The Congress of Neurological Surgeons (CNS) has developed an evidence-based guideline for the treatment of patients with myelomeningocele. Executive summaries of the Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele were published today in Neurosurgery. Full text of the guidelines can also be found on cns.org.
The burden of disease is substantial and affects development and quality of life. Children with myelomeningocele require surgical intervention at birth to close the spinal defect. Most infants with myelomeningocele have hydrocephalus and require surgical treatment. There is currently little agreement among leading practitioners about the optimal timing of closure of the myelomeningocele defect of the spine. There are differences in practice with regards to team approach to closure, type of closure techniques, administration of antibiotics, and benefits of amputation or preservation of the neural placode. Additionally, as technology advances, in utero (or fetal) closure of the myelomeningocele defect has been advocated by many experts, but the long-term positive and negative effects of in utero closure have not been well studied. While in utero closure of myelomeningocele is an option for some pregnant women whose infants met criteria for diagnosis of Spina Bifida as fetuses, globally, this option is not readily available.
This systematic review was conducted to evaluate all available evidence to aid clinicians and guide clinical practice by determining the best options for management of pediatric myelomeningocele. Guidelines authors aimed to systematically review the literature and make evidence based recommendations about the timing of closure after birth, and to investigate the evidence concerning persistent ventriculomegaly and cognitive impairment. The goals of this effort were to discern the most effective strategies for a variety of myelomeningocele-related problems, including the timing and type of closure for myelomeningocele. The authors also considered the use of technical advances such as in utero or fetal closure, closure techniques, endoscopic treatment of hydrocephalus/ventriculomegaly, and other factors affecting infants and children with myelomeningocele. According to Dr. David Bauer, "it is the Guideline Task Force's aim that these systematic reviews and subsequent evidence based recommendations will lead to improvement in the quality of life for infants and children with myelomeningocele."
These clinical practice guidelines include five clinical questions pertaining to patients with myelomeningocele and provide seven recommendations.
This evidence based guideline and systematic review of the literature relevant to infants and children with myelomeningocele were accomplished in order to hopefully improve the quality of life for patients with myelomeningocele globally. Dr. Catherine Mazzola (Task Force Chair) states, "The life-long effects of myelomeningocele are profound and complex, and for children living in third world nations, may prove fatal, in many cases. It is through continued critical research efforts as specialists who treat these children that possibly one day researchers may find better treatment and eventually, a cure for myelomeningocele."
These guidelines were developed by the CNS and endorsed by the CNS, the American Association of Neurological Surgeons and the Spina Bifida Association.
Mazzola, M.A. et al. (2019) Congress of Neurological Surgeons Systematic Review and Evidence-Based Guidelines for Pediatric Myelomeningocele: Executive Summary. Neurosurgery. doi.org/10.1093/neuros/nyz261.