RISK FACTORS:1st trimester maternal valproic acid and derivatives (valproate sodium) use, High risk pregnancy - previous children with spina bifida, Insufficient maternal levels of folic acid,>90% of spina bifida are the product of low-risk pregnancy
RISK FACTORS
β’ 1st trimester maternal valproic acid and derivatives (valproate sodium) use
β’ High risk pregnancy - previous children with spina bifi da
β’ Insufficient maternal levels of folic acid
β’ >90% of spina bifida infants are the product of low-risk pregnancies
GENERAL MEASURES
β’ Multidisciplinary approach: Pediatric neurosurgery, orthopedics, urology, nursing, social services, pediatrics
and physical therapy
β’ Most patients with myelomeningocele have neurogenic bladder necessitating intermittent catheterization to
prevent severe secondary urologic disorders
SURGICAL MEASURES
β’ Myelomeningocele repair - ideally within 24-48 hours of birth
β’ CSF diversion (usually ventriculoperitoneal shunt) usually required at birth or shortly thereafter
β’ Orthopedic correction of extremity and spinal deformities is more elective but requires evaluation early
β’ Infants with clinical evidence of hindbrain compression despite adequate CSF diversion require prompt
posterior fossa decompression
β’ Prenatal (fetal) surgery to close myelomeningocele defects is being tested in several institutions, but remains
an investigative procedure
ACTIVITY
β’ Determined by level of the lesion
β’ Optimized by physical therapists and multidisciplinary team
DIET
β’ Obesity a major cause of morbidity in myelomeningocele patients
β’ Modified as needed to facilitate bowel and bladder training
PATIENT EDUCATION
β’ Genetic counseling
β’ Signs and symptoms of shunt malfunction
β’ Bowel/bladder care
PATIENT MONITORING Regular followup in spina bifida clinic, with multidisciplinary assessment including pediatrics, neurosurgery, orthopedics, and urology
PREVENTION/AVOIDANCE
β’ Adequate folate (0.4 mg/day) intake by sexually active women before pregnancy and through fi rst trimester
β’ For women with prior NTD-affected pregnancy, give 4 mg/day of folate before conception and through first
trimester
POSSIBLE COMPLICATIONS
β’ Late neurological deterioration due to tethering of spinal cord
β’ Shunt obstruction: headache, nausea and vomiting, visual disturbances, cognitive difficulty; the latter two
problems may be chronic, unaccompanied by headache or vomiting
β’ Shunt obstruction may result in hydromyelia, which may manifest only with intrinsic muscle weakness of the
hands
β’ Inadequate bladder hygiene can result in hydronephrosis progressing to renal failure
β’ Seizures may result from cortical migration disorders or herald shunt malfunction
β’ Myelomeningocele patients are at risk for latex allergy.
Use latex precautions.
EXPECTED COURSE/PROGNOSIS
β’ >80% of treated patients with open neural tube defects have normal IQ
β’ Since 1970s, management techniques have improved, a result of the creation of multidisciplinary spina bifida
clinics
β’ Shunt infection and malfunction less common, but still a major cause of morbidity
β’ Generally, early prediction of motor and intellectual outcome in neonates with Chiari II and myelomeningocele
is hazardous
β’ Infants with head circumference > 50 cm at birth (eg, severe hydrocephalus) have dismal cognitive prognosis