Laurence Moon Bardet Biedl syndrome and anaesthesia

Abstract

Laurence Moon Bardet Biedl is a congenital syndrome which is rarely seen and accompanied by multipl anomalies including skeleton and viscera.This case report describes the presentation of a child with Bardet-Biedl syndrome. Bardet-Biedl syndrome is an autosomal recessive condition that includes retinal dystrophy, dystrophic extremities (commonly polydactyly), obesity, hypo-genitalism, and renal disease. Cognitive deficit has also been considered part of the syndrome. The most common feature of Bardet-Biedl syndrome is retinal dystrophy. The renal disease often goes undetected until specific radiological testing is done after diagnosis of Bardet-Biedl syndrome. This is significant in that early death often occurs in this condition because of the renal disease. A twelve year old male patient with Laurence Moon Bardet Biedl syndrome was reported and literature was reviewed.