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Case Studies | Neuroscience | India | Volume 11 Issue 10, October 2022

Giant Occipital Encephalocele: A Study of 5 Patients

Rohit Kamlesh Yadav ^[2] | Tathagata Datta | Shahid Iftekhar Sadique

Abstract: Background: When the occipital encephalocele is greater than or equal to the size of the head, it is referred to as a giant occipital encephalocele (GOE). Giant occipital encephaloceles are rare lesions; because of their enormous size they pose a surgical challenge. This series consists of 5 patients with giant encephaloceles treated at our institute. Material and methods: This was a retrospective observational study. The patients' head size, sex, and age at presentation were all assessed. We also checked for any related neural tube defects. Imaging was utilized to both design the surgical treatment and check for accompanying brain anomalies. It was also assessed whether a ventriculoperitoneal (VP) shunt was required. Results: During the study period of 1 years, 5 patients of GOE were admitted in our department. Most patients were <1 year of age. Among them, two were male and the remaining three were female. Of 5 patients who underwent surgery, excision and repair of GOE were performed as primary surgery in 4 patients. Of those 5 patients, one patients underwent VP shunt placement after excision due to raised intracranial pressure (ICP). VP shunt was placed as initial surgery in one patients, which was followed by excision and repair after about 5 weeks. Those patients who underwent VP shunt placement as primary surgery showed a decrease in the size of the encephalocele. The content of the sac was only CSF in 4 patients and CSF and gliotic brain tissue in remaining 1 patient. Associated cranial malformations included microcephaly, Chiari malformation, corpus callosal agenesis, and hydrocephalus. Hydrocephalus was symmetrical in all of them. One patient expired in the follow?up. The exact cause of mortality could not be ascertained. The probable reason may be the associated other congenital diseases. Conclusion: GOE is an unusual entity with limited information about its management. Careful evaluation, accurate imaging of the patient, and care throughout the intraoperative and postoperative periods, with an emphasis on factors influencing the prognosis, may lead to favourable result.

Keywords: neural tube defect, encephalocele, hydrocephalus, ventriculoperitoneal shunt

Edition: Volume 11 Issue 10, October 2022,

Pages: 520 - 523