Domino Hepatectomy and Implantation With Innovative Outflow Reconstruction for an Adult Patient With Maple Syrup...

Maple syrup urine disease is characterized by deficient metabolism of branched chain amino acids leading to neurological and developmental delays. Because livers with maple syrup urine disease are histologically normal except for a single enzyme deficiency, which is also present in other organs, liver transplant provides adequate enzymes for metabolism of branched chain amino acids, and the explanted liver can be used as a domino graft for another person needing a liver transplant. We report on the case of a rare individual who survived into adulthood with ante-natal diagnosis and strict dietary management. His recent clinical course was complicated by the development of diabetes mellitus leading to recurrent instances of diabetic ketoacidosis or maple syrup urine disease crisis necessitating liver transplant. Our technical modifications enabled a safe and quick domino hepatectomy by minimizing anesthesia time and the anhepatic phase and timely preparation of the domino graft for an uneventful implantation.