Anaesthesia and Progressive Muscular Dystrophy

Abstract

The presentation and features of Duchenne's progressive muscular dystrophy (Duchenne's PMD) are described and the increased risks associated with anaesthesia are considered. Hazards associated with induction of anaesthesia and immediate postoperative recovery have been stressed in recent case reports, and these are summarized. Features of a hyperpyrexia-like response including cardiac arrest, increased serum creatine phosphokinase concentration, myoglobinuria and metabolic acidosis following suxamethonium or halothane, or both, have been described in patients with Duchenne's PMD. Subsequent in vitro muscle tests have suggested that it is possible that a malignant hyperpyrexia response to general anaesthesia may occur. Six children known to have Duchenne's PMD who developed delayed respiratory insufficiency following anaesthesia and required controlled pulmonary ventilation are reported. In five of the children, cardiac arrest occurred despite apparently adequate respiratory support. Suxamethonium was common to the anaesthetic received by all six patients. In one of these patients subsequent anaesthetics, without suxamethonium, were uneventful and delayed muscle weakness did not occur.

Anaesthetic management during labour of a manifesting carrier of Duchenne muscular dystrophy

“…1,3,4 Rhabdomyolysis, raised creatine kinase, myoglobinuria, metabolic acidosis and acute renal failure are reported, suggesting a malignant hyperpyrexia-like syndrome. 1,[3][4][5][6] The triggers for the above are depolarising neuromuscular blocking drugs and inhaled volatile agents. In planning an anaesthetic for a male patient with DMD, depolarising neuromuscular blocking drugs and inhaled volatile agents should be avoided.…”

“…Regional anaesthesia is recommended where possible. 1 Females are usually asymptomatic carriers of the abnormal gene. However, 2.5% of female carriers have symptoms of the disease.…”

“…General anaesthesia in affected individuals is associated with life-threatening peri-operative complications including cardiac arrhythmias, cardiac arrest and a malignant hyperpyrexia-like syndrome. 1 It is recommended that suxamethonium and inhaled volatile agents should be avoided in this population. Females who carry the abnormal X chromosome are usually asymptomatic but, rarely, can be manifesting carriers of the disease with symptoms and signs of the disease process.…”

The anesthetic management of a patient with Emery-Dreifuss muscular dystrophy for orthopedic surgery

“…It is unknown whether there is delayed gastric emptying in patients with EDMD, although in patients with Duchenne muscular dystrophy (DMD) delayed gastric emptying has been reported. 11 In view of the potential airway problems in EDMD, the method of airway management will be a question of judgement in each case, taking into account any risk of regurgitation from the history, and the difficulty in tracheal intubation. Pharmacological prophylaxis with a pro-kinetic agent would seem prudent with the premedication in any case, regardless of the ultimate method of airway management.…”

Verborgene intrakardiale Leitungsstörungen und deren spontaner Verlauf bei Patienten mit progressiver Muskeldystrophie

“…Hochgradige AVBlockierungen sind bei PMD-Patienten im Gegenteil zu myotoner und Emery-Dreifuss-Muskeldystrophie (4, 7, 8, 10) jedoch nur in Einzelfälle publiziert worden (17,20,26). Bei einigen Patienten mit PMD wurde ein Herzstillstand während einer Vollnarkose beobachtet (22). Über invasive elektrophysiologische Untersuchungen wurde bislang lediglich bei 10 Patienten mit facioscapulohumeraler Muskeldystrophie (Landouzy-Déjerine) berichtet (24).…”