Liver Transplantation

Published on 22/03/2015 by admin

Filed under Pediatrics

Last modified 22/03/2015

Print this page

This article have been viewed 944 times

Chapter 360 Liver Transplantation

Development of liver transplantation requires continuous refinements in the management of end organ disease, surgical technique, and perioperative care. The development of better immunosuppressive management (cyclosporine in 1978 and tacrolimus in 1989) and enhancements in our understanding of the relationship between recipient and host immune systems have resulted in better long-term survival. Paralleling this, advancements in the abdominal organ procurement techniques and organ preservation solutions have made possible the procurement and transportation of organs over long distances, with the creation of a national system for matching these donor organs with waiting recipients (the Organ Procurement and Transplantation Network [OPTN] and the United Network for Organ Sharing[ UNOS]).

With improved outcomes, the indications for liver replacement in acute and chronic liver disease have increased dramatically. This increase has resulted in a crisis of organ availability: In 2007 there were >12,000 patients awaiting liver transplantation in the USA, of whom 3% were children.

Indications

The diseases for which liver transplantation is indicated can be categorized into the following groups:

• Obstructive biliary tract disease: biliary atresia, sclerosing cholangitis, traumatic or post-surgical injury

• Metabolic disorders: α₁-Antitrypsin deficiency, tyrosinemia type I, glycogen storage disease type IV, Wilson disease, neonatal hemochromatosis, Crigler-Najar type I, familial hypercholesterolemia, primary oxalosis, organic academia, urea cycle defects

• Acute hepatitis: fulminant hepatic failure, viral, toxin, or drug induced

• Chronic hepatitis with cirrhosis: hepatitis B or C, autoimmune

• Intrahepatic cholestasis: idiopathic neonatal hepatitis, Alagille syndrome, familial intrahepatic cholestasis (Byler disease)

• Miscellaneous: cryptogenic cirrhosis, congenital hepatic fibrosis, Caroli disease, cystic fibrosis, polycystic liver disease, cirrhosis induced by total parenteral nutrition (TPN)

• Primary liver tumors: benign tumors (hamartomas, hemangioendothelioma), unresectable hepatoblastoma, and hepatocellular carcinoma

Biliary atresia is the most common indication for liver transplantation in children, followed by metabolic and inborn disorders, autoimmune and familial cholestatic disorders, and acute hepatic necrosis. Children with biliary artesia (or any other obstructive biliary disorder) who do not achieve successful drainage require liver transplantation within the first year of life, yet some patients with successful drainage later develop cirrhosis with portal hypertension (variceal bleeding and ascites). Some inborn errors of metabolism can cause structural damage and portal hypertension; other inborn errors manifest principally by their hepatic enzyme deficiency, with serious complications on the brain or liver. Some metabolic disorders place patients at risk for decompensation throughout their entire lives, and others (e.g., Crigler-Najar) manifest principally after adolescence. Acute hepatic necrosis (acute liver failure) often lacks a precise etiology and has required the most intense concentration of multimodal management and support and organ graft options yet devised. Primary hepatic malignancies in children are usually of an advanced stage, yet adjuvant chemotherapy, total hepatectomy and transplantation have provided cure and long-term survival for the majority of patients thus treated. Some diseases do not produce life-threatening complications, yet their impact on growth, development, and quality of life can be so devastating that liver transplantation is a valid therapy and cure. The distribution of liver grafts does follow guidelines based on severity of liver failure as reflected in the Pediatric End-Stage Liver Disease (PELD) scoring system developed by UNOS, which takes into calculation the measurable values of bilirubin, creatinine, and international normalization ratio (INR).

Contraindications to liver transplantation include uncontrolled infection of extrahepatic origin, uncontrolled extrahepatic malignancies, and severely disabling and uncorrectable disease in other organ systems, principally the heart and lungs. Though combined liver and heart or lung transplantation has been performed in adults and children, such cases require special consideration. Also, disabling neurologic disease can preclude liver transplantation if the outcome will not allow the child to develop some measure of independence and quality of life.

Buy Membership for Pediatrics Category to continue reading. Learn more here

Related

Nelson Textbook of Pediatrics Expert Consult