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Iranian Journal of Pediatrics
Tehran University of Medical Sciences Press
ISSN: 1018-4406
EISSN: 2008-2150
Vol. 20, No. 1, 2010, pp. 118-122
Bioline Code: pe10017
Full paper language: English
Document type: Research Article
Document available free of charge

Iranian Journal of Pediatrics, Vol. 20, No. 1, 2010, pp. 118-122

 en Tecto-Cerebellar Dysraphia Manifesting as Occipital Meningocoele Associated with Congenital Melanocytic Nevi and Pectus Excavatum
Agrawal, Amit; Joharapurkar, Sudhakar Ratanlal & Khan, Ata-Ullah

Abstract

Background: Only few reported cases of tectocerebellar dysraphia with occipital encephalocele have been reported in the literature.
Case Presentation: Three month baby boy, the first child of healthy, consanguineous parents presented with a small swelling over the occipital region since birth. The child also used to have apneic spells without cyanosis and spontaneous recovery. CT scan showed absence of the cerebellar vermis, absence of tectum and the 4th ventricle communicating with the occipital menigocoele sac and an occipital bone defect. The excision of the encephalocoele sac was performed, however the child continued to have apneic spells and did not do well.
Conclusion: In our child irregular respiration probably was the manifestation of the tecto-cerebellar dysraphia syndrome complex and associated shunt malfunction followed by seizures decompensated the physiology of the child leading to fatal outcome.

Keywords
Dandy-Walker Syndrome; Meningocele; Nevus, Melanocytic; Pectus Excavatum

 
© © 2010 by Center of Excellence for Pediatrics, Children’s Medical Center, Tehran University of Medical Sciences,All rights reserved.
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