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