Anaesthetic Problems in Myotonic Dystrophy

Abstract

A previously undiagnosed case of myotonic dystrophy presenting with apnoea of 2.5 h duration following thiopentone is described. A review of the anaesthetic outcome from 49 operations in 17 patients with myotonic dystrophy in the Aberdeen area is presented. The type of operation and intra- and postoperative problems are analysed. The results reveal a 52% complication rate in previously diagnosed cases and a 35% complication rate in undiagnosed cases. In the series, 29% of the anaesthetics were administered to symptomatic patients before formal diagnosis. To avoid potential hazards it behoves the anaesthetist to remain alert to the possibility of the undiagnosed disease. The symptomatology and associated findings of the 17 patients at initial diagnosis are presented. The literature has been reviewed and anaesthetic implications noted.

Desflurane anaesthesia in myotonic dystrophy

“…[1–3] Myotonic crisis can be precipitated intraoperatively by succinylcholine, etomidate, methohexital and neostigmine and physical factors like hypothermia, electrocautery and surgical stimulation. A generalized and/or localized contracture (myotonia) should be prevented by prewarming the operation theater (OT) and IV fluids, avoidance of succinylcholine and shivering and gentle handling of muscles during surgery.…”

“…Numerous case reports of postanaesthetic myotonic crisis, prolonged apnea, delayed recovery, arrhythmias, need for prolonged ventilatory support, postoperative aspiration, hypoventilation, respiratory failure and even deaths in these patients underscore the seriousness of the disorder. [1–3] Depolarizing muscle relaxants are hazardous while the response to non-depolarizing muscle relaxants is reported to be normal. [4] There has been reluctance to use volatile anaesthetics for fear of malignant hyperthermia (MH) and their cardiorespiratory depressant effects.…”

Continuous infusion of propofol in dystrophia myotonica

“…7 It is the most severe of a group of hereditary muscle diseases, collectively called the myotonic syndrome, charaeterised by myotonia, weakness and atrophy of certain groups of muscles; the three best described being dystrophia myotonica, myotonica congenita and paramyotonica eongenita. The clinical features of the disease have been well reviewed by Prnzanski, s and more recently by Aldridge.…”

“…The clinical features of the disease have been well reviewed by Prnzanski, s and more recently by Aldridge. 7 Patients with dystrophia myotonica pose major anaesthetic management problems for not only is it a true multi-system disease, 2 but there is an abnormal response to many anaesthetic drugs and there are frequently postoperative cardio-respiratory complications.…”

Étude descriptive de la mortalité dans la dystrophie myotonique au Saguenay-Lac-Saint-Jean /

“…Kaufman (1960) affirme aussi que la rachianesthésie ne met pas à l'abri des complications post-opératoires. Aldridge (1985) rapporte que plusieurs des difficultés post-opératoires résultent d'un diagnostic non établi avant l'intervention ou d'une sous-estimation des risques d'insuffisance respiratoire postopératoire. Zuck (1987) rapporte un cas de décès post-opératoire causé par un oedème pulmonaire et relie cette complication à un défaut éventuel de la membrane cellulaire.…”