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Tecto-Cerebellar Dysraphia Manifesting as Occipital Meningocoele Associated with Congenital Melanocytic Nevi and Pectus Excavatum

Author(s): Amit Agrawal | Sudhakar Ratanlal Joharapurkar | Ata-Ullah Khan

Journal: Iranian Journal of Pediatrics
ISSN 2008-2142

Volume: 20;
Issue: 1;
Start page: 118;
Date: 2010;
Original page

Keywords: Dandy-Walker Syndrome | Meningocele | Nevus | Melanocytic | Pectus Excavatum

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.
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