Neurosurgical Interventions for Occipital Encephalocele

The aim of this study is to find the outcome of repair and resection of the occipital encephalocele.

Case series.

The clinical data of fifty consecutive occipital encephalocele patients were retrieved from medical records including operative notes, postoperative follow-up visits, and postsurgical complications were noted for analysis from November 2009 to November 2013 at the Department of Neurosurgery, Jinnah Postgraduate Medical Centre, Karachi, Pakistan. All patients were assessed by computed tomography scan, magnetic resonance imaging brain, and ultrasound when needed. Physician's assessment, physical examination, and his/her questions to the family at follow-up were used as a tool to determine if there was a developmental delay rather than quantitative analysis like hydrocephalus questionnaires. Patients who developed complications and delayed milestone were regarded as no improvement and those who did not develop complications and achieved appropriate milestone were regarded as improved at 18 months follow-up.

Of 50 patients, 17 were males and 33 were females. The average age at presentation was 2.4 months. 16 (32%) patients had increased head circumference and hydrocephalus, 2 (4%) had associated Dandy-Walker cyst, 3 (6%) developed developmental delays, and 8 (15%) had a seizure disorder. None of our patients had neurological deficits. The size of the sac ranged from 2 cm × 3 cm to 27 cm × 15 cm. 9 (18%) patients were admitted with the complication of sac rupture and 2 (4%) patients sac ruptured after admission. Only one patient (2%) had a cerebrospinal fluid leak postoperatively that was repaired primarily without patch graft or dura seal while 4 (8%) developed hydrocephalus after repair of the sac which was treated with placement of ventriculoperitoneal shunt. One (2%) patient did not recover from anesthesia and expired.

Encephalocele is commonly seen in the practice of neurosurgery in the world as well as in Pakistan. Modern neuroimaging, neurosurgical techniques, and neonatal neurological intensive care have greatly improved morbidity and mortality in the care of encephalocele.