ANAESTHESIA FOR A PATIENT SUFFERING FROM FAMILIAL DYSAUTONOMIA (RILEY-DAY SYNDROME)

A case of familial dysautonomia (Riley-Day syndrome) is presented. Details of the anaesthetic technique employed whilst a gastrostomy and hernia repair were performed are reported. Moderately heavy sedation and propranolol together with atropine as the premedication employed may have been responsible for the uneventful course of anaesthesia. The pathophysiology of the condition is presented in terms of the implications for the anaesthetist who undertakes the management of such a case.