Milne Andrew, Mon Wint Yu, Down James, Obichere Austin, Ackland Gareth L
From the University College London Hospitals, London, United Kingdom.
A A Case Rep. 2015 May 1;4(9):111-3. doi: 10.1213/XAA.0000000000000141.
Familial dysautonomia (Riley-Day syndrome) is a rare multisystem disorder associated with an excess risk of perioperative morbidity and mortality. Because life expectancy is limited, few reports consider the perioperative management of familial dysautonomia in adults with advanced disease and end-organ dysfunction. Here, we report on the management of an adult patient with familial dysautonomia, highlighting recent developments in perioperative technology and pharmacology of special relevance to this challenging population.
