Congenital Hydrocephalus in Central and Southern Tunisia: A 15-Year Retrospective Study of 102 Patients on the Clinical Spectrum, Management, and High Burden of Neural Tube Defects.

Abstract

Congenital hydrocephalus (CH) represents a significant cause of pediatric morbidity. This study characterizes the clinical presentation, epidemiology, and long-term neurodevelopmental outcomes of children with CH, with emphasis on the role of prenatal diagnosis. Retrospective analysis of 102 children with CH who underwent neurosurgical intervention at the Department of Neurosurgery, Habib Bourguiba Hospital, Sfax, Tunisia, over 15 years. Data regarding demographics, clinical presentation, etiology, surgical outcomes, and long-term neurodevelopmental outcomes were systematically analyzed. Estimated incidence was 0.1 per 1000 live births, with median age at presentation of 7 months (male predominance, sex ratio 1.4:1). Familial history was documented in 8% of cases. Presenting features included increased head circumference (89%) and bulging fontanelle (70%). Hydrocephalus was associated with myelomeningocele in 38 cases (20% prenatally detected), with spina bifida representing the most common etiology (40%). Postoperative complications occurred in 43% of cases. Long-term follow-up (1 month to 16 years; median: 4 years) of 55 patients demonstrated that 32 (58%) attended mainstream school. Among 27 patients with sphincter dysfunction, 23 (85%) had concurrent myelomeningocele. Normal speech development occurred in 39%, while 15 patients (15%) exhibited paraplegia and 22 (22%) developed seizure disorder. Congenital hydrocephalus management requires multidisciplinary approach. These findings provide epidemiological data to inform prenatal diagnosis and prognostication strategies in resource-limited settings.