Domino Liver Transplantation: Anesthetic Considerations for a Patient With Maple Syrup Urine Disease and Type I Diabetes Mellitus

TL;DR

This paper discusses the challenges and management of liver transplantation in a patient with two rare metabolic disorders.

Contribution

The first reported case of domino liver transplantation in an adult with both maple syrup urine disease and type I diabetes.

Findings

The patient required intensive metabolic preconditioning due to complex disease interactions.

Successful surgery was followed by excellent glycemic control on a normal diet.

Mild to moderate cellular graft rejection occurred four months post-transplant.

Abstract

In this report, we describe the case of a patient with concomitant maple syrup urine disease (MSUD) and type I diabetes mellitus (T1DM) who underwent domino liver transplantation (DLT) , and the associated perioperative management. To the best of our knowledge, a DLT in an adult with both MSUD and T1DM has not been previously reported in the literature. Intensive care admission with multidisciplinary oversight is necessary for metabolic preconditioning prior to surgery. The complex interplay between these two disease processes presented with grossly elevated baseline insulin requirements and refractory intraoperative hyperglycemia. Following the successful procedure, the patient maintained excellent glycemic control on a normal diet. Four months post transplant, the patient presented with mild to moderate cellular graft rejection.

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