Spina bifida (SB) is a congenital condition, in which the brain, spinal cord, and/or meninges do not develop normally.

The National Institute of Health (NIH) classifies SB into four types including: occulta, closed neural tube defects, meningocele, and myelomeningocele (MM). The scope of this inquiry should include patients with myelomeningocele. Myelomeningocele, which is the most severe type of SB, is characterized by the exposure of the spinal or neural tube through an opening in the spine.

This can cause partial or complete paralysis as well as urinary and bowel dysfunction.

For the purposes of this topic nomination, studies should, at minimum include 80% of patients with SB with MM who are 18 years of age. Studies should be published after 1966, and may be a full article report of a clinical study, but should not include meeting abstracts, editorials, letters, or a commentary. Studies may include prospective case series had to report baseline values, if applicable, but case series studies with non-consecutive enrollment of patients should be excluded. Studies reporting results of systematic reviews, meta-analyses, or guidelines developed by others should also be excluded. In addition to the above mentioned inclusion criteria, the topic nomination focuses on the following key questions for research: TOPIC 1: Is the rate of treatment for hydrocephalus (HC) in children with SB, who have a MM, higher or lower in infants who underwent fetal closure? Compared to standard, post-natal closure? Does in utero closure of myelomeningocele decrease the rate of shunt placement in children with SB?

TOPIC 2: Does in utero closure affect or improve ambulatory status as compared to post-natal closure for infants with SB with MM? TOPIC 3: Does Chiari II decompression in children with SB improve outcome? TOPIC 4: Does closure of the MM defect within 24 - 48 hours reduce the rate of infection for infants with SB?

TOPIC 5: Is persistent ventriculomegaly (VM) associated with cognitive impairment in children with SB?

TOPIC 6: Does shunt placement at the time of closure decrease the rate of cerebrospinal fluid (CSF) leak or infection in infants with MM with SB?

TOPIC 7: Does a team approach to closure with skin and/or muscle flaps of MM affect the rate of CSF leak or wound healing?

TOPIC 8: Is the risk/rate of tethering for infants/children with SB higher in infants who had in utero closure than in infants with MM closed post-natally?

TOPIC 9: Does placode amputation decrease the risk of tethering of the spinal cord in infants with SB and an open spinal defect?

TOPIC 10: Does de-tethering really improve bladder function/urinary dysfunction in children with SB or MM?

Background and Burden of Disease According to the NIH, SB is the most common neural tube defect in the United States . A total of 1,500 to 2,000 babies born in the United States every year are born with SB.1 Infants who are born with MM have a death rate of approximately 10%, according to the MOMS study. The NIH also estimates approximately166,000 individuals currently live with SB, in the US.1

Data from the National Birth Defects Prevention Network also shows a higher reported incidence in Hispanic women (3.80 per 10,000 live births) than in Non-Hispanic black or African-American women (2.73 per 10,000 live births) or in women identified as Non-Hispanic white (3.09 per 10,000 live births). 1 It is also important to note the incidence of SB in the world is likely under-reported, due to the limitations of surveillance data collection. Recent changes in the International Classification of Disease (ICD) coding system from ICD9 to ICD10 may also contribute to the under-reporting of SB internationally. The risk of disease is also higher in vulnerable populations (women with low socioeconomic status and/or women who reside in low socioeconomic status areas) where mothers of infants with SB often do not receive the necessary health care and/or access to adequate vitamins/nutrition. Spina bifida drastically affects development and quality of life. Research has shown SB patient outcomes are poorer in patients without private insurance.

Current Clinical Practice

There is a strong need for clinical practice guidelines on this topic. There is currently little agreement among leading practitioners about the optimal timing of closure of the MM 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 MM 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 MM is an option for some women pregnant with fetuses with SB, globally, this option is not readily available. Additionally, the effect of HC and/or ventriculomegaly (VM) on infants and children with SB has not been well reported. Some experts advocate choroid plexus coagulation (CPC) and endoscopic third ventriculostomies (ETV) for children with HC or VM. Others doubt or have concerns about the long-term efficacy of these options and opt instead to insert a ventriculoperitoneal shunting (VPS) at birth.

Other experts advocate conservative non-surgical management of HC and VM because of possible complications of VPS.

The purpose of this topic nomination is to evaluate all available evidence, from which, the Congress of Neurological Surgeons (CNS) will develop a guideline to aid clinicians and guide clinical practice by determining the best options for management of pediatric patients with SB with MM. The CNS maintains in-house infrastructure to lead, promote, and support the creation and methodological processes to produce evidence-based guidelines, which are critical tools to confront a rapidly changing health care environment. Using the CNS s high quality, rigorous methodological process, a multidisciplinary task force will develop recommendations based on the available evidence provided by the Evidence Based Practice Center. Throughout development, the task force will use evidence-based methodologies and strictly adhere to a priori defined criteria as defined by the Institute of Medicine s (IOM) standards for conducting systematic reviews and clinical evidence-based guidelines.

This topic was initiated by the Section on Pediatric Neurosurgery of the American Academy of Neurological Surgeons (AANS)/CNS. The CNS also recruited from a variety of institutions and subspecialty disciplines in an effort to have as broad a representation of opinions and expertise as possible, including representatives from urology, neurology, and neonatology. It is CNS s goal to follow the IOM recommendations to be inclusive and inter-disciplinary when constructing our guidelines and subsequent recommendations.7 A conscientious effort will also be made to be sure that any conflict of interest is fully disclosed and avoided. Participants who have published extensively in certain areas will be asked to recuse themselves from voting and will be assigned to evaluate evidence in other topics. Every effort will be made to ensure that the guideline is accurate, reliable, and non-biased. The CNS guidelines attempt to provide essential information for clinicians, globally, helping to improve patient care and outcomes. In addition to developing high quality guidelines, CNS is also committed to dissemination of guidelines in multiple, open access formats, such as publication in peer-reviewed journals, publication on the CNS webpage, webinars, conference seminars and courses, as well as other promotional efforts.

Please choose a description that best describes your role or perspective: (you may select more than one category if appropriate)

professional society

If you are you making a suggestion on behalf of an organization, please state the name of the organization

Congress of Neurological Surgeons

Please tell us how you heard about the Effective Health Care Program

previous experience, colleagues, and other partners