The anaesthetic management of a patient with familial dysautonomia

Abstract

Familial dysautonomia is a rare inherited disease of the nervous system, involving mainly peripheral sensory and sympathetic nerves. The autonomic instability is of greatest importance for the anaesthetist although the sensory defect is the most dramatic symptom of the disease. A case report of a 15-year-old girl undergoing two operations for severe scoliosis is presented with a discussion of the anaesthetic problems encountered in this disease.