Open AccessLarge vertex meningoencephalocele with schizencephaly: An interesting case with neurosurgical challenge
Author(s) -
Sudhansu Sekhar Mishra,
Satya Bhusan Senapati,
Srikanta Das,
Rama Chandra Deo
Publication year - 2014
Publication title -
journal of pediatric neurosciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.247
H-Index - 18
eISSN - 1998-3948
pISSN - 1817-1745
DOI - 10.4103/1817-1745.139315
Subject(s) - schizencephaly , medicine , encephalocele , magnetic resonance imaging , convulsion , pediatrics , congenital disorder , presentation (obstetrics) , radiology , surgery , epilepsy , psychiatry
An encephalocele is a developmental disorder of neural tube closure, where as schizencephaly is a developmental disorder of neuronal migration. There are only few case reports suggesting association of schizencephaly with meningoencephalocele. We report a case of 6-year-old child who was brought by her parent with complaints of progressive swelling over her vertex since birth, left sided hemiparesis and intermittent generalized tonic clonic convulsion for last 3 years. Clinical examination, computed tomography (CT) and magnetic resonance imaging (MRI) of brain revealed a large calvarial defect with meningoencephalocele and associated schizencephaly. Initially, ventriculoperitoneal (VP) shunt was carried out, followed by successful repairing of the defect. This case is interesting for late presentation, management difficulties and associated anomalies "schizencephaly", which is a rare developmental disorder.