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Liver transplantation is now the accepted standard of care for
the management of irreversible end-stage liver disease in children.
Since the first successful liver transplant by Starzl in 1966, continuous improvements
in patient selection, surgical technique and postoperative care,
as well as advances in organ preservation and immunosuppression
management have resulted in improved graft and patient survival.
Earlier referral for transplantation, and advances in the clinical
management of rejection, infection, and biliary and vascular complications
have also contributed to improved outcomes.
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Each year, approximately 600 children have received a liver transplant
in the United States.1 Of these, 15% have
been living donor liver transplants. In 2007, approximately 400 children
were waiting for a liver transplant on the United Network for Organ
Sharing (UNOS) national waiting list. The risk of death while waiting
for a transplant is related to age. Young children less than 1 year
old have the highest death rate of all waiting list candidates,
adult or pediatric, nearly 6 times the death rate for older children
and adults.
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Indications for liver transplantation are listed in Table
130-1. Liver transplantation is indicated for life threatening
failure of liver function due to either chronic liver conditions
or acute hepatic necrosis. End-stage liver disease because of cholestatic
liver disease represents the reason that the majority of children
are referred for liver transplantation. Of these, biliary atresia
is the most common single diagnosis, accounting for approximately
45% of all children undergoing liver transplant.2 Other
common indications include metabolic liver diseases, acute liver
failure, hepatic malignancy, and chronic hepatitis.
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Children with advanced liver disease may develop cirrhosis and
present clinically with decreased synthetic liver function, evidenced
by jaundice, hypoalbuminemia, coagulopathy, or poor growth. Coagulopathy
may be exacerbated by associated vitamin K deficiency because of
poor intestinal absorption of fat-soluble vitamins. Hepatic cirrhosis
is often associated with portal hypertension, presenting clinically with
hepatosplenomegaly, ascites, increased risk for variceal hemorrhage,
and encephalopathy. Acute hepatic necrosis typically presents within
8 weeks of a gastrointestinal illness, associated with jaundice,
fever, and significant elevation ...