the billiary system is a network of tiny tubular structures and ducts that drain bile from the liver into the small intestine whereby the bile helps with the digestive process bile is a liquid secreted by the liver and it's made up of cholesterol bile salts and waste products like bilirubin biliary atresia is where we have blockage in the ducts or tubes that carry bile from the liver to the gall bladder so we have disruption in the normal hepatic biliary system if this is left untreated we can have progressive damage and due to inflammation fibrosis bilirubic cirrhosis and eventually liver failure may develop biliary atresia is either congenital or acquired and it's a disease that affects newborns the incidence of biliary atresia is approximately one in ten to fifteen thousand birth and it seems to affect girls more than boys at a ratio of two to one let's talk about the different types of biliary atresia we have the fetal embryonic form which happens in the first two weeks of life it may be associated with other congenital defects like interrupted inferior vena cava congenital heart abnormalities and mal rotation of the abdominal viscera the postnatal form is usually found in babies age two to eight weeks so initially the baby is healthy but then symptoms start to develop around the two week period here after birth we have progressive inflammation and obliteration of the extra hepatic bile ducts this form is not associated with other congenital defects and in the beginning the baby will have a short jaundice free period the cause of biliary atresia could be due to infectious agents like rio virus retrovirus cytomegalovirus or human papilloma virus it's also considered to be an autoimmune mechanism by the body where the body's own cells help to destroy the ducts which allow the passage of bile and the third cause can be congenital defects so there's some kind of defective development in the bilirubi tree or we have some kind of ductal plate malformation so when the ductal plate is remodeling it does so in an abnormal way and this may lead to the ductal plate malformation the signs and symptoms of biliary atresia include jaundice which is where we have yellow coloring of the skin and eyes and this is due to high levels of bilirubin in the bloodstream we also have acolytic or clay coloured stools and that's because there isn't any bile or bilirubin being emptied into the intestines and therefore there's a lack of colouring in the stool in the stools leaving them gray in colour we also have dark urine which is due to the fact that bilirubin is getting filtered by the kidneys and excreted in the urine which gives us dark coloured urine appearance we can also have an enlarged firm liver which can occur due to inflammation of the liver and liver cirrhosis over time which can occur due to scarring of the liver tissue now there's a few different ways to diagnose biliary atresia one of which can be just blood tests which assess liver function x-rays could also be undertaken x-rays of the abdomen to visualize an enlarged liver or spleen an abdominal ultrasound can also be performed to find out the size of the gallbladder and detect any abnormalities in size a heda scan helps to determine the flow of bile and in this type of scan a radioactive dye is injected into the infant's vein and the dye actually acts like bilirubin so if the baby has biliary atresia the liver will take up the dye but it would not be able to flow through the damaged biliary system into the small intestines a colangiography is another diagnostic test whereby a contrast material is injected through the gallbladder to find out the communication between the biliary tree and the gastrointestinal tract and lastly another diagnostic method is with a liver biopsy the treatment for biliary atresia the first option is something known as a kasai procedure and this is an operation to create an open duct so bile can drain from the liver the surgeon removes the damaged ducts outside the liver the extra hepatic ducts and replaces them with a piece of the body's own intestine the new duct allows bile to pass from the liver into the intestine the cacai procedure it's not a cure for atresia but it does resolve the problem temporarily and allows the baby to grow and have fairly good health for several years when the procedure doesn't work which it may be a possibility that it doesn't work a liver transplantation is the next step to correct the problem here is a diagram to show you how the casci procedure works the small intestine is attached here directly onto the liver the rest of the intestine is stitched here to the small intestine to form a root ny connection a liver transplant is the final treatment option and if there's not enough bile flow with the carcine procedure the liver transplant is then performed the damaged liver is removed and replaced by a healthy liver from a donor after the transplant surgery the child's health may improve quickly but there may be a risk of transplant rejection by the body as such there needs to be lifelong monitoring of the liver transplant and routine assessments to make sure the new liver is functioning correctly