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British Journal of Anaesthesia, 1985, Vol. 57, No. 11 1119-1130
© 1985 The Board of Management and Trustees of the British Journal of Anaesthesia

case-report

ANAESTHETIC PROBLEMS IN MYOTONIC DYSTROPHY

A Case Report and Review of the Aberdeen Experience Comprising 48 General Anaesthetics in a Further 16 Patients

L. M. ALDRIDGE, F.F.A.R.C.S., D.C.H.

Department of Anaesthetics, Aberdeen Royal Infirmary Foresterhill, Aberdeen AB9 2ZD.

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 17patientswith 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.


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