Anaesthetic management of an obstetrical patient with arthrogryposis multiplex congenita

Abstract

Arthrogryposis Multiplex Congenita (AMC) describes a rare multifactorial syndrome caused by a variety of neurogenic and myopathic disorders first diagnosed at birth and often progressing to a state of significant disability. It includes abnormalities of the cardiovascular, respiratory, nervous and genitourinary systems. The syndrome is seen primarily in the paediatric population, and orthopedic procedures are common. There remains much confusion regarding diagnosis, aetiology, genetic probabilities, natural history and management.1-2 Occasionally an adult with this sydrome presents for an anaesthetic. The following is a case report of a patient with the manifestations of this syndrome presenting for a primary Caesarean section. © 1988 Canadian Anesthesiologists.