Neonate: Myelomeningocele/Myeloschisis — Caring for the Neonate with Meningomyelocele — Clinical Pathway: ICU

Neonatal Myelomeningocele/Myeloschisis Clinical Pathway — ICU

Caring for the Neonate with Myelomeningocele

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. A pediatric neurosurgeon will release the spinal cord from the MMC sac, if one is present, and close multiple layers over the defect to protect the spinal cord. Historically, this defect was repaired post-birth when damage was already done. Currently, fetal surgery is an option for some neonates.

A 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:

  • Reduced hindbrain herniation component of the Chiari II malformation
  • Reduced need for ventricular shunting
  • Reduced incidence or severity of neurologic morbidity, such as impaired motor function

Reference

A Randomized Trial of Prenatal Versus Postnatal Repair of Myelomeningocele  

Prenatal Closure

If a mother whose fetus has a myelomeningocele is a candidate for prenatal spina bifida treatment, the surgery is performed between 23 and 25 6/7 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. With our new myofascial repair techniques, a patch is rarely needed but always available to close the defect. Delivery must be by C-section once fetal surgery is done.

Prenatal 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 6/7
  3. Maternal age ≥ 18 years
  4. Singleton pregnancy
  5. Written confirmation of normal karyotype, elevated AFAFP and positive acetylcholinesterase (ACHE)

Postnatal Closure

If postnatal spina bifida treatment is recommended/selected, a C-section is typically planned at 37-38 weeks.

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