Spina Bifida (Myelodysplasia)
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| Spina Bifida | neural tube defect, failure to close (typically at caudal end)
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| Etiology | no definite cause. links between maternal decreased folic acid prior to conception, hot tub soaks & exposure to teratogens (alcohol)
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| prognosis | good w/survival rate 90%
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| Repair lesion | surgery typically performed w/in the first 24hrs of life
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| Classification | aperta & occulta
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| Aperta (visible or open) | Myelomeningocel (most common)cyst includes CSF, meninges & herniated cord tissue. Meningocele cyst includes CSF & meninges - spinal cord intact
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| Occulta (hidden) | no spinal cord involvement, lacks closure of the spinous processes, maybe indicated by a tuft of hair or dimple on the low back
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| Clinical Presentation | sensory & motor impairments, hydrocephalus/chiari, seizures, tethered cord, orthopedic impairments, bowel/bladder dysfunction, cognitive impairments, integumentary, other
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| Sensory & Motor Impairments | thoracic-no LE function, high lumbar-hip flex & hip add, midlumbar-some knee ext, low lumbar-knee ext, some hamstrings, DF
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| Hydrocephalus/Chiari II Malformation | V/P shunt (80%) water on the brain
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| Seizsures | 15-20%
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| Tethered Cord | 6-12 years old (common)
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| Orthopedic Impairments | muscle weakness utero (clubfoot 50%), hip subluxation (muscle imbalance), scolosis 20% (congential or acquired), osteoporosis
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| Cognitive impairments | can have below avg cognitive abilities
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| other | obesity 20%, latex allergy up to 73% (due to being in hospital so long)
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| Associated Abnormalities | hydrocephalus
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| hydrocephalus | increased CSF in ventricles
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| Increased CSF in Ventricles due to | overproduction of CSF, obstruction of normal flow, Arnold Chiari II malformation
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| Arnold Chiari II malformation | deformity of the cerebellum, medulla & cervical spinal cord (posterior cerebellum herniates through foramen magnum causing obstruction of CSF flow between brain spinal cord
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| Hydrocephalus management | signs/symptoms & surgical shunt placement
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| Signs/Symptoms of Hydrocephalus | increased head circumference in infants & depressed vital signs, respiratory arrest
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| Surgical shunt placement for hydrocephalus | typically ventriculo-periotoneal (VP) shunt & thin flexible tube deviating CSF from ventricles to peritoneal cavity for reabsorption
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| Shunt Malfunction in Infant | vomiting 'sunsetting' eyes (downward deviation of eyes) irritability, increased head circumference
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| Shunt Malfunction in toddlers | vomiting, irritability, headaches, seizures, new nystagmus
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| Shunt malfunction in school-age | headaches, lethargy, irritability, seizures, edema/redness along shunt, personality changes, memory loss
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| Tethered Cord Sx | numbness/loss of sensation in LE, restless legs during sleep, spasticity in LE, clonus, decreased LE strength, gait deviations, PROGRESSIVE loss of control over bladder & bowel (incontinence)
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| PT Intervention | Thoracic level, high lumber, low lumber, sacral
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| PT intervention thoracic level | encourage WB as early as possible, standing frame - must!, functional mobility & play
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| PT intervention high lumber | place in hip abd/ER to counteract mm imbalance & develop acetabulum, standing frame (w/c primary mobility)
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| PT intervention low lumber | most of LE function available, ambulatory w/braces, possibly AD, focus on age-appropriate gross motor skill, strengthening, posture, decreasing deformities
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| PT intervention sacral | limitations w/bowel/bladder function, ankle/foot stability (bracing), strength, balance, coordination
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