Spina Bifida Lecture Notes PDF
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Dr. Dragana Djurić
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Summary
These lecture notes provide an introduction to spina bifida, covering its etiology, diagnosis, and associated complications. Key aspects include neural tube defects, different types of spinal bifida like myelomeningocele and meningocele, and the impacts on a patient's development. A crucial note is the importance of folic acid in preventing this condition.
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Introduction to Spina bifida PTY224 LEC 5.1 BY: DR.DRAGANA DJURIC Objectives â–ªDescribe SB, epidemiology & aetiology â–ª Problems and medical/surgical management of SB â–ªComplications of SB â–ªAssessment and outcome measures â–ªKey features of physiotherapy management of client with SB (Infancy, adolescen...
Introduction to Spina bifida PTY224 LEC 5.1 BY: DR.DRAGANA DJURIC Objectives â–ªDescribe SB, epidemiology & aetiology â–ª Problems and medical/surgical management of SB â–ªComplications of SB â–ªAssessment and outcome measures â–ªKey features of physiotherapy management of client with SB (Infancy, adolescence, adulthood) Spina Bifida definitions â–ªNeural tube defect – occurs in embryo â–ªSynonyms: â–ªSpina bifida â–ªMyelomeningocele or meningomyelocele â–ªMeningocele Occurs at around 21 days (as an embryo) Occurs due to failure of closure of the neural tube to form spinal cord and vertebrae. Subsequent abnormal mesodermal (muscle & nervous tissue) development 1.Myelomeningocele â–ªSac containing neural material protrudes through spinal vertebrae. â–ªNerves at level of sac and below sac are affected 2. Meningocele â–ªNO neural material in sac â–ªDefect contains CSF and meninges only â–ªNO CNS dysfunction 3. Spina bifida occulta (‘hidden’) â–ªNonfusion of the halves of the vertebral arches â–ªNo disturbance of underlying neural tissue â–ªMay have tuft of hair overlying â–ª4-8% incidence in general population Etiology â–ª? Genetic (chromosomal abnormalities, racial variation in incidence, risk for siblings 2-3%) â–ª? Environmental (? Teratogens – higher incidence in Celts, low incidence in African blacks) â–ª? Dietary factors (high maternal alcohol, anti- epileptic meds, low level of maternal folic acid prior to conception) â–ªNo definitive cause identified â–ªLikely to be multifactorial Etiology â–ª Affects 1:1000 pregnancies â–ª Prenatal diagnoses â–ª Able to be detected by ultrasound at 18weeks gestation â–ª Alphafetoprotein levels screening, amniotic fluid analysis â–ª (23% pregnancies where prenatal diagnoses –terminated ) â–ª survival of high SB defects is low in utero, hence tend to see mainly mid to low thoracic and lumbar defects â–ª *trauma to neural sac during vaginal delivery, hence tend to elect for C-section as delivery mode Prevention: Folic acid Vitamin- food and supplement Folic acid should be taken daily in the upcoming months before conceiving and also during pregnancy 400 micrograms folic acid tablet every day before you're pregnant and until you're 12 weeks pregnant Intra-uterine repair â–ªControversy regarding surgical repair of sac in utero â–ªMay reduce further damage to remaining nerves â–ªMay reduce cerebral consequences- reduce shunt, Chiari II symptoms â–ªEvidence based? Repair of sac in 21/52 fetus Signs & symptoms â–ªRelated to the level of the spinal defect â–ª Paralysis distal to the defect (Usually have flaccid paralysis rather than spastic paralysis ) â–ª Sensory loss (may not exactly correlate with motor loss) â–ª Bowel and bladder dysfunction Bladder & bowel dysfunctions Neurogenic bladder 95%, of patient- innervation of bladder which arise at the sacral level of the spinal cord. Neurogenic bladder- urinary conditions in people who lack bladder control due to a brain, spinal cord or nerve problem Urologist – follow up Training before 7 years- catheterization, self catheterization( intermittent catheterization), - role of nursing Catheterization, self- Brain signs and symptoms â–ªMany patients will have associated brain and brainstem symptoms â–ªCognitive, sensory and perceptual motor function may be impaired â–ªLower IQ than population mean (tho’ this is debated) ▪‘learning disabilities’ 80% Arnold Chiari malformation â–ªAssociated malformation of brain – tendency for posterior lobes/cerebellum to be located further inferiorly â–ªBoth the cerebellum and brain stem tissue extend into the foramen magnum (the hole at the skull base for passing of the spinal cord). â–ªCNS abnormalities â–ªArnold Chiari malformation is considered to be primary cause of hydrocephalus â–ªSubsequent hydrocephalus- abnormal accumulation of CSF Hydrocephalus PT Assessment â–ªFunctional level classification systems : Spina Bifida Neurological Scale (SBNS) â–ªTo determine the motor level, the PT tests the strength of key muscle groups â–ªLight touch and pinprick sensation should be reassessed through the use of cotton balls and a sterilized safety pin, respectively, in each dermatomal level â–ªEvaluation of ROM should also be completed Outcome measures â–ªBattelle Developmental Inventory â–ª Pediatric Evaluation of Disability Inventory (PEDI)- functional skill level, caregiver assistance, and modifications or adaptations used. â–ª WeeFIM -impact of functional impairments for children of preschool and young elementary school age. â–ª 18-item, 7-level scale that assesses a child’s consistent performance in three primary domains (self-care, mobility, and cognition) â–ªSpina Bifida Health-Related Quality of Life Musculoskeletal deformities â–ªLacking postural stability and symmetrical alignment â–ªDelayed motor development ( sitting at 11 months ) â–ªPostural deviations and contracture â–ªScoliosis, kyphosis, lordosis- in children with higher spinal lesion â–ªHip contracture, deformity of femur and acetabulum-1/3 to half of children Musculoskeletal deformities â–ªTorsional deformities -External or internal tibial torsion â–ªKnee- Genu Varus, genu Valgus â–ªKnee Fl or EX contracture â–ª (Thoracic & High lumbar SB: more than 70% had knee joint contractures) Foot deformities common due to unopposed muscle action around ankle â–ª Calcaneovarus or calcaneovalgus foot due to absence of gastroc/soleus â–ª Talipes equinovarus (clubfoot) due to L4 or L5 lesion â–ª In walking and in non ambulatory patient Orthopaedic deformities on spine, hip, knee and foot â–ªNegatively affect â–ª Positioning â–ª Body image â–ª Weight bearing (in sitting and standing) â–ª ADL â–ª Energy expenditure â–ª Mobility from infancy through adulthood Gait defects: ‘crouch standing’ â–ªPersistent hip & knee flexion,ï‚ lumbar lordosis â–ªMay occur due to muscle weakness eg insufficient soleus strength to maintain tibia vertical â–ªMay occur due to orthopaedic deformities eg calcaneal valgus resulting in tibial internal rotation & knee flexion â–ªHip & knee flexion contractures may develop ? To walk or not to walk â–ªIncreasing demand to walk further and faster, changes in social and environmental demands â–ªGait aids may only be suitable indoors â–ªIncreasing energy expenditure due to heavier body through adolescence â–ªIncreasing demands on balance due to increased height â–ªDevelopment of obesity â–ªReduction in gait due to foot ulcers, subsequent immobility during healing time Medical/surgical management â–ªExternal closure of defect – reduce risk of CNS infection, reduce risk of further damage to spinal nerves 24- 72 hours after delivery â–ª Ventriculoperitoneai (VP) shunt for hydrocephalus â–ªHydrocephalus 80-95% of SB cases (Shaer, 2007) â–ªShunt –in first week of life or in the first 6 month of life â–ªComplications: infection, blockage, multiple revision Ventriculo- peritoneal (VP) shunt Clubfoot â–ª 30–50% of patients. â–ª Conservative management: The Ponseti method -serial manipulation and long-leg casting to gradually correct the clubfoot deformity. â–ªSurgery for severe cases: soft-tissue release surgery between 10-12 month of age. Before: note lumbar scoliosis After surgery: note improved sitting balance Other Complications â–ªPressure sores â–ª Occur in 85 – 95% of SB by adulthood â–ª Due to pressure from wheelchair, cast or brace; urine/stool soiling causing skin breakdown; friction/shear origin; â–ª Osteoporosis â–ª Fractures due to decreased bone mineral density (no evidence that passive weight bearing will decrease fracture rates) â–ª Latex allergy â–ª 18 – 40% of SB children have allergy (compared to 1-5% of controls) â–ª (latex present in gloves, wheelchair seats & tires, foam lining on splints and braces etc) Neurological Complications â–ªShunt blockage (drowsy, HA, seizures), VP shunt failure ▪‘tethering’ of cord leading to progression of neurological spinal symptoms – eg onset of upper limb weakness â–ªpronounced spasticity, General physical therapy plan of care/goals: â–ªPrevent loss of range of motion (ROM) in upper and lower extremities as well as upper extremity overuse injuries; â–ªmaximize independent functional mobility; â–ªoptimize health-related quality of life; â–ªmaximize health and fitness including strength and aerobic capacity Physical therapy interventions: â–ªPatient and family education regarding long-term implications of SB and the risks for obesity, osteoporosis, and urinary tract infections; â–ªexercises to maintain ROM and improve strength; â–ªfunctional mobility training (transfers, wheeled mobility, ambulation) â–ªPrecautions during physical therapy: â–ªDecreased cardiorespiratory endurance â–ªincreased risk for fracture secondary to osteoporosis; increased risk for hip dislocation and skin breakdown secondary to decreased sensation; â–ª latex allergy; â–ªincontinence during exercise; â–ª difficulties with thermoregulation; increased risk of falls; â–ªincreased spasticity with exercise Management- EBP â–ªUpper extremity strength training programs increase strength and independent wheelchair mobility in children with spina bifida â–ª Treadmill training improves gait speed and aerobic fitness in ambulatory children with spina bifida Mobility training using braces â–ªHip KAFO â–ªRGO â–ªKAFO â–ªAFO Walker, twisting cable Weight bearing opportunities Gait aids School time â–ªClassroom seating and the potential need for adaptive seating or standing devices should be evaluated. â–ªFunctional transfers including sit-to-stand and floor-to-stand transfers should be assessed. â–ªThe School Function Assessment (SFA)-assessment of the extent to which the child’s impairments impact school-related tasks such as â–ªmoving around the classroom and school â–ª interacting with peers and teachers â–ª and using classroom materials. Wheelchairs Spina bifida - adolescence â–ªReview mobility needs, energy expenditure and social/environmental goals, â–ªOptimise activity to minimise obesity risk â–ªTrain for self reliance (stretches, pressure care, continence) â–ªMx of ‘over use’ – early degenerative changes in shoulders, other heavily used joints (OA, ligamentous instability) Summary â–ªSpina bifida results from a defect in formation of the neural tube in the embryo â–ªMay present with spinal and brain neurological deficits â–ªAssociated musculoskeletal deformities â–ªAt high risk of multiple complications due to effect of abnormal movement upon growing child â–ªNeed life-long management of condition by physiotherapist and multidisciplinary team References â–ªPalisano, R., Orlin, M. Schreiber, J. (2021). Campbell’s Physical Therapy for Children (6 th ed.). Missouri: Elsevier Saunders â–ªMc Keogh, E et al. (2021). Tecklin’s Pediatric physical therapy (6th ed.). Walters Kluwer Health References â–ªOliveira A, Jácome C, Marques A. Physical fitness and exercise training on individuals with spina bifida: a systematic review. Res Dev Disabil. 2014;35:1119–1136. â–ªMahony K, Hunt A, Daley D, Sims S, Adams R. Inter-tester reliability and precision of manual muscle testing and hand-held dynamometry in lower limb muscles of children with spina bifida. Phys Occup Ther Pediatr. 2009;29:44–59. â–ªde Groot JF, Takken T, van Brussel M et al.. Randomized controlled study of home-based treadmill training for ambulatory children with spina bifida. Neurorehabil Neural Repair. 2011;25:597–606. References â–ªZwinkels M, Verschuren O, Janssen TW et al.. Exercise training programs to improve hand rim wheelchair propulsion capacity: a systematic review. Clin Rehabil. 2014;28:847–861. â–ªhttp://www.asbha.org.au/ â–ªhttp://www.ninds.nih.gov/disorders/spina_bifida/spina_bifida.ht m
