Ellis-Van Crevald syndrome.

Indian Pediatr

Published: August 2004

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Ellis-Van Creveld (EVC) syndrome is an autosomal recessive disorder. Around 150 cases are described in published literature and in Bangladesh, it is even rare.  The patient usually comes with short stature, dental deformity, and cardiac deformity.

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Talon cusp is a rare dental anomaly often associated with systemic conditions such as Rubenstein-Taybi Syndrome, Ellis-van Crevald Syndrome and Incontentia pigmenti achromians. It has not been previously reported as occurring in Alagille Syndrome. Early diagnosis of talon cusps is crucial for the correct management and avoidance of complications.

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We report the occurrence of a pyriform sinus cyst in a patient with Ellis-van Crevald syndrome. The patient presented with congenital stridor. The cyst was drained but recurred.

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