McArdle's disease and anaesthesia: Case reports. Review of potential problems and association with malignant hyperthermia

Abstract

McArdle's disease does not seem to cause severe perioperative problems in routine anaesthetic care. However, measures for preventing muscle ischaemia and rhabdomyolysis should be kept in mind, as well as the potential for these patients to develop postoperative fatigue, myoglobinuria and renal failure. Although no clinical association with malignant hyperthermia has been established, many of these patients can have a positive in vitro contracture test, and simple prophylactic measures, as with malignant hyperthermia, may be recommended if otherwise not contraindicated.

McArdle Disease and Pregnancy

“…Despite being the most common of the glycogen storage diseases, it is an uncommon disease, being transmitted as a recessive autosomic autosomal trait 4 . There are incidence reports from 1:100000 inhabitants in Texas to 1:650000 inhabitants in Norway 7,14 . Its features are essentially exercise intolerance, with premature fatigue, myalgia and muscle cramps.…”

Enfermedad de McArdle o glucogenosis tipo v: ¿influye en el manejo anestésico?

“…La asociación entre ambas es débil y no se han descrito casos en la literatura. Se recomiendan medidas profilác-ticas, evitándose los agentes desencadenantes de la HM 1,2,6 . No se recomienda la profilaxis con dantrolene de forma rutinaria, pero sí el acceso rápido a dicha medicación en caso de sospecha de HM 2,5 y preparación de la máquina de anestesia para pacientes susceptibles de HM.…”

Myophosphorylase deficiency (McArdle disease) in a patient with normal pregnancy and normal pregnancy outcome

“…1 Perez et al and Maté-Muň oz et al report that adults with McArdle disease can re-condition their fitness with pre-exercise sucrose administration 9 and that training is possible under controlled conditions. 10 Bollig et al 3 had also reported that patients with McArdle disease can have significant complications associated with surgery and anaesthesia such as hypoglycaemia, rhabdomyolysis, myoglobinuria, acute kidney injury and malignant hyperthermia. The literature favours regional anaesthesia with concerns being raised regarding muscle relaxation during general anaesthesia.…”

“…The literature favours regional anaesthesia with concerns being raised regarding muscle relaxation during general anaesthesia. 3 Our patient developed myoglobinuria following swimming in early pregnancy which settled. Labour occurred and as expected the myometrium not being skeletal muscle performed without problems and she reached full dilatation but developed a persistent occipito posterior presentation and was not deliverable vaginally.…”

“…4 The subsequent deficiency of substrate for the muscle cells during exercise can result in rhabdomyolysis, myoglobinuria and acute renal failure with a significant number of patients having a raised serum CK even at rest. 3 The disorder is often noted to be associated with exercise intolerance in childhood with the development of muscle pain and stiffness. The first report of pregnancy and labour in a woman with McArdle disease was in 1973 and the authors of that paper were able to discuss the options of management with Dr McArdle.…”