Congenital myotonic dystrophy in children - A review of ten years' experience

Abstract

A review of the anaesthetic management of children with congenital myotonic dystrophy anaesthetised at the Royal Children's Hospital over the past ten years is presented. Seven children underwent a total of fourteen anaesthetics. Anaesthetic considerations must include the degree of muscle weakness and hypotonia altering muscle relaxant requirements, aspiration risk due to palatopharyngeal dysfunction, and cardiomyopathy. Succinylcholine caused muscle contracture in a patient without clinical myotonia. This drug should be avoided. Although a low threshold to institute postoperative respiratory support must exist when treating neonates and infants, the older children did not clinically exhibit increased sensitivity to respiratory depressant drugs.