The normal drainage of bile (a substance required for digestion) is from the liver through a system of ducts (tubes) into the intestine. Biliary atresia is a condition in which the ducts are not present (the exact cause is unknown, but the ducts seem to be injured for some reason and scar closed) and bile cannot move from the liver to the intestines.
This causes backup of bile in the liver which leads to liver damage. It also does not allow normal absorption of formula, so poor weight gain is also a problem.
Jaundice (yellowing of the eyes and skin) raises the concern for biliary atresia, and although there can be many less serious reasons for jaundice, any jaundice in a baby older than 2 weeks should be evaluated by a doctor.
Stool that is tan or clay-colored is another classic symptom of biliary atresia.
Tests for biliary atresia may include lab work, an ultrasound, other imaging studies, a liver biopsy, and an operation in which a type if dye is introduced into the duct system to see if the ducts are open or blocked. If biliary atresia is diagnosed during the operation, then usually at that same operation, a reconstruction is done.
A piece of intestine is sewn directly to the liver so the bile from the liver can drain directly into the intestine. Without treatment, the accumulating bile will ultimately lead to irreversible liver damage, and the patient will require liver transplantation. A successful operation provides bile drainage and may allow the liver to recover. Unfortunately, even with proper surgical reconstruction, patients often progress over time to develop liver disease requiring transplantation.
Up to one-third of patients who undergo reconstruction have good recovery of bile drainage and may not require transplantation. Another one-third of patients will have some improvement but, over years, will have a gradual decline in liver function and require liver transplantation as an older child; however, it may be many years before this occurs and affords the children time to grow. Their increased size increases the pool of available liver donors and transplantation is often technically more straightforward when they are older and bigger.
Another one-third of patients will fail to improve and require liver transplantation within the first year of life. Not all patients are candidates for corrective surgery depending upon the severity of liver disease at presentation. All patients with biliary atresia, even if they have good response to surgery, require long-term follow-up of their liver function.
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