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Neural Tube Defects, Pediatric

Eric B. Levey, MD and Sarah A. Korth, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

Neural tube defects (NTDs): CNS malformations due to abnormalities of neural tube closure during early embryonic development that can be either open or closed defects. Spina bifida (...

DIAGNOSIS

HISTORY

  • Anencephaly, occipital encephaloceles, and open forms of SB are usually diagnosed prenatally and are obvious at birth.

  • Occult frontal encephaloceles may come to attention because of de...

TREATMENT

GENERAL MEASURES

  • Route of delivery

    • For most NTDs with vertex presentation, no clear benefit of cesarean section (CS)

    • Infants who undergo fetal surgery delivered by CS

  • Clean intermittent catheter...

ONGOING CARE

Children with symptomatic SB ideally should be followed in a multidisciplinary SB clinic that includes neurosurgery, urology, and orthopedics as well as a medical generalist (pediatrics, ...

ADDITIONAL READING

  • Adzick NS, Thom EA, Spong CY, et al; for MOMS Investigators. A randomized trial of prenatal versus postnatal repair of myelomeningocele. N Engl J Med.  2011;364(11):993–1004. [...

CODES

ICD9

  • 740.0 Anencephalus

  • 742.0 Encephalocele

  • 756.17 Spina bifida occulta

  • 741.93 Spina bifida without mention of hydrocephalus, lumbar region

  • 741.00 Spina bifida with hydrocephalus, unspecified region

  • 7...

FAQ

  • Q: Are neural defects genetic?

  • A: Genetic factors are definitely involved, but in most cases, a single genetic cause is not found.

  • Q: What is the chance of having another child with an NTD?

  • A: Recurr...

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