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Peds Disorder-SB

Spina Bifida (Myelodysplasia)

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