Giant Encephalocele: A Study of 14 Patients

Abstract

Background: Giant encephalocele is a rare condition and few published reports are available in the English literature. It is a challenge to neurosurgeons, even today. This series consists of 14 patients with giant encephaloceles treated at our institute. Material and Observation: Over a period of 8 years, from 2002 to 2009, 110 patients with encephaloceles were managed at our institute. Amongst them, 14 were children with giant encephaloceles. All patients had CT/MRI or both prior to surgery, and all were operated upon. Four patients were neonates, under 1 month of age, and 9/14 patients (64%) were under 3 months. The youngest child was a newborn baby aged 2 days. Except for 1 with an anterior encephalocele, the rest were patients with occipital encephaloceles. A CT scan was performed on 5 and an MRI on 1 patient. Both CT and MRI scans were performed on the other 8 patients. MRI/CT showed hydrocephalus in 10/14 patients. Of these, 7 required ventriculoperitoneal (VP) shunt, and the remaining 3 with mild to moderate hydrocephalus did not. Of the 7 patients who underwent VP shunt, 5 had a shunt during the encephalocele repair and 2 had a postoperative shunt for increasing hydrocephalus. Results: Other associated anomalies recorded were acquired Chiari malformation in 3 patients, secondary craniostenosis with microcephaly in 5, and syringomyelia in 1 patient. All the patients underwent repair of encephalocele and 4 had suturectomy of coronal suture for the secondary craniostenosis. There were 2 postoperative deaths due to hypothermia. Among the 12 surviving patients, 9 had a good outcome and 3 had poor mental development. The present study shows overall good outcomes in 9/14 (66%) patients.

Inherent Challenges in the Management of Neonatal Giant Occipital Encephalocele. A Case Series

“…Anterior encephalocele are more common in male but posterior encephalocele are commonly seen in females. On review of literature, we found a single case series of giant encephalocele [1]. Hydrocephalus with giant occipital encephalocele is very rare.…”

“…Hydrocephalus with giant occipital encephalocele is very rare. In Mahapatra series only one neonate with occipital giant encephalocele had hydrocephalus out of three cases [1]. Magnetic resonance imaging of Brain is the investigation of choice in these cases.…”

“…Neonatal giant encephaloceles are rare with incidence of 3.6% with occipital predominance [1]. Giant encephaloceles means encephaloceles that are larger than the skull.…”

“…Giant encephaloceles means encephaloceles that are larger than the skull. The defect is usually present in the occipital bone and sac may contain meninges, brain or both [1]. Majority of occipital encephaloceles are seen in female.…”

“…Majority of occipital encephaloceles are seen in female. Hydrocephalus is seen in half of the cases of giant occipital encephaloceles [1]. They are associated with other congenital abnormalities like microgyria, polygyria, craniostenosis, microcephaly and micrognathia [1,2].…”