Familial dysautonomia (FD), a rare genetic disorder, is characterized by autonomic instability, pulmonary infections, oesophageal dysmotility, spinal abnormalities and episodic ‘dysautonomic crisis’ characterized by rash, vomiting, sweating and hypertension. Frequent anaesthetic complications have been reported.

We performed a comprehensive literature search of perioperative management of FD using an OVID-based search strategy. Identified reports were reviewed to identify perioperative complications as well as anaesthetic techniques and perioperative management strategies developed to minimize or prevent these complications.

Eighteen case reports or series of perioperative management of FD were identified in the literature for a total of 179 patients undergoing 290 anaesthetics. Intraoperative cardiovascular lability, including cardiac arrests and postoperative pulmonary complications were commonly reported. Preoperative hydration, minimizing the use of volatile anaesthetic agents, postoperative ventilation, use of regional anaesthesia and minimally invasive surgical techniques reduced the incidence of these complications.

While patients with FD are reported to have a relatively high rate of various perioperative complications, a full understanding of its pathophysiology can be used to develop a perioperative management strategy to anticipate and prevent many of these complications.