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Tectocerebellar Dysraphia and Occipital Encephalocele: An Unusual Association with Abdominal Situs Inversus and Congenital Heart Disease

Sriram Krishnamurthy2, 3 Contact Information, Seema Kapoor2, Vipul Sharma2 and Anjali Prakash1

(1)  Department of Radiodiagnosis, Maulana Azad Medical College and associated Lok Nayak Hospital, New Delhi, India
(2)  Department of Pediatrics, Maulana Azad Medical College and associated Lok Nayak Hospital, New Delhi, 110002, India
(3)  Department of Pediatrics, Maulana Azad Medical College, New Delhi, 110002, India

Received: 7 January 2007  Accepted: 9 March 2008  Published online: 22 September 2008

Abstract  Tectocerebellar dysraphia is a rare constellation of malformations comprising of occipital encephalocele, aplasia of the cerebellar vermis and deformity of the tectum. We describe a 7 month old infant who presented with tectocerebellar dysraphia associated with double outlet right ventricle, pulmonary stenosis and abdominal situs inversus. This association has not been reported in the literature, to the best of our knowledge.

Key words  Tectocerebellar dysraphia - Occipital encephalocele - Double outlet right ventricle - Abdominal situs inversus


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