Abstract
INTRODUCTION: Mitochondrial DNA depletion syndrome (MDS) is a kind of autosomal recessive genetic disorder associated with a reduction in mitochondrial DNA (mtDNA) copy number caused by mutations in nuclear genes during nucleotide synthesis, which affects the energy production of tissues and organs. Changes in hemodynamics during liver transplantation may lead to high energy-demanding organs and tissues being vulnerable. This report described the intraoperative management during liver transplantation in a child with MDS. Ultimately, the child was discharged smoothly without any complications. PRESENTATION OF THE CASE: A five-year-old boy was diagnosed with mitochondrial depletion syndrome preoperatively and scheduled for living donor liver transplantation. The incidence of postreperfusion syndrome (PRS) could not be avoided for 30 min after opening, despite our best efforts to aggressively prevent it before opening. While ensuring hemodynamic stability, we actively prevented and adopted high-energy-demand organ protection strategies to reduce the incidence of postoperative complications. Finally, the child was discharged 28 days after the operation, and no other complications were found. DISCUSSION: Liver transplantation can be performed for liver failure in this disease to improve the quality of life and prolong the life of patients. As this child has mitochondrial DNA depletion syndrome, the disruption of cellular energy generation caused by mitochondrial malfunction puts high-energy-demanding organs and tissues at risk during surgery. It motivates us to pay closer attention to the prevention and treatment of PRS in anesthetic management to minimize damage to the child's organs and tissues with high energy demands. CONCLUSIONS: This report describes the intraoperative management during liver transplantation in a child with mitochondrial depletion syndrome. To increase the safety of perioperative anesthesia and reduce mortality in patients with mitochondrial disease, for such patients, maintaining an acid-base balance and a stable internal environment is essential. We should also pay attention to protecting body temperature, using vasoactive drugs beforehand to lessen the incidence of PRS, and protecting high-energy-demanding organs afterward.