Abstract

Clinical experience of 50 patients with the CHARGE association is reviewed and problems with management of children born with multiple system involvement is highlighted. It was found that the outlook for survival was poor if more than one of the following three features was present: cyanotic cardiac lesions, bilateral posterior choanal atresia, or tracheo-oesophageal fistula. Mortality was largely due not to the structural heart defects or choanal abnormalities, but reflected underlying pharyngeal and laryngeal incoordination, which resulted in aspiration of secretions. Outcome is likely to be improved if collaboration between specialist surgical teams allows necessary procedures to be performed using the minimum number of anaesthetics. Examination of both the short and long term management of these children has stressed the importance of a multidisciplinary approach to their care.