Case Report: Perioperative Management of a Patient with Familial Dysautonomia

Division

North Florida

Hospital

Ocala Regional Medical Center

Document Type

Case Report

Publication Date

9-8-2021

Keywords

familial dysautonomia

Disciplines

Anesthesiology | Congenital, Hereditary, and Neonatal Diseases and Abnormalities | Nervous System Diseases

Abstract

Familial dysautonomia is a rare autosomal recessive neurodegenerative disease aff ecting cells of the autonomic nervous system. Patients with this disease are insensitive to pain but their autonomic nervous system is still activated with noxious stimuli. This report details a case of a patient with familial dysautonomia who underwent right ankle open reduction and internal fi xation for a bimalleolar right ankle fracture. The patients preoperative and intraoperative course were uneventful but shortly after handoff to the intensive care unit, the patient experienced an autonomic crisis. Management of these patients is complex, requiring maintenance of physiologic homeostasis as well as preventing hemodynamic instability caused by noxious stimuli. Any deviations from baseline may cause an autonomic crisis, as happened in our patient. Herein, we detail the perioperative management of a patient with familial dysautonomia in further detail.

Publisher or Conference

Journal of Intensive Care Medicine

Link to Full Text

Share

COinS