Long-Term Results of Surgical Treatment for Congenital Hydrocephalus: A Comprehensive Systematic Review and Meta-Analysis

Introduction: Congenital hydrocephalus, particularly prevalent in low-income countries, lacks robust evidence on long-term outcomes. Therefore, the authors conducted a systematic review and single-arm meta-analysis to address this gap.

Methods: Fulfilling PRISMA guidelines, the researchers systematically explored the Embase, PubMed, and Web of Science databases to uncover articles reporting cohorts of more than four patients with congenital hydrocephalus being followed-up for at least five years or more. Congenital hydrocephalus was defined as any pathological condition affecting the cerebrospinal fluid dynamics during the early stages of life. The evaluated outcomes encompassed: revisions, procedure-related mortality, overall mortality, infections, and cognitive development.

Results: From 4,788 studies initially found, 8 studies with 962 patients were included in the analysis. Myelomeningocele and post-hemorrhagic hydrocephalus accounted for 38.9% and 18.9% of cases, respectively. Treatments included ventriculoatrial shunts in 64.3% and ventriculoperitoneal shunts in 34.7%. The median follow-up period was 10 years (range: 5.5-45 years). Revisions occurred in 455 patients at a rate of 77% (95% CI: 62%-91%; I2=95%). Infections were seen in 172 patients, resulting in a 17% (95% CI: 8%-26%; I2=92%) risk. A total of 185 patients died, providing a mortality rate of 20% (95% CI: 5%-35%; I2=97%). Procedure-related deaths were observed in 28 patients of 605, showing a risk of 21% (95% CI: 3%-39%; I2=98%). Cognitive impairment was reported in 72 out of 320 patients in three studies.

Conclusion : Congenital hydrocephalus represents a serious medical condition. A substantial proportion of patients will require some form of revision. The mortality rate after five years from initial treatment remains high. The outcomes display significant heterogeneity, making it challenging to draw definitive conclusions. What remains is the heavy burden that congenital hydrocephalus places on affected individuals, particularly in low-income countries.