Anaesthesia for a Patient Suffering From Familial Dysautonomia (Riley-Day Syndrome)

Perioperative management of familial dysautonomia

“…Eight of the reports present the anaesthetic experience of only a single patient, but six of these eight do report multiple episodes of anaesthetic care in the same patient [9][10][11][12][13][14][15]. Intraoperative complications were primarily cardiovascular with haemodynamic variability including episodes of hypotension, hypertension, bradycardia and dysrhythmias [6,7,[9][10][11][12][13][16][17][18][19], complete heart block on one occasion [9] and three occurrences of cardiac arrest that were successfully treated [7,13].…”

“…Postoperative complications included primarily cyclic vomiting and pulmonary complications such as pneumonia, atelectasis, excessive secretions and respiratory arrest requiring reintubation on two occasions [6,7,9,12,16,[19][20][21]. Haemodynamic lability was reported in the postoperative period [6,7,9,12,16,[19][20][21], but less frequently than in the intraoperative period [6,7,[9][10][11][12][13][16][17][18][19]. One patient experienced a postoperative seizure [9] and one patient intraoperative confusion [15].…”

“…Early management strategies consisted primarily of premedication followed by various general anaesthetic techniques that consisted of nitrous oxide alone, nitrous oxide supplemented with various anaesthetic agents or anaesthetic agents alone [7,[9][10][11]16]. In early reports, now obsolete anaesthetics such as tribromoethanol and the volatile agents ether, methoxyflurane and cyclopropane were used [7,9,16].…”

The role of monitoring the depth of anesthesia in a case of hereditary sensory and autonomic neuropathy (Riley Day syndrome)

“…Anesthesia for familial dysautonomia/hereditary sensory and autonomic neuropathy (HSAN) (Riley‐Day syndrome) has come a long way from the case report by Inkster in 1971 (2) to the recent extensive article by Ngai et al. (1).…”