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Myelomeningocele

Guidelines

Key sources

The following summarized guidelines for the evaluation and management of myelomeningocele are prepared by our editorial team based on guidelines from the U.S. Preventive Services Task Force (USPSTF 2023), the Society of Obstetricians and Gynaecologists of Canada (SOGC 2021), the Congress of Neurological Surgeons (CNS 2019), the American College of Obstetricians and Gynecologists (ACOG 2017), and the World Health Organization ...
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Screening and diagnosis

Indications for screening: as per SOGC 2021 guidelines, obtain second-trimester anatomical ultrasound with detailed fetal intracranial and spinal imaging as the primary screening technology to detect fetal structural abnormalities including open and closed neural tube defects (anencephaly, encephalocele, myelomeningocele, and other spina bifida malformations).
B
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Diagnostic investigations

Diagnostic imaging: as per SOGC 2021 guidelines, consider obtaining prenatal MRI if further detailed assessment of the fetal CNS is required for diagnostic or management counseling.
B

Diagnostic procedures

Amniocentesis
As per SOGC 2021 guidelines:
Evaluate the amniotic fluid specimen for fetal genetic abnormalities, if a diagnostic amniocentesis is performed, following ultrasound detection of fetal anomalies including confirmed or suspected open or closed neural tube defects. Obtain chromosomal microarray and other genetic testing, as considered appropriate after assessment of fetal anomalies and family history, during evaluation with amniotic fluid α-fetoprotein and amniotic fluid acetylcholinesterase, if required by protocols for fetal surgery decisions.
A
Consider obtaining fetal exome sequencing in fetuses with myelomeningocele or other spina bifida anomalies, only after multidisciplinary counseling and after appropriate criteria for molecular genetic sequencing are met.
B

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  • Autopsy

Medical management

Setting of care: as per SOGC 2021 guidelines, refer patients with positive screening results on imaging for an open or closed neural tube defect (ultrasound with or without maternal serum α-fetoprotein) to experienced providers for confirmation, genetic/etiologic investigation and diagnosis, and pregnancy management counseling.
A

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  • Antibiotic prophylaxis for ventriculitis

Therapeutic procedures

Management of delivery
As per SOGC 2021 guidelines:
Consider offering either Cesarean or vaginal delivery for fetuses with myelomeningocele in a vertex presentation. Individualize intrapartum care based on head size, myelomeningocele lesion size, lower limb position, and mobility considerations.
C
Perform a pre-labor Cesarean delivery at 37 weeks at the latest to prevent possible rupture of the hysterotomy scar during labor if prenatal myelomeningocele surgical repair is conducted.
A

Surgical interventions

General principles: as per SOGC 2021 guidelines, offer a choice of 3 obstetrical care management options in families with an isolated open/closed neural tube defect after diagnostic and genetic testing:
prenatal surgical repair of myelomeningocele and prognosis
postnatal surgical repair of myelomeningocele and prognosis
pregnancy termination with autopsy.
A

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  • Prenatal surgical closure

Preventative measures

Periconceptional folic acid supplementation: as per USPSTF 2023 guidelines, offer folic acid supplementation of 400-800 µg/day in all individuals planning to or who could become pregnant, to prevent fetal neural tube defects.
A

Follow-up and surveillance

Post-closure follow-up
As per CNS 2019 guidelines:
Obtain careful follow-up in patients with either prenatal or postnatal closure for the development of tethered spinal cord with the associated loss of ambulatory function.
B
Obtain continued surveillance for tethered cord syndrome and/or the development of inclusion cysts in patients with prenatal and postnatal closure of myelomeningocele.
B

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  • Subsequent pregnancy planning

Quality improvement

Optimal folat concentrations: as per WHO 2015 guidelines, aim RBC folate concentrations at > 400 ng/mL (906 nmol/L) in females of reproductive age to achieve the greatest reduction of neural tube defects at the population level. Consider using this threshold as an indicator of folate insufficiency in females of reproductive age. Use this threshold only at the population level as it cannot predict the individual risk of having a neural tube defect-affected pregnancy because low folate concentrations cannot explain all cases of neural tube defects.
B
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