Familial dysautonomia is a rare disorder seen almost exclusively in Jewish children. On the basis of past experience, there has been great reluctance to subject these patients to general anesthesia. The principal autonomic disorder that makes anesthesia dangerous appears to be a lack of endogenous catecholamine when the stress of anesthesia or surgery or both calls for this substance. In the case described here, carefully titrated exogenous epinephrine was a decisive factor in the successful management of anesthesia for major thoracic surgery.
Bartels J, Mazzia VDB. Familial Dysautonomia. JAMA. 1970;212(2):318–319. doi:10.1001/jama.1970.03170150072018
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