Clinical Presentations and Surgical Outcome In Patients With Occipital Encephalocele

Aurangzeb Kalhoro, Kashif Ahmed, Abdul Sattar M Hashim

Abstract

Objective

To document the clinical presentations and outcomes of the surgical management in patients with occipital encephalocele.

Study design

Descriptive case series.

Place & Duration of study

Neurospinal and Cancer Care Institute Karachi, from June 2017 and October 2021.

Methods

Patients with occipital encephalocele were included in this study. The demographic data and clinical presentations were recorded. The cognition function and developmental delays were assessed by the pediatric neurologist. Investigations performed included ultrasound, CT scan and MRI brain for the assessment of bony defect and the content of the encephalocele and brain. All patients were operated. At follow-up any associated complications and neurological development were recorded. The assessment of the outcomes included wound healing, head circumference size, symptoms of meningitis if any, and the presence of neurological deficit. Descriptive statistics were used to present the data.

Results

A total of 21 patients with occipital encephalocele were managed during the study period. There were 12 male and 9 female patients. The age of the patients was between 4-days to 15-months with the mean age of 2.4-months. Enlarged head circumference with associated hydrocephalus was observed in four patients at presentation. One patient developed cerebrospinal fluid (CSF) leak. In one patient with Dandy Walker syndrome Y-connector was placed during surgery. The follow up continued for 18-months. A patient who developed CSF leak which did not stop spontaneously in postoperative period, the ventriculoperitoneal shunt was placed. No patient developed neurological deficits after surgery.

Conclusion

Encephalocele is an infrequently encountered anomaly. Males were more often involved. Surgical procedure was tailored according to the content of the anomaly and associated conditions. CSF leak was observed in one patient who later developed hydrocephalus.

Key words

Occipital encephalocele, Neural tube defect, CSF leak, Hydrocephalus, Neurological delay.