A patient with familial dysautonomia may have a number of serious complications associated with anesthesia and surgery. These can be minimized by careful preoperative planning and perioperative management.


Recommendations for managing a patient with familial dysautonomia:

(1) Make sure that the patient is well-hydrated prior to surgery.

(2) Protect the airway to minimize the risk of aspiration.

(3) Avoid or minimize the use of volatile anesthetics.

(4) Use regional anesthesia if possible.

(5) Use minimally invasive surgery if possible.

(6) Monitor the patient closely for hypertension and cardiac lability.

(7) Protect the eyes from drying out.

(8) Consider use of medications (benzodiazepines, clonidine) to reduce vomiting.

(9) Consider use of medications (flurocortisone, midodrine, etc) to minimize cardiovascular lability.

(10) Be prepared for transient gastrointestinal disorders following surgery.


To read more or access our algorithms and calculators, please log in or register.