Cardiac Arrest during Major Spinal Scoliosis Surgery in a Patient with Duchenne's Muscular Dystrophy Undergoing Intravenous Anaesthesia

Abstract

A 14-year-old Chinese boy with Duchenne's Muscular Dystrophy underwent a posterior spinal fusion and Texas Scottish Rite Hospital instrumentation for deteriorating scoliosis of the thoracolumbar spine. He was anaesthetized using a target-controlled infusion (TCI) of propofol and an alfentanil infusion supplemented by nitrous oxide. Seven hours into the procedure he suddenly developed a broad complex bradycardia followed by asysole from which he was promptly resuscitated. The operation was completed and he suffered no neurological deficit. The possible cause of this event in view of the risk of cardiac and metabolic complications in Duchenne's Muscular Dystrophy is discussed and the anaesthetic technique utilized in this case is described. CASE REPORT A Chinese boy with Duchenne's Muscular Dystrophy (DMD) first presented at the age of nine years with proximal muscle weakness, abduction contracture of both hips and equinus deformity of the right ankle. A bilateral ilio-tibial tract release was carried out at this time under general anaesthesia and was uneventful apart from a short-lived, unexplained postoperative pyrexia which resolved spontaneously and was not further investigated. He was wheelchair-bound within two years and by the age of 13 years had developed a thoracolumbar scoliosis. This was rapidly progressive with a deterioration of the spinal curve from 66 0 to 80 0 over the year prior to surgery. Preoperative assessment revealed obvious clinical 'ER.C.A.

Neuromuscular and mitochondrial disorders: what is relevant to the anaesthesiologist?

“…cardiomyopathy, upregulation of AChR). Two case reports of acute heart failure during spinal surgery in DMD patients during propofol-opioid anaesthesia [23,24] demonstrate that using total intravenous anaesthesia (TIVA) in DMD still may cause major problems. A recent report of an infant who developed postoperative rhabdomyolysis and death due to sepsis after the last of nine uneventful anaesthetics with halogenated agents illustrates the complexity of the topic [25 ].…”

Anesthesia and Duchenne or Becker muscular dystrophy: review of 117 anesthetic exposures

“…Fourteen patients (25.5%) had intraoperative complications while receiving volatile anesthetics (no succinylcholine) , and an additional 12 patients (21.8%) had early postoperative complications after receiving volatile anesthetics (no succinylcholine) . Complications during the use of nontriggering anesthetics were reported in only four patients (7.3%), and all were attributed to cardiac arrest or cardiomyopathy .…”

Duchenne muscular dystrophy: Which is the best anesthetic agent?

“…However, it is not clear from the review whether these children had pre‐existing cardiomyopathy, nor what concentrations of volatile anesthetics were used. Furthermore, two case reports describe intraoperative cardiac arrest In DMD children during spinal surgery under propofol‐sufentanyl anesthesia without succinylcholine or volatile anesthetics (4,5).…”