Our program is designed to manage all aspects of liver and hepatobiliary diseases in children. We specialize in multidisciplinary management of portal hypertension, liver tumors, disorders of the bile ducts, and liver-based metabolic diseases in children, and work collaboratively with Children’s National Medical Center to further provide interdisciplinary management or surgery for hepatic tumors and any related surgery for short bowel syndrome.
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For those children that do not require a transplant, we are able to utilize the extensive expertise of our joint program to offer surgery to relieve portal hypertension (shunts), correct biliary atresia (Kasai portoenterostomy), treat liver tumors such as hepatoblastoma with combination treatment of chemotherapy and resection, or treat liver disease related to TPN. Some patients with short bowel syndrome may require other surgical interventions as well. In addition, some children may require transplantation for metabolic diseases, while others may stabilize with optimal metabolic support.
Indications for Liver Transplantation
Liver diseases can be categorized as either cholestatic, hepatocellular, metabolic or liver tumors. While the most common indication for liver transplantation in children is chronic liver failure secondary to cholestatic liver disease, other indications are listed below.
Cholestatic: Includes biliary atresia, Alagille syndrome, nonsyndromic biliary paucity and progressive familial intrahepatic cholestasis. Some infants who present with giant cell hepatitis or neonatal hepatitis of unknown etiology develop persistent cholestasis and rapid progression to cirrhosis and portal hypertension, making them candidates for liver transplantation in the first 2-3 years of life. We have an experienced team of surgeons and hepatologists to manage these problems.
Hepatocellular: Includes acute and subacute hepatic failure, autoimmune liver disease types I and II, chronic hepatitis B or C, polycystic liver disease and hepatic tumors. Medical therapies exist and many patients will respond and avoid or delay the need for transplantation. Our pediatric hepatologists can direct the use of standard medications or sometimes offer newer agents only available through clinical trials. Those who require transplantation may then continue to be cared for by the same team managing them before the transplant.
Metabolic: Includes alpha-1-antitrypsin deficiency, tyrosinemia type I, Wilson’s disease, neonatal hemochromatosis, glycogen storage disease type I, cystic fibrosis and inborn errors of metabolism, such as Crigler-Najjar Syndrome Type I, ornithine transcarbamylase (OTC) deficiency, maple syrup liver disease (MSUD), and familial hypercholesterolemia.
The metabolic genetics team at CNMC is among the most experienced in the world with one of the largest such patient populations. Working in partnership with Georgetown’s liver transplant specialists, we are able to offer optimal integrated care, and cure or improve significantly many children with these disabling diseases.
Tumors: Both hepatoblastoma and hepatocellular carcinoma can occur in children. These tumors may be cured by partial liver resection. However, they may also respond to chemotherapy, undergoing significant size reduction. Those tumors too large to resect or associated with cirrhosis require transplantation. We have an experienced team to deal with the management of these tumors, including nursing support for chemotherapy, infusion services, nutritional counseling, antibiotics and continuous follow-up. Our oncologists (at CNMC) may also be able to offer participation in a variety of experimental protocols for management of these tumors.
The bottom line is that by using the latest diagnostic techniques, advanced surgical techniques and powerful new chemotherapeutic regimens, an excellent cure rate can be achieved.
