Surgical management of myelomeningocele in low-income and lower-middle-income countries: a systematic review

OBJECTIVE: Myelomeningocele (MMC) is one of the most severe forms of a neural tube defect, resulting in lifelong disability for children and their families. The lowest-income countries are thought to carry the highest burden of disease and have the greatest disparities in national folic acid fortification policies. Despite carrying the greatest burden, the surgical management and patient-related outcomes of MMC in these countries have not yet been described. The objective of this systematic review was to summarize clinical outcomes following surgical MMC closure among patients in the lowest-income countries, and to compare these findings with country-level preventative measures that improve patient outcomes. METHODS: Seven databases (PubMed MEDLINE, Embase, Scopus, Web of Science, Global Index Medicus, Latin American and Caribbean Health Science Literature, and Scientific Electronic Library Online) were searched from inception to June 2024 according to a priori study criteria. Inclusion criteria were studies that 1) were published in or translated into the English language, 2) had the full text available, 3) reported on surgical closure of MMC in a lower-middle-income country (LMIC) or low-income country (LIC), and 4) provided surgical outcomes. The proportion or risk of each outcome was estimated using disaggregated data from each paper. The findings were then compared to the national folic acid fortification policy status. RESULTS: Of 4640 studies, 27 met the inclusion criteria. Twelve of 80 (15%) LICs and LMICs reported postoperative outcomes for a total of 2967 patients who underwent postnatal surgery. The median age at surgery was 21 days (IQR 16-77 days). The median overall complication rate was 13% (IQR 8%-21%). Frequent 30-day postoperative complications included infection (median 16%, IQR 8%-24%), CSF leakage (median 12%, IQR 6%-18%), and wound dehiscence (median 8%, IQR 4%-17%). The median rate of any hydrocephalus was 64% (IQR 34%-77%). The median 30-day postoperative mortality rate was 9% (IQR 3%-9%), and the rates for 1-year, 5-year, and 10-year mortality were 17%, 29%, and 43%, respectively. The most commonly reported functional outcomes were ambulatory status and bladder function. CONCLUSIONS: In the lowest-income countries, among published studies all MMC closures occurred postnatally with delayed timing to surgical intervention, resulting in a higher risk of complications and mortality. Data regarding postoperative MMC outcomes were published for only 15% of LICs and LMICs. Global priorities should focus on identifying the highest-risk populations to prevent and effectively treat MMC.