Congenital Hydrocephalus with Occipital Meningoencephalocele: A Case Emphasizing the Importance of Early Diagnosis and Surgical Management

Abstract

Congenital Hydrocephalus with Occipital Meningoencephalocele: A Case Emphasizing the Importance of Early Diagnosis and Surgical Management.
Background: Occipital meningoencephalocele is a significant neural tube defect, commonly seen with hydrocephalus, which is a contributory factor for worsening neurological and developmental outcomes. Early identification and surgical intervention are essential in limiting morbidity and mortality. This paper describes a case of a neonate with hydrocephalus and occipital meningoencephalocele, focusing on the importance of early diagnosis and intervention, cerebrospinal fluid diversion, and surgical repair. Case Presentation: A male infant was born by cesarean section with a giant occipital cystic mass noted at birth. Clinical exam showed hydrocephalus and newborn hyperbilirubinemia. Lab tests were notable for mild anemia, hypoalbuminemia, leukopenia, and hyperbilirubinemia.Imaging was consistent with an occipital defect and herniation of neural tissue consistent with meningoencephalocele. The infant underwent the placement of a ventriculoperitoneal (VP) shunt on DOL 5 for hydrocephalus and then stabilized in the NICU. The infant recovered from the procedure with stable neurological and systemic status, and gradual improvement observed. Conclusion: This case highlights the importance of early diagnosis and surgical management of hydrocephalus with occipital meningoencephalocele. Prompt intervention with VP shunt placement and planned excision of the encephalocele sac can improve survival and developmental outcomes. Comprehensive antenatal care, early neuroimaging, and multidisciplinary management remain essential in optimizing prognosis for affected neonates.
Keywords: Occipital Meningoencephalocele, Hydrocephalus, Neural Tube Defect, Ventriculoperitoneal Shunt, Early Surgical Management