Interdisciplinary Management of the Child with Spina Bifida 38th SBA

National Conference Samuel R. Rosenfeld, M.D.CHOC Childrens Hospital

Rancho Los Amigos National Rehabilitation Center

University of California, Irvine26 June 2011

Disclosure

Consultant, 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 Deformities

ParalysisPositioningMuscle imbalanceSpasticityMixed tone: spastic and flaccid

Motor Imbalance

AsymmetryAbsence of motorsPosition / gravity

Interdisciplinary TeamNurse practitioner / case managerOrthopaedic surgeonPediatricianNeurosurgeonUrologistPhysical therapistOccupational therapistOrthotistPsychologistSocial workerDietician

Goals of Interdisciplinary Management

Mainstream childrenDevelop independenceCompetence in the communityPersonality developmentTransition into adulthood

Be Aware of Fluctuating CNS PathologyFunctional deteriorationProgressive weaknessSpasticityScoliosis above the dysraphic defectCognitive impairmentFoot deformityIntrinsic hand atrophyNeurogenic bladder changes

Neurosurgical EvaluationCT BrainMRI brain, entire spinal cordCranial ultrasoundShunt surveyShunt O Gram

Neurosurgical InterventionClosure myelomengoceleResection leptomyelolipomaShunting hydrocephalusEndoscopic third ventriculostomyBrainstem decompressionFenestration of syrinxCentral canal subarachnoid shuntRelease spinal cord tethering

Urologic EvaluationRenal ultrasoundVoiding cystourethrogramUrodynamicsUro-flow with pelvic ultrasoundRenal scan

Urologic Intervention Smooth muscle relaxation (anticholinergic /

antispasmodic) oxybutynin (Ditropan) tolterodine (Detrol)

Clean intermittant catheterizationUrinary diversionVesicostomyBladder augmentationUreteral reimplantationContinent stoma

Bowel ManagementCone enemaAntegrade Continence Enema (ACE)

age 4 to 18 years median use every other day must have committed support from specialist nurse major psychological issues

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

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