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spina bifida spina bifida Presentation Transcript

  • Spina bifida
    Presenter Dr Narayanan sk
    Chairpersons Dr Ravishanker
    Dr Manjunath
  • Spinal Dysraphism
    Spinal Dysraphism is a generalised term for a group of congenital abnormalities that can cause progressive neurological damage and therefore the deterioration of neural and physical function. The abnormalities are also sometimes known as neural tube defects.
  • Spina bifida
    Spina bifida latin term for split spine.
    Congenital disorder in which 2 halves of the posterior vertebral arch has failed to fuse.
    This can lead to hernial protrusion of spinal cord and its meninges
    Results in variable neurological defects depending on the location and severity of the lesion.
    View slide
  • Embryology
    In the third and fourth week of intra uterine life the neural groove appears as a dorsal Focal thickening caused by the proliferation of ectodermalcells.Theses cells increase in number and height .A groove is formed in the sagital plane .the groove deepens causing the lateral portion to face eacher.
    View slide
  • By about 21 st day cell adhesion causes formation of Neural tube
    Initial fusion occurs near the center of embryo,then it proceeds in both the directions and the cephalic end closes first.Thesuperfical ectoderm separates and forms the neural arch and paraspinal muscles
  • The embryonic origin of spina bifida , if the abnormality occurs between 26 -28 days , during the phase of closure of neural tube its is known as NEURALIZATION DEFECT
    It includes myelomeningocele and anencephaly
    If the abnormality occurs between 28 -48 days ,during the phase of canalization it is known as POST NEURALIZATION DEFECT
    It includes meningocele, lipomenigocele and diastematomyslia
  • The exact mechanisms by which humans develop this abnormality remain elusive
    The proposed theories are
    • Primary failure of closure of the neural tube
    • Secondary rupture of the neural tube due to increase in csf pressure
  • Types of spina bifida
    Spina bifida occulta
    Spina bifida cystica
    • Meningocele
    • Myelomeningocele
  • Spina bifida occulta
    Mildest form
    Incomplete formation of posterior arch of the spinal column
    Usually incidental finding
    Seen in LS spine, commonest S1
    Occasionally patient may have fatty deposit, hemangioma or tuft of hair
  • Spina bifida cystica
    • Two types:
    Meningocele- protrusion of only the dura and arachnoid through the defect in the vertebral lamina forming a cystic swelling usually in lumbosacral area
    Spinal cord remains in the vertebral canal
  • Spina bifida cystica
    10 times more frequent
    portion of the spinal cord or the nerve roots are displaced through the spina bifida defect into the sac
  • Incidence
    1 in 1000 live births
    Female: male ratio= 1.2:1
    Mainly seen in Hispanic women
    Incidence decreased by about 80% due to prenatal screening using USG and measurement of maternal serum α fetoprotein & administration of folate before and after pregnancy
  • Etiology
    Exact cause not known
    Proposed theories are :
    exposure to fungus Phytophthorainfestans by excessive consumption of potatoes
    Use of anti seizure drugs(sodium valproate)
    Genetic – 6-8%
    Couple with one affected infant- risk of subsequent sibling acquiring major CNS malformation is 1 in 14
  • Folic acid deficiency-
    80-100% reduction in neural tube defects with administration of folic acid.
    Daily dose 0.4 mg.
    max dose 1mg/day
    Up to 4mg/day to women who have given birth to a prior affected child
  • Associated conditions
    Hydrocephalus– dilatation of ventricles of brain from excessive CSF
    Seen in 80-90% of meningomyelocele
    Patients with thoracic and upper lumbar lesions have a higher incidence than those with lower lumbar and sacral lesions.
  • Hydrosyringomyelia
    Accumulation of CSF in the enlarged central canal of the spinal cord.
    Can cause three problems in patients with myelomeningocele
    Increase in level of paralysis
    Progressive scoiliosis
    Weakness of hands and upper extremities
  • Arnold chiari malformation
    Caudal displacement of posterior lobe of cerebellum
    Causes dysfunction of lower cranial nerves which results in weakness and paralysis of vocal cords and difficulty in feeding
    Placement of VP shunt often resolves this problem
  • Tethered spinal cord
    Usually a MRI finding
    Usual clinical findings are loss of motor function, development of spasticity in lower limbs, development of scoliosis before 6 years age in absence of vertebral anomalies, back pain and increased lumbar lordosis and changes in urologic function.
    MRI evaluation should be performed.
  • Urological dysfunction
    Almost all children with meningomyelocele have bladder dysfunction.
    Chronic renal failure and sepsis from UTI were the most common causes of delayed mortality
    Mainstay of treatment is clean intermittent catheterisation to prevent development of hydronephrosis to maintain bladder compliance and capacity.
  • Latex allergy
    Incidence 3.8 – 38%
    Manifests in form of local rashes or mucosal irritation
    Latex skin prick test and assay for latex specific immunoglobulin E in serum
    Non exposure to latex is recommended in all patients with meningomyelocele.
  • Classification –based on level of lesion
    • Thoracic level
    Most frequent deformity encountered by surgeons are spinal deformities
    No active hip flexion, no voluntary muscle control of lower extremity
    • Upper lumbar level
    Hip dysplasia and dislocation due to imbalance of flexors and adductors
    No extension/ abduction
    • Lower lumbar level
    Hip adductor strenghth and quadriceps power to provide active hip extension
    Weakness of foot, ankle, hip abductors and extensors lurching gait imposing great deal of stress on unbraced knee
    • Sacral level
    Weakness of peroneus and intrinsic muscles of foot
    Partial paralysis and insensate skin can lead to foot problems and neurogenic ulcers
    Not all patients have one of these classic levels of paralysis.
  • Multidisciplinary care
    • Management must be integrated to treat the child as a whole and provide the family the necessary support.
    • Best assessed and treated by multidisciplinary clinics.
    • Consists of:
    Administrative or registered nurse to function as patient’s advocate. Needs addressed over time including educational, vocational and sexual counselling.
    Orthotist – to provide and repair lower extremity and spinal orthrosis.
    Physical therapist- to aid lower extremity functional assessment, bracing needs and ROM exercises for daily activities.
    Nurse to teach about skin care and self catheterisation
  • Psychologist to help parents cope with many challenges and stresses related to child’s diability.
    Urologist to monitor genitourinary function and maximise bladder control
    Neurosurgeon to monitor shunt dysfunction and development of tethered cord
    Social worker to assist family in finding financial support
    Experienced neurodevelopmentalpaediatricianto oversee the whole process.
    Finally an orthopaedician to produce a stable posture for sitting or standing.
  • Orthopaedic management
  • Orthotic management
    Principle component of management
    Main goal is to stabilise joints in the absence of lower extremity muscle function and facilitate weight bearing and ambulation.
    Specific protocols by spinal level
    • Thoracic and upper lumbar levels
    Unable to pull themselves to a standing position
    Usually full lower extremity bracing is required ( HKAFO)
    When patient is ready for upright position standing frames or wheel standers can be given.
    Wheel standers allows children significant amount of mobility on flat surfaces.
    Once they are comfortable with upright position, then it can be converted to HKAFO
    • Lower lumbar and sacral levels
    Good quadriceps function
    Should be able to function with AFO
    First prescription should always be KAFO, after initial adaptation and ambulation efforts, can be converted to AFO.
    Frequent falls, loss of confidence and recurrent knee effusions KAFO should be reinstituted.
  • Braces
    • Ankle- Foot Orthroses
    Polypropylene AFO are mainstay of lower extremity bracing
    Usually used in sacral level lesions.
    Main function is to protect foot and toes during weight bearing and to stabilise the ankle joint.
    • Knee ankle foot orthroses
    Usually used in upper lumbar lesion where quadriceps action is weak.
    KAFO consists of AFO component, thigh cuff and knee hinge.
    • Hip knee ankle foot orthroses
    Used in patients with inadequate hip strength or in patients who cannot bear weight with KAFO
    Consists of two long leg braces (KAFO) connected by a pelvic band and a free hip joint.
    • Reciprocating gate orthroses
    Sophisticated form of HKAFO
    Unique feature is two long leg components are connected by a spring loaded cable, flexion of the hip with advancement of the limb produces passive controlateral hip extension allowing energy efficient stride.
  • Other mobility aids are standing frame, parapodium, wheel chair.
  • Surgical management of specific problems
  • Foot
    75 % of children with myelomeningocele have foot deformities that can seriously limit function.
    The main goal of orthopedic treatment is to have a plantigrade, mobile, braceable foot.
    Manipulation and casting should be used with caution.
    Study by Frawley et al showed calcaneal deformity to be the most common, followed by equinus, valgus, club foot and vertical talus.
    All major foot deformities have high frequency of recurrence.
  • Calcaneal deformity
    Seen in 1/3 rd of children with meningomyelocele.
    Level of lesion L5-S1
    Due to active anterior leg muscles and inactive posterior muscles.
    Supple deformity manipulation and splinting in neutral position.
  • Muscle imbalance simple tenotomy of ankle dorsiflexors and evertors
    “Anterolateral release”
    tendons of the extensor hallucislongus, extensor digitorumcommunis ,anterior tibial,peroneusbrevis and longus tendons are exicised
  • Combined with transfer of tibialis anterior tendon to calcaneum.
  • Equinus deformity
    Seen with high lumbar or thoracic level lesions.
    Equinus deformity can be prevented by gentle passive manipulation, bracing and splinting.
    If deformity persists:
    Tendoachilles lengthening
    • Open
    • Percutaneus
    • Careful postoperative casting for few weeks, followed by fitting of orthroses.
    • Long flexors must be released if toe deformity presentmay result in pressure sores.
  • Club foot
    Seen in 30% of meningomyelocele.
    Irrespective of level of lesion.
    Characterised by :
    Severe rigidity
    Supinationvarus deformity
    Rotation malalignment of calcaneum and talus
    Subluxation of calcaneocuboid & talonavicular joint
    Recurrence of deformity is common after adequate surgical correction.
    Manipulation and casting techniques are difficult due to absence of pain and protective sensation which can lead to fractures and pressure sores.
  • Ideal age for surgery : 10-18 months
    Radical posteromedial- lateral release through Cincinnati incision.
    Talarenucleation( VO procedure)
    Difficulty in wound closure is common and rotational flaps can be done for primary closure.
    Recent studies show wound can be left open as much as necessary with foot in correct position and meticulous postoperative care.
  • VO procedure
    Verebely- Ogston procedure
    Decancellation procedure- removing of cancellous bone from talus and cuboid.
    Leaves a hollow shell of bone and more space for correction.
    Foot is manipulated into neutral position and because of the collapse of talus and cuboid correction of residual deformity.
  • Varus deformity
    Isolated varus deformity of hind foot is very rare.
    Imbalance between invertors and evertors should be evaluated.
    Lateral closing wedge osteotomy of calcaneum is done and calcaneum should be translated laterally if possible to increase the correction.
  • Cavovarus deformity
    Occurs in sacral level lesion.
    Cavus is the primary deformity which produces varus.
    Rigidity of the varus deformity should be determined before planning for surgery.
    Supple deformity radical plantar release without hind foot bone surgery is done.
  • Radical plantar release
    Release of the plantar aponeurosis, abductor hallucis, short flexors of calcaneum is done.
  • If the deformity is rigid despite radical plantar release, closing wedge osteotomy is done.
    Triple arthrodesis rarely indicated as a salvage procedure.
    Subtalar, calcaneocuboid and talonavicular joint are fused.
  • Supination deformity
    Occurs in L5-S1 level lesion.
    Mainly due to unopposed action of anterior tibial muscle.
    Supple deformity simple tenotomy of anterior tibial tendon.
    If some amount of gastrocnemius-soleus activity is present with no spasticity, the anterior tibial tendon can be transferred to the mid foot in line with 3rd metatarsal.
    Split anterior tibial transfer can be done with the lateral half of tendon inserted in the cuboid.
    Osteotomy of 1st cuneiform or base of 1st metatarsal may be required for residual deformity.
  • Hind foot valgus
    Initially hind foot valgus can be controlled by a well fitted orthroses but as the child becomes taller and heavier the control of deformity becomes difficult and then surgical treatment is indicated.
    Hind foot valgus > 10 mm of lateral shift of calcaneum is significant.
    Grice extraarticulararthrodesis is the classical treatment of this problem.
  • Grice extraarticulararthrodesis
    Extraarticular fusion of subtalar joint with bone graft is done.
    For rigid fixation and hold of the graft, a k-wire or screw can be used anteriorly from the talar neck into the calcaneum.
  • Vertical talus
    Seen in 10% children with meningomyelocele.
    Characterised by malalignment of hind foot and mid foot.
    • Two types:Developmental and Congenital
    Both requires surgical correction once the deformity is partially corrected by manipulation and serial casting.
    Ideal age for surgery : 12-18 months
    Posteromedial or lateral release should be performed.
    Anterior tibial tendon can be resected or transferred into talar neck.
    If required, subtalararthrodesis can be done.
  • Pescavus deformity
    Seen in sacral level lesion.
    Painful callosities under the metatarsal head and difficulties with shoe wear.
    For isolated cavus deformity with no hind foot varus, radical plantar release is done.
    If varus deformity is done, medial subtalar release is done followed by immobilisation in POP cast.
  • Toe deformity
    Claw or hammer toe occurs in sacral lesions.
    For flexible claw defomities, simple tenotomy of flexors at the level of proximal phalanx is done.
    For rigid claw deformity, partial resection of IP joint or arthrodesis.
    If cavus deformity is present, Jones procedure can be performed- tendon suspension method.
  • Extensor hallucislongus tendon is attached to neck of first metatarsal; interphalangeal joint is arthrodesed and fixed by medullary wire and by suturing distal end of extensor hallucislongus tendon to soft tissues over proximal phalanx.
  • Ankle
    • valgus deformity
    Common deformity in ambulatory patients, irrespective of level of lesion.
    Deformities arise from distal tibia or subtalar joint.
    Common sequelae is skin irritation or breakdown over the medial malleolus from excessive pressure over the orthroses.
    To evaluate valgus ankle deformity, 4 factors must be determined.
    Skeletal maturity
    Degree of fibular shortening
    Degree of valgus tilt in ankle mortise
    Lateral shift of calcaneum in relation to weight bearing axis of tibia.
  • Operative treatment is indicated when ankle valgus deformity causes problems with orthrotic fitting and cannot be relieved by orthroses.
    • Tenodesis of tendoachilles to fibula above the physis if the valgus tilt is between 10-25 degrees.
    Valgus deformity is due to lateral compartment paralysis, so underdevelopment of fibula will be present, pulling down the fibula during weight bearing and dorsiflexion
  • Hemiepiphysiodesis of distal tibial epiphysis
    Done in skeletally immature patients.
    Medial tibialphysis is closed by direct surgical ablation or stapling or using maleolar screws.
    Growth arrest 0f the medial side combined with continued growth in the lateral side corrects the deformity.
    Advantage is immediate weight bearing.
  • Supramalleolarvarusderotationosteotomy
    Recommended in children older than 10 years.
    Seen in low lumbar level lesions.
    Severe fibular shortening 10-20mm
    Valgus tilt > 20 degrees
    External tibial torsion.
    Postoperatively above knee casts for 3 weeks followed by below knee casts for 2 weeks then full weight bearing.
  • Distal 1/3rd fibular oblique osteotomy is done followed by medial based wedge osteotomy above the epiphysis.Size of the wedge depends on valgus to be corrected.Fixation with plate and screws
  • Knee
    • Flexion contractures
    More common than extention contractures
    Seen in thoracic or lumbar level lesions
    contractures of 20 degree are common at birth
    Becomes fixed causes
    • the typical position assumed when supine—hips in abduction, flexion, and external rotation; knees in flexion; and feet in equinus;
    • gradual contracture of the hamstring and biceps muscles, with contracture of the posterior knee capsule from quadriceps weakness and prolonged sitting
    • spasticity of the hamstrings that may occur with the tethered cord syndrome;
    • hip flexion contracture or calcaneal deformity in the ambulatory patient. Knee flexion contractures
  • Knee flexion upto 20 degrees ---effective bracing and standing program
    20- 30 degree----Radical flexor release can be done
    30-45 degree supracondylarextentionosteotomy of femur is done
    If hip contractures are present both should be operated at the same time
  • Radical flexor release
    Minimal procedure – partial division of
  • supracondylarextentionosteotomy of femur
  • Extention contractures
    Due to unopposed quadriceps action
    Serial casting successful in some patients
    Surgical management consist of
    • V-Y quardriceps lengthening
    • Capsular release
    • Postrerior displacement of the hamstring muscle
  • V-Y quadriceps lengthening
    A, Detachment of rectus femoris tendon from rectus femoris, vastusmedialis, and vastuslateralis muscles; vastusmedialis and lateralis muscles are separated from iliotibial band, lateral hamstrings, medial hamstrings, and sartorius muscles. B, When knee is flexed, hamstring muscles and tensor fasciae latae slip posterior to knee axis, restoring normal function. Quadriceps muscles are repaired in lengthened position.
  • Valgus or varus deformity
    Supracondylar or tibialosteotomy with internal fixation can be done
    Hemiepiphysiodesis can be done using staples if recoganised early
  • Hip deformities
    Mainly due to:
    Muscle imbalance
    Congenital dysplasia
    Habitual posture
  • Flexion contracture
    Seen in high lumbar or thoracic level lesion.
    Caused due to unopposed action of hip flexors, habitual posture & spasticity of hip flexors.
    Flexion contracture of 20-30 degrees can be accomodated by lumbar lordosis and knee flexion.
    >30 degrees anterior hip release of sartorius, rectus femoris, iliopsoas, tensor fascia lata & anterior hip capsule.
    In this procedure, upto 60 degrees gets corrected.
    If flexion persistent subtrochanteric extension osteotomy.
  • Flexion abduction external rotation contracture
    Seen in thoracic level lesion.
    The occurrence of this deformity may be decreased by the use of night splints and range of motion exercises.
    Complete hip release done only when deformity interferes with bracing.
  • External rotation contracture
    Seen in low lumbar lesions.
    Initially bracing & physiotherapy.
    If persists subtrochanteric medial rotation osteotomy.
  • Abduction contracture
    Seen in high level lesions.
    Fascial release indicated when abduction contracture causes pelvic obliquity & scoliosis which inteferes with function or bracing.
    Fascial release fascia over gluteus medius, minimus & tensor fascia lata is divided and fasciotomy done.
  • Adduction contracture
    Seen in high level lesions.
    Due to spasticity and contracture of adductor muscles.
    Surgery indicated when it causes pelvic obliquity & inteferes with sitting & walking.
    Adductor release done.
  • Hip subluxation/dislocation
    Seen in 50% of children with meningomyelocele.
    Three types:
    Developmental treated by Pavlik harness, traction, closed reduction & hip spica application.
    Teratological seen in high level lesions, initially acetabulumdyplastic, with head of femur displaced proximally, should not be treated initially.
  • Paralytic
    Most common type- 50-70%
    Seen in low level lesions L3-L4.
    Due to imbalance between adduction & abduction forces.
    Principles of treatment:
    Obtain concentric reduction
    Correct bony abnormality
    Seek to balance flexor-adductor & extensor-abductor imbalance.
    Main goal – maximal function rather than radiograph reduction.
  • Reduction
    Should be atttempted only where child will derive considerable benefit from the procedure.
    Not always successful stiff painful hip
    If required anterior open reduction with capsulorrhaphy.
  • Correction of bony abnormality
    Femoral deformity corrected by proximal femoral resection & inter position arthroplasty.
    Done in severely retarded multiply handicapped with dislocated hips and severe adduction contraction.
  • Acetabular deformitycorrected by :
    Pemberton osteotomy
    Shelf procedure
    Steel triple innominateosteotomy
    Chiariosteotomy- preferred, as concentric reduction is not required & the osteotomy does not redirect the acetabulumposteriorly. It is in the direction of the paralytic dislocation.
  • ChiariosteotomyAn osteotomy of the pelvis is performed at the superior margin of the acetabulum, and the pelvis inferior to the osteotomy along with the femur is displaced medially
  • Muscle balancing procedures
    • Simple release of ilioposas tendon with adductor release
    Adductor longus tendon is sepereted from brevis and exiced
    Adductor brevis is separated from pectineus and lesser trochanter
    Identified ilioposas released
  • Posterior transfer of adductor muscle mass on the ischium to convert it into more of hip extensor.
    Transfer of iliopsoas tendon- 1st described by Mustard in 1952.anterolateraltransfer.mobilising the iliopsoas tendon with the lesser trochanter into the pelvis, re rooting it through a large trough in the ilium and through the gluteal muscles and re inserting the tendon into the greater trochanteric.
  • Sharrardprocedure:
    1959, posterior transfer of iliopsoas tendon.
    Transfer of iliopsoas muscle through a hole in the ilium underneath the femoral nerve with reinsertion of the tendon to the posterior greater trochanter through a drill hole and reattachment of iliacusportionto the outer wall of the ilium.
  • Transfer of external oblique muscle:
    Thomas and colleagues described transfer of external oblique abdominis to greater trochanter.
    Modified by Phillips and Lindseth adductors, external oblique & tensor fascia lata transferred.
    Adductors sutured to ischium.
    External oblique with aponeurosis to greater trochanter.
    TFL rotated externally and sutured to iliac crest proximally and tendon of gluteus distally
    mcKay described tendon transfer with femoral osteotomy.
  • Pelvic obliquity
    Three types–
    Infrapelvic contracture of adductors and TFL
    Supra pelvic uncompenseted scoliosis resulting in boney deformity of LS spine
    Pelvic boney deformity of sacrum and SI joint
    Infrapelvic –splinting , ROM exercises and position
    Suprapelvic—scoliosis by orthoses and spinal fusion
    Pelvic--- obliquty is 20 degree pelvic osteotomy is done
    Upto 40 degree gets corrected
  • Pelvic osteotomyPreoperative determination of size of iliac wedge to be removed and transferred. , Exposure of ilium. , After bilateral osteotomies and removal of wedge from low side, deformity is corrected. , Transferred iliac wedge is fixed with two Kirschner wires.
  • Spinal deformities
    Most common skeletal deformity
    Higher the lesion, greater the incidence.
    Deformity may be in the form of scoliosis, lordosis or kyphosis which may be either congenital, developmental or mixed.
    Congenital spinal deformities are managed as in any other patient.
    Developmental spinal deformity treated according to severity, evidence of progression and skeletal maturity.
    By using spinal braces for developmental deformities, the progression of the curve can be retarded until child is 10-12 years.
    Non operative treatment is not usually successful.
  • Overall goals are to correct deformity, preserve sitting balance, maintain level pelvis, preserve respiratory function, increase the trunk height.
    Preoperatively surgeon must ensure shunt function is stable, no UTI, weight bearing skin of pelvis and upper thigh are free of pressure sores, and skin over spine should be normal.
    Postoperatively patient must be managed in ICU until stable.
    Urinary tract must be kept clean to avoid infections.
  • Scoliosis
    Most common and usually progressive
    Incidence related to level of lesion.
    100% T12
    80% L2
    70% L3
    60% L4
    25% L5
    5% S1
    Thoracic lumbosacralorthrosis used if curve is upto 30°.
  • Indications for spinal fusion include a progressive increase in angular deformity that cannot be controlled by bracing, unacceptable deformities and progressive thoracic lordosis.
    Most cases require anterior and posterior procedures.
    Anterior procedure is done initially.
    Anterior release with discectomy & intervertebral fusion
    Anterior release with strut grafting
    Anterior fusion
    Correction with Zielke instrumentation.
  • 2nd stage posterior procedure is done 7 days after the anterior procedure.
    Choice of instrumentation includes:
    Square ended
    Harrington rods with sacral hooks
    Spinal instrumentation.
  • Kyphosis
    Seen in 10% patients with meningomyelocele.
    Associated with thoracic and lumbar lesions
    Apex deformity varies from T12-L5, most common L2
    Anterior and posterior procedures are recommended.
    Anterior done first consists of discectomies throughout the deformity with fusion accomplished by intervertebralarthrodesis or strut grafting.
    Posterior procedure is done 7 days later and involves long fusion with instrumentation to include the sacrum.
    Harrington or Lugue instrumentation is recommended.
    Goal of surgery is to align the trunk vertically over sacrum and pelvis.
  • Hyperlordosis
    Less common deformity
    Can lead to difficulty in sitting, intertriginous skin breakdown, difficulty with self catheterisation in females because of posterior rotation of perineum.
    Treatment by combination of anterior and posterior spinal release and posterior instrumentation.
    In rigid deformities, postural reduction in traction after spinal release, before definitive instrumentation may improve the deformity.
  • Hemimyelodysplasia
    Rare manifestation characterised by significantly assymetric involvement of lower extremities with one leg being significantly affected and the controlateral leg being normal or nearly normal.
    This condition is referred as hemi spina bifida.
    There may be limb length inequality due to paralysis induced growth inhibition on the affected side which may require orthotic management, epiphyseodesis or lenghthening.