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We present a case of a 2 staged liver transplant on a 12-month-old 8kg male with newly diagnosed mitochondrial depletion syndrome causing acute liver failure, refractory lactic acidosis, and cardiopulmonary collapse requiring ECMO. The patient’s past medical history at the time of presentation included history of prematurity at 32 weeks, developmental delay, and a brother with an undiagnosed pancreatic disorder. The patient was brought in by parents to the emergency department with a cough, minimal PO intake for 5 days, and increased fatigue who was found to be URI positive. Initial labs were concerning for anemia, elevated bilirubin, elevated AST/ALT, hyponatremia, coagulopathy, and metabolic acidosis secondary to an elevated lactate of 11.5 mmol/L. An ultrasound of the liver showed hepatocellular inflammation with small perihepatic and pericholecystic fluid. He was admitted to the floor for observation and continued testing. 4 days after admission he had an acute decompensation due to fulminant liver failure and persistent severe lactic acidosis requiring intubation, resuscitation, and VA ECMO support. The leading diagnosis was that he had an inborn error of metabolism causing liver necrosis. Whole exome sequencing was performed to better delineate the cause of his persistent lactic acidosis and liver failure. Due to the dire nature of the patient's medical course, a multidisciplinary meeting was held and he was listed status 1A for a liver transplant due to a necrotic liver. The operative plan was for the transplant surgery team to perform a hepatectomy with return to the ICU post operatively to allow improvement in his physiology and for his lactic acidosis to correct itself and then receive a whole liver transplant the following day. The patient remained intubated and sedated and was taken to the operating room with the OR nursing staff, PICU team, ECMO staff, CRRT nurse, anesthesia team, and surgical team. He was then terminally extubated 2 months after his initial presentation.


Anesthesiology | Pediatrics


Presented at the Society for Pediatric Anesthesia/American Academy of Pediatrics Section on Anesthesiology and Pain Medicine (SPA-AAP) Pediatric Anesthesiology 2024 Conference; Anaheim, CA; April 12-14, 2024.

Anesthetic and Multidisciplinary Management of a 2-staged Liver Transplant​