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