Perioperative care of a child with Ullrich congenital muscular dystrophy during posterior spinal fusion

Abstract

Ullrich congenital muscular dystrophy (UCMD) is one of a group of disorders known as congenital muscular dystrophies. Severe hypotonia and early diaphragmatic involvement may lead to respiratory failure early in the disease process. We present the case of a nine-year-old with UCMD who required operative intervention for progressive scoliosis. In these patients, anaesthetic issues relate to difficulties with endotracheal intubation, as well as the potential for postoperative respiratory failure, given early diaphragmatic involvement. As with other types of muscular dystrophy, succinylcholine is absolutely contraindicated, while a prolonged effect may be seen following routine doses of nondepolarising neuromuscular blocking agents. Additional perioperative concerns relate to the surgical procedure primarily, including tailoring the intraoperative anaesthetic to facilitate neurophysiological monitoring, as well as the use of techniques to limit intraoperative blood loss. The perioperative management of patients with UCMD is discussed and options for intraoperative anaesthetic care are reviewed.