Canadian Journal of Anesthesia, Vol 12, 558-568, Copyright © 1965 by Canadian Anesthesiologists' Society

Familial Dysautonomia as an Anaesthetic Hazard

¹ Chief Anaesthetist, The Children's Hospital, Winnipeg, Manitoba; Assistant Professor of Surgery (Anaesthesia), University of Manitoba, Winnipeg, Manitoba

Familial dysautonomia is described as presented in the literature and as it appeared in a young girl who required six anaesthetics for major surgical procedures. Intravenous thiopental was used for induction and invariably caused a fairly sharp drop in blood pressure. A technique of light anaesthesia with nitrous oxide and oxygen was quite satisfactory for maintenance. Pain sensation is often poor and stronger analgesics are usually unnecessary. Methoxyflurane, halothane, and ether were used and their effects on pulse, blood pressure, EEG, EKG, and blood gases measured. All these agents caused striking depression of blood pressure and pulse rate. Methoxyflurane maintained more stable conditions than did halothane.

Post-operative nausea, intestinal stasis, hyperpyrexia, unstable blood pressure, convulsions, and respiratory infections are common. Chlorpromazine proved most useful in controlling nausea, temperature, and blood pressure.

The nature of the disease remains unknown. No gross or microscopic pathology has been described. The evidence suggests an enzymatic defect affecting the function and site of action of acetylcholine. This manifests itself throughout the whole nervous system but may act particularly in the reticular activating substance and thalamic nuclei.

Note:

Presented at the Annual Meeting of the Canadian Anaesthetists' Society, Charlottetown, P.E.I., June 21–25, 1965