Contemporary Perioperative Management of Adult Familial Dysautonomia (Riley-Day Syndrome). (1st May 2015)

• Record Type: Journal Article
• Authors: Milne, Andrew; Mon, Wint Yu; Down, James; Obichere, Austin; Ackland, Gareth L.
• Is Part Of: A & A case reports. Volume 4:Number 9(2015)
• Publication Date: 2015-05-01
• Subjects: Anesthesiology -- Periodicals; Anesthesia -- Periodicals; Anesthesiology; Analgesia; Anesthesiology; Periodicals; 617.9605
• DOI: 10.1213/XAA.0000000000000141
• Languages: English
• ISSNs: 2325-7237

Abstract

Abstract : 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.