Clinical Pathway for the Care of the Neonate with Myelomeningocele/Myeloschisis

Caring for the Neonate with Meningomyelocele

Spina bifida (myelomeningocele) is a birth defect of the spinal column in which a section of the spinal cord and spinal nerves are exposed through an opening in the back. Continuous exposure of the spinal cord in amniotic fluid is thought to result in progressive neurologic injury. Until recently, this defect was repaired post-birth when damage was already done. Currently, fetal surgery is an option for some.

A recent randomized, controlled clinical trial demonstrated that prenatal spina bifida repair resulted in improved outcomes to the degree that the benefits of the surgery outweigh the maternal risks.

The study found that prenatal repair resulted in:

Reference

A Randomized Trial of Prenatal versus Postnatal Repair of Myelomeningocele
N. Scott Adzick, M.D., Elizabeth A. Thom, Ph.D., Catherine Y. Spong, M.D., et al.
N Engl J Med 2011; 364:993-100.

PRE-NATAL REPAIR

If a mother whose fetus has an myelomeningocele is a candidate for prenatal spina bifida treatment, the surgery is performed between 19 and 25 weeks’ gestation. A pediatric neurosurgeon removes the MMC sac, if one is present, and closes skin over the defect to protect the spinal cord from exposure to the amniotic fluid. A patch is sometimes needed to close the defect. Delivery must be by cesarean section once fetal surgery is done.

Pre-natall Surgery for MMC may be offered at The Children’s Hospital of Philadelphia in the following circumstances:

  1. Myelomeningocele at level T1 through S1 with hindbrain herniation.
    • Lesion can extend below S1, but the highest level cannot be outside the T1-S1 range
    • Lesion level and hindbrain herniation will be confirmed by ultrasound and MRI
  2. Gestational age at the time of fetal surgery must be no greater than 25 weeks, 6 days.
  3. Maternal age ≥ 18 years
  4. Singleton pregnancy
  5. Written confirmation of normal karyotype, elevated AFAFP and positive acetycholinesterase (ACHE)

POST-NATAL REPAIR

If post-natal spina bifida treatment is recommended/selected, a C-section, is typically planned at 37 weeks.

The newborn undergoes repair of the defect using the same approach used with the fetal repair within 24 to 48 hours after birth.