Spina Bifida: the Orthopaedic Perspective

Maximizing Ambulatory Potential in Spina Bifida39th SBA National ConferenceSamuel R. Rosenfeld, M.D.CHOC Childrens HospitalRancho Los Amigos National Rehabilitation CenterUniversity of California, Irvine 30 June 2012 DisclosureConsultant, Zimmer Spine

I have no potential conflicts with this presentation

MyelodysplasiaCongenital defects of the vertebrae with neural element abnormalities

MyelomenigoceleExposed neural elements

MeningoceleVertebral arch defectsProtrusion of meningesIntact overlying skin

Caudal Regression SyndromeLumbar / sacral agenesisCloacal exstrophyMyelocystocele complex spinal dysraphism 5% of all covered spina bifida 50% associated with cloacal exstrophy all with hydrocephalus and hydromyelia

Associated Neural Axis DeformitiesArnold Chiari malformationHydrocephalusHydromyeliaSyringomyeliaArachnoid cystDiastematomyeliaSpinal cord tetheringLeptomyelolipoma

Associated Musculoskeletal DeformitiesParalysisPositioningMuscle imbalanceSpasticityMixed tone: spastic and flaccid

Motor ImbalanceAsymmetryAbsence of motorsPosition / gravity

Interdisciplinary TeamNurse practitioner / case managerOrthopaedic surgeonPediatricianNeurosurgeonUrologistPhysical therapistOccupational therapistOrthotistPsychologistSocial workerDieticianGoals of Interdisciplinary ManagementMainstream childrenDevelop independenceCompetence in the communityPersonality developmentTransition into adulthoodBe Aware of Fluctuating CNS PathologyFunctional deteriorationProgressive weaknessSpasticityScoliosis above the dysraphic defectCognitive impairmentFoot deformityIntrinsic hand atrophyNeurogenic bladder changes

Orthopaedic Surgery EvaluationScoliosis Xrays: sitting, standing, supineCT spineXrays of hips, knees, feet: standing, supineScanogramBone ageDexa bone densitometry

Orthopaedic InterventionCorrection spinal deformityHip managementKnee managementCorrection of foot deformity to facilitate orthotic managementOrthotic collaboration

#4

#18

What problems are unique to the child with Spina Bifida?What is the most significant physical impairment leading to the inability to maintain ambulatory status?

What is the most significant physical impairment leading to the inability to maintain independent sitting activities?Define Neurologic LevelsThoracicHigh LumbarLow LumbarSacralAmbulatorsWheelchairStraight spineLevel pelvisExtended hips / knees

Straight spineLevel pelvis Mobile hipsKnee flexionShoeable feetCriteria for ambulationPowerAntigravity musclesHip extensor > G+Knee extensor > F+Tricep surae > F+Criteria for ambulationRange of motionHip flexion contracture < 30 degreesKnee flexion contracture < 20 degreesBraceable hindfoot

Criteria for ambulationCrutchable upper extremitiesShoulder depressors > G+

Good gripFull elbow extensionTeres majorPectoralis majorLatissimus dorsi

Priority for ambulationEnergy efficiencySafetySpeedAppearance

Significant physical impairments leading to the inability to maintain ambulatory statusGluteus medius lurch, lateral trunk leanCrouched gaitKnee valgus (internal knee adductor moment)Knee flexion contractureTibial torsionAnkle calcaneal deformityEtiologic factors resulting in crouched gaitAnatomic (structural)Neurologic (paralytic)Spinal cord pathology (fluctuating level, spasticity)Anatomical (structural)Hip flexion contracture / lumbar kyphosisKnee flexion contractureShort fibulaAnkle calcaneal deformityRotational malalignment

Neurologic (paralytic)Absence of hip abductionMaintenance of hip flexor and quadricep strength with loss of hip extension and tricep surae powerNeuropathic joint, absence of proprioception

Spinal cord pathologyHydromyeliaSyringomyeliaDiastematomyeliaArnold-Chiari malformationSpinal cord tetheringLeptomyelolipoma Arachnoid cyst

Knee functional consequensesLack of plantar flexion strength excess knee flexionIncreased pelvic transverse motion increased transverse knee motion rotatory instability medial laxity

Orthotic managementRigid ankle to prevent dorsiflexionPrevent foot pronation, ankle eversionPosition ankle in mild plantarflexionGround (floor) reaction tibia posteriorExtend to toes with metatarsal pad to prevent toe clawing and protect insensate skinRear walker assistance

Knee flexion contractureConsider surgical intervention > 20 degrees hamstring lengthening iliotibial band lengthening posterior knee capsulotomy guided growth with anterior hemi-epiphysiodesis

Gradual orthotic correction with adjustable locked articulated ground reaction ankle foot orthotic system

Anterior hemi-epiphysiodesis

Hip flexion contractureConsider abandoning ambulatory programSurgical intervention > 30 degrees tendon lengthening hip capsulotomy reduction unilateral hip dislocation augment muscle powerProning programHKAFO, RGO, parapodium, standing frame

Significant physical impairment leading to inability to maintain independent sitting activitiesLumbar kyphosisPelvic obliquityHip contractures

Spinal orthotic managementSuspension TLSOWheelchair seating systems

Prevention of deformity and loss of functional skillsEarly aggressive managementOrthotic management coincidental with initiation of ambulatory skillsProtect insensate skinRoutine thorough neurologic re-evaluationInterdisciplinary careSurgery only to facilitate orthotic management