When these bile ducts are blocked or when they don't function normally, bile (which is the liquid that the waste products flow out in) backs up in the liver. This bile backup damages the liver and even endangers the baby's life.
Missing or incomplete bile ducts is a serious condition known as biliary atresia.
The bile normally made by the liver, to assist with the digestion of fatty foods, drains through small tube of bile ducts past the gallbladder into the intestine. Blockage of the common bile duct and resulting cholestasis in newborns is surgically treatable. Surgery with the Kasai procedure can reestablish bile flow in most infants and prevent the need for a liver transplant.
Some babies will start to gain weight, then lose weight and have other symptoms that include:
A doctor can confirm suspected biliary atresia by doing X-tests such as a cholangiogram and an abdominal ultrasound, to get a closer look at the structures of the liver and gallbladder.
Blood tests look at the makeup of elements in the blood (bilirubin levels) and specialized scans (cholescintigraphy) further examine the bile ducts and gallbladder.
No one knows exactly what causes biliary atresia. Sometimes the disorder happens by itself. Other times, babies have other diseases in addition to biliary atresia, which complicates treatment.
The Kasai procedure, or Kasai portoenterostomy, replaces the infant’s damaged bile ducts with a section of the infant’s intestine to restore bile flow from the liver to the intestine and prevent liver damage and failure. However, there are times when the scarring has gone too far and the Kasai operation doesn’t work. For this reason, it’s important to make the diagnosis, and perform a Kasai procedure, early in the baby’s life.
Referred or transferred patients generally require a stay in our hospital of approximately seven days. The patient generally receives prophylactic antibiotic therapy following the procedure.
Physicians must continue to monitor infants with biliary atresia for several years following therapy for signs of inadequate biliary flow restoration or other complications. We work together with our patients’ families and primary care physicians to coordinate follow-up care.
Biliary atresia is a rare condition, but the condition is one of the most frequent causes of liver transplantation.