Title: Giant Occipital Encephalocele – A Report of Two Rare Cases
Author: Dr Faran Ahmad, Dr Mohammad Kaif, Dr Tarun Pandey, Dr Deepak Kumar Singh
DOI: https://dx.doi.org/10.18535/jmscr/v5i2.149
Abstract
Encephalocele is a common congenital malformation in which central nervous system structure in communication with cerebrospinal fluid pathway herniate through the skull. The incidence of occipital encephalocele is 1/10,000 where as those encephalocele larger than the head size (Giant encephalocele) is very rare. These giant encephalocele can cause obstructed labour necessitating caesarean section. Various teratogens are associated with occipital encephalocele, namely sodium arsenate, clofibrate and vitamin A. Large number of CNS anomalies are associated with large encephalocele including fusion of thalamus, dysgenesis of corpus callosum, failure of aqueduct formation, formation of inter hemispheric tissue, split superior sagittal sinus, absence of falx cerebri and dysgenesis of tentorium. In utero diagnosis of encephalocele can be ascertained by foetal USG a maternal blood sample for AFP. Encephalocele can be treated by surgical removal of the sac and water tight closure of dura with closure of the non dysplastic skin but induction and maintenance of general anaesthesia is a challenge.
Keywords: Giant encephalocele, cranial congenital malformation, Paediatric neurosurgery, Hydrocephalus.
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