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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 2008-2150
Vol. 19, No. 2, 2009, pp. 180-184
Bioline Code: pe09029
Full paper language: English
Document type: Case Report
Document available free of charge

Iranian Journal of Pediatrics, Vol. 19, No. 2, 2009, pp. 180-184

 en Hemihydranencephaly; a Case Report
Balpande, Damodar Nanaji; Pathak, Chandra Shekhar; Agrawal, Amit & Singh, Brij Raj

Abstract

Background: Hemihydranencephaly is a rare disorder of the brain characterized by complete or almost complete unilateral absence of cerebral cortex with preservation of meninges, basal ganglia, pons, medulla, cerebellum, and falx.
Case Presentation: Thirteen year-old male child presented with left sided upper and lower limb weakness with facial asymmetry since the age of six months. His magnetic resonance imaging (MRI) scans demonstrated a nearly complete absence of the right cerebral hemisphere including basal ganglion, which was replaced by cerebrospinal fluid with a small residual rim of the occipital cortex. The imaging features were suggestive of right-sided hemihydranencephaly.
Conclusion: Patients with hemihydranencephaly provide an experiment of nature with potential implications for normal cognitive development and illustrate how much there is still to be learned about human development.

Keywords
Hemihydranencephaly, Brain, Congenital anomalies, Carotid artery, Aplasia

 
© © 2009 by Center of Excellence for Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences,All rights reserved.
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