- usually central fingers

 

Management

 

Simple incomplete bands

 

Observe

 

Vascular compromise with complete bands

 

Concept

 

Release via multiple Z plasties

- bands with distal lymphoedema

- if band deep may need to be released as staged procedure

 

Technique

- excise a 1- 2 mm margin of normal skin and subcutaneous tissue to minimize the risk of recurrence

- resect all constricted fascia and muscle

- identify and protect neurovascular structures

- debulk excess subcutaneous tissue, especially dorsum fingers

- close the skin with multiple Z-plasties

 

 

 

 

 

Axial Deformity

Cleft Hand

Definition

 

Longitudinal failure of formation of central 3 rays

 

Aetiology

 

Most unilateral & sporadic

Some bilateral & AD

Rare

 

Two types

 

1.  Typical 

- long ray only absent

 

2.  Atypical 

- middle three rays absent

 

Management

 

Function often good

- surgery often not required

- occasionally cleft closure undertaken

 

 

 

Hypoplastic Thumb

Associations

 

Radial club hand

VECTRAL

Holt Oram

 

Blauth Classification

 

Grade I 

 

Minor hypoplasia, all element presents

 

Management

- may need opposition tendon transfer if APB and opponens deficient

 

Grade II 

 

Thenar muscles hypoplasia / aplasia

MCPJ ligament instability

Normal skeleton

Wed space adduction contracture

 

Management

1.  Release web space

2.  Oppensplasty

- FDS RF

- stabilise MCPJ

- provide opposition 

 

Grade III

 

Skeletal hypoplasia

Variable absence MC

Aplasia of CMC

Variable absence of radial carpal bones

Thenar muscle hypoplasia

 

Management

- pollicisation

 

Grade IV

 

Absent metacarpal / rudimentary phalanges

Pouce flottant / floating thumb

 

Management

- LA & tie off at birth

- pollicisation

 

Grade V

 

Total absence

 

Management

- pollicisation

 

Pollicisation

 

Concept

 

Use index finger as thumb

 

Best Results / Aims

 

30% normal strength

Good opposition

- should get pulp to pulp pinch

 

Timing

 

Between one and two years

 

Technique

 

1.  Skin incision for new web space

 

2.  Isolating on radial and ulna NV bundles

- splitting common digital nerve

- division of radial digital artery to middle finger

 

3.  Shorten finger

- remove metacarpal

 

4.  Stabilise MCPJ

 

5.  Balance MT units

- dorsal interossei becomes thumb abductor

- palmar interossei becomes thumb adductor

 

6.  Reattach extensor tendons, leave flexors

 

 

 

Radial Club Hand

Definition

 

Radial hemimelia

- longitudinal failure of formation of parts along radial border / preaxial border

 

Incidence

 

1:100 000

4 x Ulna Club Hand

Bilateral 50% 

 

Associations

 

Scaphoid & trapezium absent in 50% cases

 

Thumb absent in 80%

 

VACTERAL

 

Vertebral segmentation deficiencies

Anal atresia

Cardiac

Tracheoesophageal fistula

Endocrine including Fanconi's anaemia

Renal abnormalities

Radial ray deficiencies atresia

Limb - CTEV, radial club hand

 

Syndromes

 

Holt-Oram Syndrome

- ASD & RCH

 

Fanconi 

- anaemia & RCH (pancytopenia)

 

TAR syndrome

- thrombocytopenia absent radius 

 

Bayne Classification

 

1.  Short distal radius

- distal radial epiphysis present but delayed in appearance

 

2.  Hypoplastic radius

- delayed proximal & distal radial epiphyses appearance

- moderate shortening of the radius & thickening of the ulna

 

3.  Partial absence of radius

- usually distal third absent

- carpus unsupported & radially deviated

 

4.  Total absence of radius

- most common type

 

Pathology

 

All pre-axial structures may be affected

- forearm is radially bowed

- deviated in radial & volar direction

-  progresses with growth

 

Ulna is bowed & short by 25%

 

Carpus attached to ulna by fibrous tissue or small synovial joint

 

There are also abnormalities of finger joints most marked in radial digits

 

Median nerve

- is thickened as carries sensation for radial nerve as well

- it is located deep to deep fascia on radial border of forearm 

- at risk from surgery

 

Radial artery can be absent

 

Clinical

 

Radial & palmar flexed hand 

- forearm is short (50-70%) & radially deviated

- thumb absent / deformed / almost normal thumb 

 

25% have significant elbow extension contracture

- radial deviation may allow hand to mouth

- imperative this function is not disturbed

 

NHx

 

Worse than ulna club hand as tend to radially deviate

- OT may not prevent re-deformity

- if occurs wrist fusion at maturity

 

Management

 

1.  Splinting & stretching from birth

- prevent excessive radial deviation

 

2.  Centralisation procedure

 

Concept

- centralising carpus over ulna

- soft tissue release at 1 - 2 years

- maintain with tendon transfers

- transfer ECRL to ECRB 

 

Prerequisites

- lack of hand to mouth function is a contraindication to surgery

- elbow mobility is prerequisite for centralisation

 

3.  Management of hypoplastic thumb

 

 

 

 

 

 

 

Ulna Club Hand

ulna club handEpidemiology

 

4 x less common than RCH

 

Very rare

 

Pathology

 

Radiohumeral synostosis common

Radial head dislocation seen in 50% cases

 

Associations

 

PFFD

Club foot

Spina bifida

 

Management

 

Good function

- stretch / splint to avoid UD

 

OT rare

- excision of proximal ulna to create one bone forearm

- lose forearm rotation

 

 

 

 

 

 

 

Congenital Anomaly Classification

1.  Failure of Formation

 

A Transverse Failure

 

Congenital Amputation

Phocomelia

 

B Axial / Longitudinal Failure

 

Radial Club Hand

Ulnar Club Hand

Cleft Hand / Foot

 

2.  Failure of Separation

 

Syndactyly 

Apert's Syndrome

 

3.  Duplication

 

Polydactyly

- pre axial (tibia & radius)

- post axial (fibula & ulna)

 

4. Overgrowth

 

Hemihypertrophy

Macrodactyly - commonest of these

 

5.  Undergrowth

 

Hypoplastic thumb - commonest of these

Hypoplastic hand & digits

 

6.  Congenital constriction bands

 

7.   Miscellaneous 

 

Congenital Trigger Digits

Camptodactylty

Kirner's Deformity

Delta Phalanx

Dyschondriostosis 

Madelung's Deformity

 

Terminology 

 

Amelia

- complete absence of limb

 

Phocomelia

- seal or flipper limb

- terminal portion attached to trunk

- intercalary defect

- deformed hand with only 3-4 digits

 

Hemimelia 

 

Absence of major portion of limb

- Radial / Ulnar / Tibial / Fibula

 

Complete

- entire distal 1/2 of limb absent

 

Incomplete

- greater portion of distal 1/2 of limb absent

 

Paraxial

- preaxial or postaxial portion of distal 1/2 of limb absent

- preaxial: thumb / radial, tibia

- postaxial: little finger / ulna, fibula

 

Terminal

- no unaffected parts distal

 

Transverse

- defect extends transversely

- complete absence of some part distal to some point on the upper extremity

- common levels are: fingers, upper third forearm, midcarpal

- 98% unilateral

- aetiology unknown except for thalidomide

 

Intercalary

- middle portion of limb deficient

- proximal & distal portions present

- can be transverse or paraxial 

- ie fibula hemimelia may have lesser toes present

 

Acrosyndactyly

- fusion of fingers distally with proximal fenestrations

 

Brachysyndactyly

- shortening of syndactyl digit

 

Aetiology

 

Normal development

- limb buds develop at 4/52

- apical ectodermal ridge controls underlying mesoderm

- limb segments develop in proximal to distal sequence

- skeletal elements formed as cartilaginous models

- models ossify

- clefts form & develop joints

- embryonic skeleton well formed at 7/52

- sonic hedgehog gene important

 

Abnormal Development

 

50% unknown

- genetic factors - Trisomy 21 / 18 / 13

- environmental factors - thalidomide / warfarin / dilantin well established

- combination

 

May cause

- arrest of development of embryonic limb

- destruction of structures already formed

 

Must occur between 4th and 7th week

 

 

Congenital Hand

Camptodactyly

MEM camp = bent tent

 

Definition

 

Greek for bent finger in sagittal plane

 

Usually little finger

 

Typical deformity is FFD of PIPJ & extension of MCPJ

 

Epidemiology

 

<1% of population

 

Strong familial predisposition

- 60% bilateral

- bimodal pattern

- present in infancy or adolescence

- 1 in 1000 live births

 

Aetiology 

 

Unknown

- not related to trauma, infection, NMD

- ? due to incomplete extensor mechanism

 

Usually due to imbalance of pull between flexor & extensor mechanisms

- contracture of collaterals 

- secondary abnormality PIPJ common

- usually base of P2 is subluxed volar to head P1

 

Most commonly accepted theory 

- an anomalous insertion of the lumbrical muscle into the flexor sheath causes the abnormality

 

Multiple causes reported

- aberrant lumbrical insertion 

- flexor sheath contracture

- phalangeal head deformity

- FDS contracture

- abnormal extensor tendon mechanism

 

Classification

 

1. Infantile

2. Adolescent

3. Severe form affecting all triphalangeal digits

 

DDx

 

Traumatic boutonnière deformity

 

Juvenile aponeurotic fibromatosis 

- involves palmar fascia & skin

- increased flexion deformity

 

NHx 

 

80% progress

- progress during growth spurts

 

Doesn't progress after maturity

 

Clinical Features

 

85% present at birth

15% present in adolescence

 

Volar skin may bowstring

- can be mild to severe FFD

- functional compromise when FFD ->90o

 

Fixed v flexible

 

Xray

 

Head of P1 has bullet shape

PIPJ is subluxed

 

Management

 

Non-operative

 

Usual treatment

- watch to see if deformity progresses especially at growth spurts

- some studies show that splinting does work if 24 hours per day

- other studies without 24 hours splinting show not much difference

- passive stretching & static & dynamic splinting

 

No series has shown surgical release does better than well motivated and controlled regimen of flexion and extension splinting

 

Operative

 

Indications

 

Progressive deformity

Failure of non-operative management

> 60° & functional disability

 

Poor outcomes especially loss of flexion

- some series report only 35% success rate

 

Technique

 

Fixed

- release all tight volar structures

- skin / FDS / volar plate / capsule

- +/- FDS transfer to the extensor hood

 

Skeletally mature

- extension osteotomy / dorsal closing wedge

- must avoid excess straightening -> stretch nv bundle 

 

Do not perform release and simultaneous transfers as need to mobilise immediately post-operatively

 

 

 

Clinodactyly

Definition

 

Clinodactyly is a fixed abnormal deviation of the finger

- in the coronal or radioulnar plane

 

Typically affects the middle phalanx of the fifth finger

- produces a radial angulation of the distal interphalangeal joint.

 

Normal if < 10-20°

 

Types

 

Primary

 

Usually little finger

- common

- AD & sporadic forms

 

80% syndromic

- Down's

- fetal alcolohol syndrome

 

M > F

 

Secondary / Delta Phalanx 

 

Commonest cause

- trapezoid shaped P2

- cosmetic problem only

 

Management

 

Primary clinodactyly 

 

Indication

- when fingers overlap 

 

Management

- closing wedge osteotomy of P1

 

Secondary / Delta Phalanx

- see additional notes

 

 

Congenital Trigger Finger

Epidemiology

 

Most common in thumb

 

Bilateral in 15%

 

Clinical

 

Present with FFD of thumb / finger

 

Pathology

 

Thumb

- constriction of A1 pulley

 

Finger

- nodules in FDS or FDP

- catching at A1, A2 or A3

- may have anomalous insertion lumbrical

- often at tendon decussation area

 

Types Trigger Thumb

 

1. True congenital

- bilateral in 50% cases

- can be sporadic

- associated with trisomy 18 / mucopolysaccharidases

 

2. Common type

- presents 6/12 - 3 years

 

NHx

 

Thumb

- spontaneous relapse 30% < 1 year

- spontaneous relapse 10% > 1 year

 

Finger

- not present at birth

- usually acquired

- more likely to spontaneously relapse

 

Operative Management

 

Indication

 

Before age 3

 

Thumb

 

> 1 year

- release A1 pulley

- careful preservation digital nerves

 

Finger

 

Extensive exposure / progressive releases / repeat intraoperative examination

- release A1 pulley

- release anomalous insertions

- partial release A2 and A3 pulley

- release single slip FDS

 

 

 

 

Delta Phalanx

Definition

 

Abnormal extra trapezoidal shaped phalanx 

 

Epidemiology

 

Strong Family History

 

Most common in

- P1 Thumb

- P2 Little finger

 

Aetiology

 

Abnormal "C" shaped epiphysis brackets one side of phalanx 

 

Deformity

 

Results in angular deformity

- typically centrally deviated

 

Little finger radially 

 

Thumb towards ulna

 

Operative Management

 

Indications

 

Functional disability

 

Technique

 

Resection isthmus of "C" shaped continuous epiphysis

- insertion of interpositional fat graft

- if excise delta phalanx -> get excessive shortening

 

 

Kirner Deformity

Definition

 

Volar-radial deviation of P3 of little finger

- AD trait

- always bilateral

- presents at age 10

- cosmetic problem only

 

DDx

 

Frost bite injury to phalanx can mimic it

 

Surgery 

 

Indication

 

For cosmesis only

 

Technique

 

2 volar osteotomies / straighten / K wire

 

 

Macrodactyly

Definition

 

Rare congenital anomaly with enlargement of the finger

 

Epidemiology

 

0.9%

 

Aetiology

 

Uncertain

Strong FHx

 

Pathology

 

All elements involved

- compared to enlargment from tumour /haemangioma

 

Index finger most commonly affected

- involved digit is stiff & angulated

- always large at birth compared with 2° enlargement

 

Classification

 

Primary

- true macrodactyly

- all tissues enlarged

 

Secondary

- neurofibromatosis

- hemangioma

- lymphangioma

- AVM

- fibrous dysplasia

- lipoma

 

NHx

 

1. Static type

- grows with child at normal rate

 

2. Progressive type

- progressive enlargement out of proportion to normal growth

 

Operative Management

 

Indications

 

Most patients will require surgery but surgery often unsuccessful

 

Options 

 

Debulking procedures

- fat & skin

- may need multiple debulks

 

Epiphysiodesis

- when digit achieved adult length

 

Finger shortening

- removal of segments from P2 & P3

- has a high complication rate

- stiffness, pain, residual deformity & skin problems

 

Ray resection

- salvage

 

Usual approach

 

Staged procedure

- operative on one side of finger at a time

- convex side first

- remove up to 25% of phalanx

- remove half of soft tissue from each side

 

Epiphysiodesis once digit same size as parents digits

 

Uncontrolled growth

 

Ray resection

 

May get accelerated growth in adjacent digits after ray amputation

 

 

 

Polydactyly

Epidemiology

 

More common in African Americans

 

Aetiology

 

Sporadic and AD

VACTERL

 

Classification

 

Preaxial

 

Type 1:  Thumb polydactyly

Type 2:  Triphalangeal thumb

Type 3:  Index polydactyly

Type 4:  Polysyndactyly

 

Central

 

Duplication of the IF / MF / RF 

- Rare

 

Postaxial

 

Type A Extra well formed ulnar digit articulating with a metatarsal

Type B Extra poorly formed ulnar digit / often a skin tag

 

Classification

 

I   Soft tissue only

II  Bone elements

III Own metacarpal

 

Management

 

Simple soft tissue 

 

Tie string & autoamputates at birth

 

Complex 

 

Operate at ~ 1 year in hand

- once old enough to be safe for operation

- general rule is to amputate outside extra digit

 

Thumb Polydactyly

 

Wassell Classification

 

Most common variety

- duplicated proximal phalanz with single metacarpal

- Type IV

 

Usually delete radial thumb

 

Issues

 

1.  Smaller thumb

 

2.  Unstable MCPJ with type IV

- important to rebalance residual digit with joint capsule & tendon insertions 

- otherwise will end up with Z deformity

- ulna deviation at MCPJ, radial at IPJ

 

3.  Angular deformity

- caused by supernumerary thumb and delay in surgery

- may need osteotomy

 

 

 

Syndactyly

Definition

 

Webbed fingers

 

Aetiology

 

Usually spontaneous but can be inherited

Due to a failure of separation during embryonic development

 

Epidemiology

 

Most common congenital hand abnormality 1:2000

 

Family History in 20-40% / AD

 

Associations

 

Polydactyly

Amniotic bands

Toe webbing

VECTRAL

Craniofacial abnormalities

Apert's syndrome

 

Classification

 

Simple / Complex 

- simple skin only

- complex has bony involvement

 

Complete / Incomplete 

- extent of finger involved distally

 

DDx

 

Acrosyndactyly 

- fusion of fingers distally with proximal fenestrations

 

Brachysyndactyly 

- shortening of syndactyl digit

 

Anatomy

 

Middle & ring finger commonest 60%

 

Thumb & index rarest

- Apert's

 

Bilateral in 50%

 

Frequent anomalous sharing of

- musculotendinous structures

- artery / veins / nerves

 

Management

 

Timing

 

Surgery aged 1 year

- minimise bony deformity

- especially if thumb / index as in Apert's syndrome

- once child safe for anaesthetic

 

Important surgical principles

 

1.  Beware NV abnormality

- usually normal number in simple syndactyly

- may be absent in complex

- may divide much more distally

 

2.   Skin shortages

- may need FT skin graft

 

3.   Release one side at a time

- may jeopardise vascularity to finger

- 3/12 between each side

 

4.   Need to create web space

- Zig Zag incisions with volar or dorsal flap for web space (Vickers) 

- important not to deepen web space too much as looks unsightly

- web space deepening often limited by digital artery bifurcation

 

5.  Division of border digits done early to avoid angular deformity

 

Complications

 

Clinodactyly

- due to shortening of graft

- not seen with Vickers technique

 

Web creep 

- return of webbing 

- revise as teenager so only need one further surgery

- web space reconstruction

 

Neurovascular injury

 

 

 

 

Madelung's Deformity

Madelung's deformity with fracture

 

Pathology

 

Premature fusion of the volar & ulna aspects of the distal radius

- progressive ulnar & volar tilt of distal radial surface

 

Ulna becomes prominent through normal growth & subluxes dorsally

 

Lunate lies deeply between lower end of radius and ulna

 

Aetiology

 

1. Volar-ulnar radial physeal arrest

- focal physeal dysplasia

 

2. Carpal tether from same area to lunate

- Vicker's ligament

 

Anatomy Vickers' ligament 

 

Vickers describes fibrous band 

- runs from radius to lunate

- volar position

- large ligament ~ 0.5cm diameter

- may be the cause

- if excise early / before age 8 the radius improves in shape

 

Epidemiology

 

Idiopathic condition

- AD with incomplete penetrance

- F > M

- often bilateral

 

Association

 

Turner's Syndrome

 

Disorder of gonadal differentiation

- 45 X / XO

- female phenotype / sterile

- webbing of neck

- low posterior hairline

- cubitus valgus

- cardiac abnormalities

 

DDx Madelung Deformity

 

Post Traumatic

Enchondromatosis (Ollier's)

Hereditary Multiple Exostoses

Turner Syndrome

Idiopathic = Dyschondrosteosis

Infection

General dysplasia

 

Classification

 

Madelung's - ulnar, volar 

Chevron - ulnar, central 

Reverse Madelung - ulnar, dorsal

 

Clinical

 

Usually asymptomatic and good function

 

Wrist is in radial deviation

 

Dorsal subluxation of distal ulna

 

Reduced dorsiflexion / supination

 

NHx

 

Not apparent in childhood

Usually becomes obvious as hits adolescent growth spurt

 

X-ray

 

Madelungs APMadelung's Lateral

 

Volar-ulna tilt of the distal radius with dorsal subluxation of ulna / distal radius triangular shape

 

Proximal carpal row becomes wedge shaped

- lunate "falls into" the radio-ulna gap

 

DRUJ widened

 

Chevron 

- proximal carpal row distorted / V-shape

- lunate in apex between radius and ulna

 

MRI

 

Look for Vicker's ligament

 

Management

 

Mothers function is a good guide to management

 

Surgical Indications

 

1. Pain 

- must operate

- surgery very good at relieving pain

- in adults division Vickers' ligament almost always relieves pain 

 

2. Deformity 

- deformity starts ~ age 9 years (doesn't occur much before then) 

- best to operate early as will get good result

 

Options

 

1.  Excision Vicker's ligament + Epiphysiolysis of fused physis with fat graft

 

2.  Complete epiphysiodeses 

 

3.  Epiphysiodeses of distal Ulna

 

4. Ulna Shortening / Darrach

 

Madelung's with Darrachs

 

5.  Opening Wedge Osteotomy

 

Madelung's Osteotomy

 

6.  Wrist Arthrodesis

 

 

 

 

 

Obstetric Brachial Plexus Injury

Definition

 

Birth injury of brachial plexus

 

Epidemiology

 

R > L 

1 / 1000 live births

1 in 10 of these develop significant impairment

Recent increase due to bigger babies / DM

 

Aetiology

 

Usually secondary to traction

 

1.  Big baby / maternal DM / > 4000 gm

2.  Breech

3.  Prolonged / difficult labour

4.  Shoulder dystocia 

- inability to deliver the shoulders after delivery of the head

- indirect traction injury on limb with excessive lateral neck flexion

5.  Forceps delivery

- direct forcep crush injury

 

Pathology

 

Spectrum of injury

- supraclavicular traction injury

- upper roots ruptured

- lower roots avulsed

 

Classification

 

Erb Palsy

Klumpke Palsy

Total Plexus

 

ERB Palsy 

 

A.  C5,6 lesion

 

Weak

- deltoid / rotator cuff

- biceps  - elbow flexion and supination

- wrist extension

 

Waiter's Tip Deformity

- shoulder adducted & internally rotation (P major / Lat dorsi)

- elbow extended

- forearm pronated

- wrist flexed 2° FCR

 

May have winging of scapula as compensation

 

Best prognosis

- 90% recover 6/12

 

B.  C5,6,7

 

Additional weakness

- triceps / loss of elbow extension

 

Prognosis

- 50% recover 6/12

 

Klumpke Palsy

 

C8,T1 lesion

- rare

- Horner's Syndrome if preganglionic

 

Weakness of

- finger flexion

- intrinsics

 

Numb forearm & hand

 

Poor prognosis

 

Total Plexus Injury

 

C5 - T1

- flaccid numb arm 

- Horner's if preganglionic

 

Worst prognosis

- 40% recover 6/12

 

NHx

 

90% recover overall

 

Biceps best indicator of recovery

- if biceps recovers in 3/12 90% will make full recovery

- also is easiest to assess in young child

- see if child will use that hand to bring something to the mouth

 

Poor prognosis 

- no recovery 3/12

- Total plexus

- Klumpke

- preganglionic (Horner's, DSN, LTN, SSN)

 

DDx

 

Pseudoparalysis

- clavicle fracture (from delivery)

- humeral fracture

 

Arthrogryposis

- nil elbow crease

- hasn't flexed in utero

 

Monoplegic CP

 

Other

- OM / Septic arthritis

- Congenital shoulder dislocation

- sprengel shoulder

- myelodysplasia

 

Management 

 

Non operative

 

Initial

 

Physiotherapy

- maintain FROM / passive ranging

- no splinting

 

3/12

- assess biceps recovery

- EMG at 3months if no recovery

 

Long term

 

Maintain shoulder ER

- prevent posterior shoulder dislocation

- reassess child in clinic every six months

 

Operative Management

 

Indications

 

No elbow flexion / biceps recovery at 3 - 6/12

- biscuit test (hold other arm, see if can eat biscuit)

- very difficult to assess shoulder or wrist extension

 

If children don't have full recovery of biceps at 3/12 will often be left with residual deficit

 

MRI

 

Valuable if see pseudomeningocoeles

- low chance of recovery of those nerve roots

 

Timing

 

6/12 to 1 year

 

Options

 

Preganglionic avulsion

- nerve transfer

 

Postganglionic

- nerve grafting

 

Preganglionic Avulsion 

 

Nerve transfer

 

A.  Accessory nerve to suprascapular 

 

B.  Intercostal nerve to MCN

 

Postganglionic injury 

 

Neuroma resection & sural nerve grafting

 

Erbs / C5/6

- resection Erb's point

- nerve grafting

 

Aims

- lateral cord / MCN 

- SS nerve

- posterior division of upper trunk to posterior cord

 

Results

 

Late Shoulder Management

 

Issue

- residual internal rotation / adduction contracture

- tight SSC / P major / T Major / short head biceps

- humeral neck retroverted

- develop dysplasia head and glenoid

- GHJ tends to dislocates posteriorly

 

Incidence

 

10%

- more common in C5/6

- due to strong P Major and LD

 

Management Summary

 

< 6/12       Observe

6/12           Nerve transfer / graft

<2  years    Release contracture Subscapularis / T major / P major

2-5 years    Lat Dorsi Transfer

>5  years    Derotation Humeral osteotomy

 

Initial

 

Monitor and maintain ER shoulder

- physio / botox

 

Investigations

 

Indication

- limited ER

- anterior shoulder crease

 

Ultrasound

- very sensitive at 6/12 age

 

Shoulder releases

 

Timing

- < 2 years

- important to do releases early 

- avoids IR contracture 

- avoids consequent need for osteotomy or reduction of dislocation

 

Release

- P major

- T major

- Subscapularis

 

Tendon Transfers

 

Timing

- 2-5 years

 

Options

 

Lat Dorsi transfer to posterolateral rotator cuff 

- act as shoulder ER 

- often combine with releases

 

Steindler's flexorplasty

 

Humeral derotation osteotomy

 

Assessment

- CT to assess bony anatomy

- may need posterior glenoid bone block

- shoulder may be dislocated

 

Timing

- > 6-8 years

 

 

 

Pseudarthrosis of Clavicle

Congenital Clavicle Pseuodarthrosis

Epidemiology

 

Rare condition

 

Aetiology

 

Theories

1. Failure of fusion of 2 ossification centres of clavicle

2. Pressure from subclavian artery

 

Clinical Features

 

Painless swelling over mid clavicle

- almost always on Right side

- presents soon after birth

 

 

 

X-ray

 

Bulbous ends overlying each other

 

DDx

 

Birth fracture of clavicle

- painful

- history of traumatic delivery

- callus on Xray

 

Cleidocranial Dysostosis

- abnormality of intramembranous ossification

- bilateral

- associated facial / jaw & pelvic bone abnormality

- no palpable lump

 

NHx

 

Painless

- no functional problem

 

Management

 

Indications

 

Cosmetic issue from lump

 

Surgical options

 

1. Excision of prominent lateral end of sternal part

2. Bone graft & internal fixation

 

 

 

 

Radial Head Dislocation

DDx

 

1. Congenital

 

Rare

- anterior or posterior

- lateral uncommon

 

2. Acquired

- unrecognised Monteggia

- osteochondroma

- enchondroma

 

3. Skeletal Dysplasia

- OI - ulna deformity

- Achondroplasia

- Down's Syndrome

- Nail-Patella

- Klinefelter's

- Marfan's

- Hereditary Multiple Exostosis

- Ehlers Danlos

- Larsens

 

Congenital


History

 

Familial

Other congenital problems

 

Xray

 

Convex radial head

Hypoplastic capitellum

Long radius compared with ulna

 

Management

 

No treatment (reconstructions fail)

- most congenital dislocations are asymptomatic 

- good function and ROM

- excise radial head as adult if painful

 

 

 

 

Radioulna Synostosis

Congenital

 

Epidemiology

 

Usually bilateral / AD

 

Pathology

 

Failure of separation of cartilaginous rods that form radius and ulna

 

Clinically

 

Forearm in varying degrees of pronation (supination rare)

- may have difficulty with ADL's

 

Management

 

Pronation is more useful position than supination

 

May need supination on one side

- osteotomy radius and ulna

- changes functional position not arc of range

- high risk of comparment syndrome

 

Acquired

 

Aetiology

 

Fractures at same level

Most common proximally

 

Management

 

Excision of synostosis and interpose fascia 

- poor results

 

 

Transverse Deficiencies

Terminal Phocomelia

 

Definition

 

Complete absence of some part distal to some point on the upper extremity

 

Incidence

 

1:20 0000

98% unilateral

 

Aetiology 

 

Usually unknown ? AR trait

Thalidomide

 

Sites

 

Commonest level is proximal forearm

 

Also

- fingers / midcarpal / wrist / mid-forearm / mid arm

 

Management

 

Non-operative / Prosthesis

 

Passive terminal device started 3/12

 

Active device as child starts to use above device

 

Krukenberg procedure

 

Radius and ulna formed into pincers

Only in blind / bilateral

 

Intercalary Phocomelia

 

Definition

 

Loss of intercalated segment

- hand suspended from trunk

- loss of elbow joint

- deformed hand with only 3-4 digits

 

Aetiology

 

Seen with thalidomide

Often bilateral

 

Three types

 

1. Complete 

- hand to trunk

 

2. Distal 

- hand to arm

 

3. Proximal 

- forearm to trunk