Dyskeratosis congenita induced cirrhosis for liver transplantation-perioperative management

Abstract

Dyskeratosis congenita (DC) is an inherited disorder with progressive multisystem involvement. End stage liver disease (ESLD) in patients with DC is rare. We describe the perioperative management of a patient with DC induced ESLD and severe hepatopulmonary syndrome for living donor liver transplantation.

Keywords

• Cirrhosis
• dyskeratosis congenita
• liver transplant
• transplant