Giant Occipital Encephalocele - Challenges in Management

Giant occipital encephalocele (GOE) is a term used when the size of the OE is greater than or equal to the size of the head. It has been limited to case reports, with only sporadic exclusive series. This is a series of GOE managed at our center over time with emphasis on practical problems faced in management.

This was a retrospective observational study. The patients were evaluated for the age of presentation, sex, and head size. Any associated neural tube defect was also looked for. Imaging was used for associated brain anomalies and to plan the surgical procedure. The requirement of ventriculoperitoneal (VP) shunt was also assessed.

During the study period of 7 years, 11 patients of GOE were admitted. Apart from one, all other patients were <1 year of age. Nine patients underwent surgical intervention, which included excision and repair of swelling with or without VP shunt placement. The content of the sac was only cerebrospinal fluid (CSF) in six patients and CSF and gliotic brain tissue in remaining patients. The attendants of two patients did not give consent for surgery and left against medical advice.

GOE is an uncommon entity with limited information about management. Careful evaluation, proper imaging of patient, and care during intraoperative and postoperative periods with emphasis of factors determining the prognosis may provide satisfactory results.