Biliary Astresia

Today I was thinking about a woman that I spoke to a while back whose child has Biliary Astresia. She has been on my mind a lot and I am wondering how her little girl is doing. I decicate this post to you.

First of all, children born with Biliary Astresia may also have Heterotaxy w/polysplenia.



What is Biliary Astresia? It is the absence or abnormality of bile ducts from birth. The blockage can happen inside or outside the ducts. The majorty of infants born with this rare conidition continue to have inflammation due to inadequate drainage and may develop scarring called cirrhosis. Babies born with Biliary Astresia may eventually require a liver transplant.

What are the purpose of the ducts? It helps carry a liquid called bile from the liver to the gallbladder. The bile ducts help removes waste form the liver. It also helps carry salts for which in turns help the small intestine break down digest fat. If the bile flow is not working properly then the tube (duct) linking the liver to the gallbladder is blocked. The blockage may lead to liver damage or cirrhossis of the liver. This can be terminal if not treated.

What are the syptoms: Newborns may look normal at birth but jaundice develops by the third week. A baby may lose weight and the jaundice may worsening in time. The newborn may also have dark urnine, enlarged spleen, floating stools, smelly stools, pale or caly colored stools and may gain weight very slowly or not at all.

What types of test can be done to confirm this? Your child’s doctor may do an abdominal or bile ducts x-ray, abdominal ultrasound, a blood test to check bilirubin levers, a liver biopsy, and HIDA scan (also called cholescintigraphy) to see if the gallbladder is working properly.

What are the treatments? Your child may need a procdure called Kasai. This is an operation that is done to link the liver to the small intestine, by going around the abnormal bile ducts. The chances of survial is better if your child has it done by time he or she is 8 weeks old. However, a liver transplant may still be needed. I have read that early surgery will improve the survial of more then 1/3 of infants born with this rare conditon.

Some of the information noted above are taken from various websites for which I noted below. I would also ask your child’s doctor for any reading material they have on Biliary Astresia.

http://www.nlm.nih.gov/medlineplus/ency/article/001145.htm
http://www.emedicine.com/ped/topic2514.htm