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DYSTROPHIA MYOTONICA—DEATH UNDER “PENTOTHAL” ANÆSTHESIA
Author(s) -
PACHOMOV N.,
CAUGHEY J. E.
Publication year - 1958
Publication title -
australasian annals of medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.596
H-Index - 70
eISSN - 1445-5994
pISSN - 0571-9283
DOI - 10.1111/imj.1958.7.2.159
Subject(s) - medicine , ptosis , anesthesia , atrophy , general anaesthesia , autopsy , surgery , myotonia , myotonic dystrophy
Summary A girl of twelve had suffered locking of the knees, lumbar lordosis and ptosis since birth. Her physical development had been generally retarded. Her maternal grandfather and her mother had both suffered “locking” of the knees and myopathy. Various muscles showed atrophy and pseudohypertrophy, and there was myotonia of the tongue. Early cataracts were present in both eyes. Examination revealed no abnormality of the heart. Operation for the ptosis, under “Pentothal”, ethyl chloride and open ether anæsthesia, was uneventful but respiration ceased suddenly 30 minutes after operation, and she died 25 minutes later. At autopsy the heart showed adipose and collagenous replacement of myocardial fibres. Similar changes were present in skeletal muscles. Cardiac involvement has been reported in 62 of 98 cases of dystvophia myotonica and in a further 18 of 35 previously reported by the author. It is usually manifested by electrocardiographic changes, and physical signs are rare. Such patients have been reported to be particularly prone to respiratory troubles during “Pentothal” anæsthesia. Enzymes concerned with the detoxication of “Pentothal” may be deficient in myotonica dystrophia .