EXPERIENCES WITH GENERAL ANESTHESIA IN PATIENTS WITH FAMILIAL DYSAUTONOMIA

The syndrome familial dysautonomia includes a combination of congenital abnormalities and clinical manifestations that make a patient a poor candidate for general anesthesia. Striking features are indifference to pain and diminished lacrimation, which predispose to corneal ulceration, and poor vasomotor control, which predisposes to hypertension during emotional crises and to hypotension during anesthesia. Three cases are described in detail. Among eight patients in whom this syndrome was observed, severe hypotension during anesthesia occurred six times and cardiac arrest twice. When this condition is suspected, a complete preoperative evaluation is needed. Patients who have this condition do not tolerate thiopental sodium or tribromoethanol as anesthetic agents. If local anesthesia after premedication with chlorpromazine is not suitable, the alternative is premedication with chlorpromazine and belladonna drug followed by a volatile general anesthetic.