Anaesthesia in arthrogryposis multiplex congenita: Case report

Abstract

The anaesthetic management of an 11-year-old male with arthrogryposis multiplex congenita (AMC) with myopathic features is described. The child presented with an obstructing renal calculus and a pyelolithotomy via an anterior transverse subcostal extraperitoneal incision was performed. In view of the associated hypoplastic musculature, severe scoliosis and expected increased sensitivity to various sedatives and inhalational anaesthetic agents, low-dose ketamine (10-15 μg·kg-1 min-1) was administered by continuous infusion, with a satisfactory result. © 1987 Canadian Anesthesiologists.