Hydrocephalus-Associated Early Mortality in Myelomeningocele: A 22-Year Institutional Experience.

Abstract

Myelomeningocele (MMC) is associated with substantial early-life morbidity and mortality. Although hydrocephalus frequently accompanies MMC, its specific relationship to early mortality remains incompletely defined in institutional cohorts. This study aimed to describe early mortality patterns in patients with MMC and to examine the association between hydrocephalus, shunt-related complications, and survival within a 22-year single-center experience. We conducted a retrospective cohort study of patients who underwent surgical repair for MMC between January 2002 and March 2024 at a tertiary center. Demographic variables, hydrocephalus status, ventriculoperitoneal shunt placement, shunt-related complications, and mortality were reviewed. Survival was analyzed using Kaplan-Meier methods, and Fisher's exact test was used for group comparisons. Given the limited number of events, analyses were primarily descriptive and hypothesis-generating. Twenty-eight patients were included, with a mean follow-up of 5 years. Overall mortality was 25% (7/28), and all deaths occurred within the first five years of life. Hydrocephalus requiring ventriculoperitoneal shunt placement was present in 22 patients (79%). Mortality occurred only among shunt-dependent patients (7/22) and was not observed in patients without hydrocephalus (0/6) (Fisher's exact P= 0.29). Most deaths were associated with shunt-related complications (5/7, 71%), particularly infection and malfunction, although systemic causes were also identified. In this single-center experience, early mortality in MMC clustered within the first five years and was observed only in shunt-dependent patients. Within the limitations of a small cohort, hydrocephalus may represent a marker of early vulnerability rather than a causal determinant. Structured surveillance during early childhood remains essential.