The most widely accepted surgical treatment for BA is Kasai portoenterostomy (KPE) which aims to restore biliary drainage from the bile duct to the gastrointestinal tract. It is expected that once the cholestasis is relieved, the liver damage is alleviated and patients can achieve long-term native liver survival (NLS).
Full Answer
What are the treatment options for biliary atresia?
Biliary atresia cannot be treated with medication. A surgery called Kasai procedure (also known as a or hepatoportoenterostomy) creates a path of bile flow from the liver into the intestine. The surgeon removes the damaged ducts outside of the liver (called extrahepatic ducts). They find smaller ducts that are still open and draining bile.
What is the long-term prognosis of biliary atresia?
· World J Surg. 1978 Sep;2(5):581–585. [PubMed] [Google Scholar] Suruga K, Kono S, Miyano T, Kitahara T, Soul-Chin C. Treatment of biliary atresia: microsurgery for hepatic portoenterostomy. Surgery.
What is biliary atresia?
Objective: The purpose of this study was to delineate the long-term prognosis of biliary atresia (BA) in Taiwan. Study design: From 1976 to 2000, 185 children were diagnosed with BA, 22 …
What is operative cholangiogram for biliary atresia?
· Treatment of biliary atresia. ... Mowat AP, Psacharopoulos HT, Williams R. Extrahepatic biliary atresia versus neonatal hepatitis. Review of 137 prospectively investigated …
Is biliary atresia fatal?
Prior to the early 1960’s, biliary atresia was considered a uniformly fatal disease. Today — with the Kasai hepatoportoenterostomy and improvements in liver transplantation — patients with biliary atresia are reaching adulthood.
How long does it take to live with biliary atresia?
Overall survival with a native liver (not transplanted) ranges from 30-55 percent at 5 years of age; and 30-40 percent at 10 years of age. It is thought that approximately 80 percent of patients with biliary atresia will require liver transplantation by the age of 20. But these statistics may change as we learn more about biliary atresia and modify our treatment approaches.
When was biliary atresia considered fatal?
From the 1960s to the present, numerous advances in the surgical approach to biliary atresia have been incorporated into routine care. Prior to the early 1960’s , biliary atresia was considered a uniformly fatal disease.
How long does biliary atresia last?
In general, the long-term outlook for patients with biliary atresia is excellent. Most biliary atresia patients can expect to live into adulthood with either their native liver or a transplanted liver.
What is the Kasai procedure?
The Kasai procedure is the preferred treatment for biliary atresia at The Children's Hospital of Philadelphia and has been performed here for more than 30 years. The operation connects the bile draining from the liver directly to the gastrointestinal (GI) tract.
What is a cholangiogram?
A cholangiogram is an X-ray imaging study performed by a surgeon in the operating room to determine if there is blockage of bile flow in the ducts leaving the liver. If this test suggests there is an obstruction, the surgeon will proceed with exploration to determine if the child has biliary atresia.
Why do children with liver disease need more calories than normal?
Vitamin deficiencies may also occur. Children with liver disease require more calories than a normal child because they do not absorb the same amount of calories and nutrition. Your physician may recommend that a pediatric nutritionist make recommendations regarding your child's diet. Nutritional guidelines may include the following:
Can a donor liver be replaced with a healthy liver?
This procedure involves removing the entire diseased liver and replacing it with a healthy donor liver. The donor liver can either come from a deceased donor or from a small portion of a living donor’s liver. After several months, this small portion will then regenerate into a fully functioning liver.
Is biliary atresia fatal?
Although biliary atresia is a serious and potentially fatal condition, surgical advances have made the long-term outlook for this disease good. For instance, the Kasai procedure can reestablish full bile flow in about 25 percent of children and partial bile flow in about 50 percent of children.
What is the function of bile?
Bile is a fluid produced by the liver that helps with digestion. Normally, bile secretes to the intestines through bile ducts. Biliary atresia prevents this by blocking the bile ducts.
What is the condition where the liver drains bile?
Biliary atresia is a rare condition that prevents the liver from draining bile to the intestines. This causes bile to build up in the liver, which can produce scarring and eventually liver failure.
Key Facts
- Bile is a fluid produced by the liver that helps with digestion. Normally, bile secretes to the intestines through bile ducts. Biliary atresia prevents this by blocking the bile ducts.
- Biliary atresia only affects infants. It usually appears about two to eight weeks after birth.
- Without treatment, biliary atresia can be fatal. While there are no medications available for this condition, there are several surgical treatments.
Causes
- The causes of biliary atresia are still not well understood. Researchers currently believe it may be caused by several factors, such as undeveloped bile ducts, a viral or bacterial infection, an autoimmune response from the body, or environmental factors, such as exposure to toxins. The following are known to increase the risk of developing this condition: 1. Premature Birth: Infants …
Symptoms
- Biliary atresia usually does not develop until two to eight weeks after birth. When it does, the following are some of its most common symptoms: 1. Clay-colored stools 2. Dark urine 3. Jaundice (yellowing of skin and eyes) 4. Swollen abdomen 5. Weight loss
Diagnosis
- Because biliary atresia can resemble several other conditions, doctors use several methods to diagnose it. They usually begin by evaluating liver function through blood tests. These measure the following: 1. Bilirubin: Bilirubin is a waste product that is processed by the liver. If bilirubin levels are high, it may be a sign that something is wrong with the liver. 2. Blood Clotting: If blood …
Treatment
- Biliary atresia cannot be treated with medications. Instead, the following two surgical operations are the only viable treatments:
Outlook
- Although biliary atresia is a serious and potentially fatal condition, surgical advances have made the long-term outlook for this disease good. For instance, the Kasai procedure can reestablish full bile flow in about 25 percent of children and partial bile flow in about 50 percent of children. While some of these children will be able to retain their native liver, the majority will eventually need a li…
Next Steps
- Our program is staffed by specialists with deep experience identifying, diagnosing, and treating biliary atresia in young children. We are also a nationally recognized leader in both liver transplantation and living donor liver transplantation. Call us at (212) 342-8585 or use our online formto schedule an appointment.