Anaesthetic Management of a Patient Having Ellis-Van Creveld Syndrome

Dr. Rutvi Shah, Dr. Shilpi Yadav, Dr. Archana Har

Abstract: Ellis-Van Creveld Syndrome is rare Aotossomal recessive condition characterized by short limb dwarfism, polydactyly, abnormal development of finger nails and congenital cardiac defects in 50% of the patients. The orofacial manifestation includes multiple gingivolabial fraenula, dental anomalies, hypodontia and malocclusion. The syndrome can be caused by mutation in EVC gene or EVC2 gene present in chromosome 4p16.

Keywords: Ellis-Van-Crevald syndrome, polydactyly, Chondroectodermal dysplasia