Encephaloceles: A Study of 110 Patients Operated on at AIIMS

Background: Encephalocele is a rare clinical entity encountered in neurosurgical practice.

Material and Methods: Over an 8-year period (2002–2009), 110 cases of encephalocele were treated at the Department of Neurosurgery, AIIMS, New Delhi.

Observations: There were 110 cases of encephalocele operated on during the study period. The vast majority of patients were younger than 2 years of age. Ten patients were neonates less than 30 days old. Seventy-three patients (66%) had occipital encephalocele, 30 patients (27%) had anterior encephalocele, and 7 had parietal encephalocele. Eleven patients (10%) had giant encephaloceles, of which 9 were occipital and 2 were anterior encephaloceles. Patients with hydrocephalus were subjected to CSF diversion in the form of ventriculoperitoneal shunt. All patients except one were operated on. Anterior encephalocele patients, in addition to repair of encephaloceles, also underwent corrective osteotomies for hypertelorism, when the hypertelorism was significant. Overall results were good. There were five postoperative deaths (4.5%). Two were neonates with giant occipital encephaloceles, who developed hypothermia during surgery and did not recover. One patient with occipital encephalocele developed wound dehiscence, followed by fulminant meningitis, leading to death. The remaining two mortalities were in infants, who underwent surgery for anterior encephalocele and developed postoperative aspiration pneumonitis leading to death.

Conclusion: Encephalocele is a rare clinical condition and accounts for less than 0.5% of our patients undergoing surgery. Occipital encephaloceles accounted for 66% cases, followed by anterior encephaloceles (30%). Overall, results of surgery were good with a mortality rate of 4.5%.