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INTRODUCTION

FOCUS POINTS

  1. Most common etiology of end-stage liver disease (ESLD) in children is cirrhosis secondary to biliary atresia.

  2. ESLD involves nearly every organ system.

  3. The primary pulmonary manifestation of ESLD is arterial hypoxemia.

  4. The most common cause of fulminant hepatic failure (FHF) is acute viral hepatitis.

  5. Indications for the highest priority status (1A) for liver transplantation include FHF and hepatic arterial thrombosis or primary graft nonfunction post-transplant.

  6. There are three surgical phases in liver transplantation: preanhepatic, anhepatic, and neohepatic.

  7. Postreperfusion syndrome (PRS) during the neohepatic phase is associated with increased perioperative morbidity and mortality.

  8. Postoperative complications following liver transplantation include bleeding, vascular occlusion events, rejection, and primary nonfunction.

CASE

A 9-year-old boy with history of biliary atresia post Kasai procedure presents with hyperbilirubinemia, transaminitis, and altered mental status. Acute hepatic failure with hepatic encephalopathy is diagnosed and he is placed on the highest priority status (1A) for liver transplantation.

HISTORY

Dr. Thomas Starzl performed the first pediatric liver transplant (LT) in a 3-year-old girl with biliary atresia in 1963. The recipient expired in the operating room secondary to intraoperative bleeding. In 1967, the first successful pediatric LT was performed.1 Prior to the introduction of cyclosporine, 2-year survival following LT was less than 30% with rudimentary immunosuppression regimens consisting of corticosteroids and azathioprine.2–4 Following the introduction of cyclosporine in 1979 survival rates increased. Current 1-year survival rates exceed 80%.5 Initially few centers performed pediatric LTs, but this has increased significantly over the past three decades. Currently, there are more than 100 centers approved to perform pediatric LT in the United States; however, only 16 centers perform more than 10 pediatric LT annually.6

Less than 10% of all LTs are performed in children. According to the United States Organ Procurement and Transplantation Network (OPTN) more than 145,000 LTs were performed in the United States between 1998 and 2016, and approximately 1600 were performed in children.5 Current survival rates for deceased donor LT (DDLT) are greater than 90% in some centers (Figure 25-1A), with higher survival rates for live donor LT.5 Inadequate cadaveric organ supply has resulted in unacceptably high pretransplant waitlist mortality (Figure 25-1B). Innovative techniques to optimize hepatic graft supply for pediatric LT include reduced liver, split liver, and live donor LT (Figure 25-1C). However, split and reduced LTs have lower graft survival compared to whole organ cadaveric and live donor transplants.7

Figure 25-1

A. Patient survival among pediatric liver transplant recipients: deceased donor. B. Pre-transplant mortality rates among pediatric patients waitlisted for a liver transplant, by age. C. Percentage of split liver transplants compared to total transplants performed among pediatric and liver transplant recipients. (Reproduced with permission, from Kasiske BL, Israni AK, Snyder JJ, et al. OPTN/SRTR 2017 Annual Data Report: Liver: Am J Trans. 2019; 19(S2): ...

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