Tag Thomas J. Ebert

Familial Dysautonomia (Riley-Day Syndrome)

Risk Autosomal recessive transmission Complete penetrance, marked variability in expression Predominantly affects Ashkenazi Jewish population (incidence 1:10,000–20,000; carrier frequency 1:27-32) Perioperative Risks Intraop: Primarily cardiovascular with hemodynamic variability Postop: Primarily cyclic vomiting and pulmonary complications Worry About Paroxysmal dysautonomic crisis…