Successful Anesthetic Management of Orthotopic Liver Transplant in a Patient with Deoxyguanosine Kinase Deficiency: A Case Report.

Deoxyguanosine kinase deficiency is a rare autosomal recessive mitochondrial DNA depletion syndrome characterized by severe progressive hepatic failure and neurologic involvement in infancy or early childhood. The disease often progresses to end-stage liver disease, necessitating liver transplant in selected patients. We present the anesthetic management of a 17-year-old female patient with end-stage liver disease due to deoxyguanosine kinase deficiency who was scheduled for liver transplant. Liver transplant is currently the only definitive treatment option for hepatic failure associated with deoxyguanosine kinase deficiency, although perioperative morbidity and mortality remain high. Anesthesia management of patients with deoxyguanosine kinase deficiency who undergo liver transplant requires aggressive blood glucose and lactic acidosis monitoring. Comprehensive preoperative assessment, with careful consideration of systemic manifestations and underlying mitochondrial dysfunction, is essential. Successful anesthetic mana-gement requires a multidisciplinary team approach, meticulous perioperative planning, and watchful intraoperative monitoring to optimize outcomes and improve prognosis in this high-risk population.