The Congress of Neurological Surgeons has created evidence-based guidelines for care of pediatric myelomeningocele. Guideline Task Force members created clinical questions, performed systematic review, and rated the quality of available evidence. The guidelines consist of seven recommendations, only one of which is supported by Level I evidence: Fetal closure of myelomeningocele is recommended to mothers/fetuses who meet inclusion criteria for the Management of Myelomeningocele Study (MOMS) as a method to reduce the risk of developing shunt-dependent hydrocephalus.
Two Level II guidelines also pertain to fetal myelomeningocele closure. For fetuses meeting MOMS criteria for fetal closure, fetal surgery may be associated with better ambulatory status in the short term (level 2), but there is insufficient evidence to make any recommendation regarding long-term ambulation (level 3). All children, whether their myelomeningocele was closed pre or post-natal, should have regular screening for development of tethered cord syndrome and/or inclusion cysts (level 2).
The authors found insufficient evidence to make any recommendations about the timing of post-natal myelomeningocele closure (within 48h versus later), or about the relationship between ventricular size and cognitive development (level 3).
These guidelines illustrate that, aside from MOMS, there is a shortage of high-quality medical literature to guide practice in care of children with myelomeningocele. Many questions that these authors attempted to address could not be answered due to insufficient evidence.