Biliary Atresia is a rare ailment in newborn children. If left untreated or only partly treated, it can be fatal either immediately or when the infant reaches the ages two or three. It is a particularly rare disorder where one in every 20,000 babies is afflicted. The exact cause of this disorder is not yet known at this time.
It occurs when the ducts which carry bile from the liver to the intestines are either blocked or absent. Bile is important because it is necessary for the proper absorption of certain nutrients such as the vitamins A, D, E and K. When bile ducts are blocked or absent, bile cannot flow properly and gets stuck in the liver, causing “jaundice”, which is the yellowing of the skin. The baby will also be malnourished due to the inability to absorb nutrients. Cirrhosis, which is the destruction of healthy liver cells, also happens as a result of biliary atresia.
Symptoms
About three to six weeks after birth, the baby will appear jaundiced, and will have a large and swollen abdomen. Usually, neonatal jaundice is normal to some newborn babies. It happens when the serum bilirubin levels drop, which is the result of metabolic or physiological adjustments by the baby’s body after birth. When afflicted with neonatal jaundice, a baby is exposed to phototherapy. Phototherapy is the process of bombarding the baby’s abdomen with colored lights to help break down the bilirubin.
If, after intense exposure to phototherapy, the baby will still appear jaundiced, liver enzymes are measured and ultrasound may be used to search for a secondary cause. Other symptoms include stools that are pale grey and darkened urine. Babies will also intense itching, which will make them extremely irritable and uncomfortable. In rare cases, a special needle may be used to extract a tiny part of the baby’s liver to help make an accurate diagnosis.
Treatment - The Kasai Procedure
The preferred procedure is to remove the biliary ducts outside the liver and attach the small intestines directly to the part of the liver where bile is found or is supposed to be drained. This connection forms sort of a Y-shape because the segment of intestine that connects to the liver also connects to the rest of the intestines. Because of this, the connection is technically named "Roux-en-Y hepatoportojejunostomy”, also known as the Kasai procedure.
Age and Success
The Kasai procedure works better when the patient is considerably younger. About 8 out of 10 who receive the Kasai procedure will have a re-established bile flow. Some of them will have some bile drainage while some of them will have complete bile drainage. The other 2 infants who are not helped by the Kasai procedure have no other option but to seek a liver transplant. The success of the operation is more likely when the patient is two months of age, while the result of the operation is usually poor after three months of age. Because of this, jaundiced infants after one month must automatically be tested for biliary atresia.
Liver transplantation is still an important element in the long-term treatment of biliary atresia. It is important to note that pediatric liver transplant is now a highly successful therapy, so there’s always a glimmer of hope for infants who are afflicted with biliary atresia.
