RESUMEN
Dysfunction of oxidative phosphorylation and the mitochondrial respiratory chain leads to a heterogeneous group of pathogenic mitochondrial variations. The TRMU gene codes for transfer RNA 5- methylaminomethyl-2-thiouridylate methyltransferase and is essential for posttranscriptional modification of the mitochondrial transfer RNA, and alterations in the TRMU gene can lead to infantile liver failure at approximately 6 months of age. Orthotopic liver transplant is a curative option. We present a case of a patient with TRMU alteration who underwent liver transplant at 11 months of age to treat infantile end- stage liver disease. The patient had liver failure due to long-standing allograft rejection and required another liver transplant at age 24 years, and here we discuss the perioperative care of this patient. Coordination of the care team to prevent rhabdomyolysis or alternative negative catabolic effects was the cornerstone of management in addition to evaluation of unusual electrocardiographic findings in the immediate postoperative period. Although the patient's postoperative course was complicated by repair of a bile leak, liver retransplant successfully restored the patient's preoperative quality of life.