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Description

We discuss the case of a 13-month-old previously healthy male who presented with acute liver failure (ALF) of unknown etiology. He progressed to fulminant cardiopulmonary failure requiring mechanical ventilation and high dose inotropes with persistent lactate >20. He was cannulated to VA ECMO and tandem continuous renal replacement therapy (CRRT) with single pass albumin dialysis (SPAD) and plasma exchange (PLEX) therapies. He required high dose inotropes on ECMO and had persistently elevated lactate despite maximal medical therapy. Limited imaging studies suggested cirrhotic liver and possible intestinal pneumatosis. Due to evidence of fulminant ALF and potential for a reversible process, he was listed for liver transplant status 1A. Given his refractory acidosis, a multidisciplinary team planned for laparotomy to exclude intestinal ischemia and then proceed with hepatectomy and portocaval shunt. An organ offer had been accepted with a backup recipient. Anhepatic time would continue to be supported with CRRT, SPAD and PLEX twice daily. Multiple multidisciplinary huddles occurred prior to the OR for optimal communication and planning. To prepare for the first hepatectomy on ECMO in our institution, we followed our program’s bleeding protocol for our congenital diaphragmatic hernia repairs on ECMO. Intraoperatively, he had minimal surgical bleeding outside of expected losses. Preparations were made for variable venous return pressures due to IVC clamping, but they remained manageable. The liver was noted to be necrotic without evidence of pneumatosis of the bowel and hepatectomy was completed successfully. The portal flow was managed via portocaval shunt. His acidosis resolved with improved coagulopathy and inotrope requirements in the following 24 hours. He proceeded back to the OR the following day for a liver transplant. The liver was successfully re-perfused. His biliary reconstruction was left incomplete due to bleeding. The bile duct was cannulated and connected to an external drainage bag. Patient perfusion pressure was adequate throughout the case. Postoperatively, he quickly weaned from inotropes and liver support therapies. Graft function was excellent. ECMO flows were weaned aggressively, and he was decannulated on post liver transplant day #1. He was taken back to the operating room for biliary reconstruction and second stage closure on post operative day #5. The process to complete a novel and high-risk operation is worth sharing as ALF is becoming a more common indication to consider extracorporeal support. We encourage closed-loop and frequent preoperative discussions with anesthesiology, surgery, intensive care and ECMO practitioners to ensure adequate planning.

Publication Date

2-2024

Disciplines

Critical Care | Pediatrics | Surgery

When and Where Presented

Presented at the 40th Annual ECMO and the Advanced Therapies for Respiratory Failure Symposium; Keystone, Colorado; February 25-28, 2024.

Successful Hepatectomy, Anhepatic State, and Liver Transplant on ECMO

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