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Detailed information of MENINGOCELE
MENINGOCELE
DEFINITION:
A neural tube defect affecting the spine resulting in the
herniation of the meninges through a vertebral defect in the lumbar
region.
EPIDEMIOLOGY:
- incidence: 1-2/1000 live births (of spina bifida cystica)
- age of onset:
- less than 26 days gestational age
- risk factors:
- multifactorial inheritance pattern:
TYPES:
1. Posterior Meningocele
- most common type of meningocele
- hernia contains no neural tissue
- projects externally through a defect in the posterior aspect
of the vertebral arch
- usually covered by a thick -> thin layer of skin or membrane
- may or may not leak CSF
- usually not associated with any neurologic deficiencies
2. Anterior Meningocele
- uncommon type of meningocele
- hernia contains no neural tissue
- projects internally through a defect in the anterior aspect
of the vertebrae into the pelvis
- complications include:
- constipation and/or bladder dysfunction
- females may have associated anomalies of the GU tract
- retrovaginal fistula
- vaginal septa
CLINICAL FEATURES:
1. Meningocele
- a fluid-filled midline mass that may transilluminate
- usually in the lumbar region but also in the cervical region
- covered with skin or membrane:
- skin-covered
- found along the neuroaxis
- usually free of the associated anomalies
- membrane-covered
- usually found rostrally
- more likely to be associated with anomalies
2. Complications
- these depend on the level and severity of the lesion
- bladder and bowel sphincter dysfunction
- orthopedic problems:
- equinovarus deformity, club foot, gait disturbances
INVESTIGATIONS:
1. Imaging Studies
1. Vertebral X-Ray
- defect in the closure of the vertebral arches and laminae
2. CT/MRI
- to rule out associated anomalies and to determine the
extent of neural tissue involvement
2. Prenatal Diagnosis
- elevated maternal serum alpha-feto-protein (AFP)
- level II ultrasound
- amniocentesis - elevated AFP and acetylcholinesterase
MANAGEMENT:
1. Surgery
- correction to prevent infection
- immediate if membrane covered +/- CSF leak
2. Supportive
- bowel and bladder problems
- orthopedic problems
- genetic counselling
3. Prevention
- women in the child-bearing years should be encouraged to eat
foods rich in folic acid (orange juice, broccoli, whole grains,
beans, peanut butter, spinach, peas)
- women who plan to become pregnant should take a supplement
of 0.4 mg of folic acid daily
- decrease recurrence rate in patients at risk with 4 mg of
folic acid daily from the time of conception through the first
trimester
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Pediatric Database - MENINGOCELE
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