Signs & Symptoms
The usual history is a full term infant who appears normal at birth but develops jaundice after the age of 2 to 3 weeks.
The stools are characteristically pale or even white in colour.
What causes it
Biliary atresia is a disorder of uncertain cause.
About the condition
Biliary atresia is a disorder characterised by progressive obliteration of the extrahepatic biliary system, resulting in obstruction to bile flow. It is the most common surgically treatable cause of cholestatic jaundice encountered during the newborn period. If not corrected surgically, secondary biliary cirrhosis invariably results. Therefore, it seems logical that these patients would eventually develop some degree of liver failure that may necessitate liver transplantation.
Diagnosis and Treatment Options
The diagnosis is based on blood tests to confirm conjugated hyperbilirubinaemia, ultrasound to confirm an atretic gall bladder, radio-isotope HIDA (iminodiacetic acid) scan to confirm obstruction to bile flow, liver biopsy to look for cirrhosis and eventual surgical exploration to determine feasibility of a bile drainage procedure to relieve obstruction.
Early diagnosis of this disease is very important. If surgery is performed before the baby is 2 months old, success is much more likely. After 3 months, success of the operation is poor. For this reason, all infants who are jaundiced after the age of 4 weeks should be evaluated for biliary atresia.
Surgical treatment involves excision of the atretic biliary ducts outside the liver and attaching the small intestine directly to the liver hilum where bile is found or expected to drain. The segment of intestine that connects to the liver also connects to the rest of the intestine and forms a Y connection called a "Roux-en-Y hepatoportojejunostomy," or Kasai procedure.
One third of the patients have clearance of jaundice following surgery and long term survival with their native livers.
One third of the patients have clearance of jaundice albeit with some stable chronic liver disease.
In the remaining one third, bile flow is inadequate following surgery. These children succumb to complications of biliary cirrhosis in the first few years of life unless liver transplantation is performed.
Long term complications following Kasai are cholangitis and portal hypertension.
Prior to the development of liver transplantation as a therapeutic option for children with end-stage liver disease, the long-term survival rate for infants with biliary atresia following portoenterostomy was 47-60% at 5 years and 25-35% at 10 years.
Following liver transplantation survival in these children is more than 85% at 1 year and more than 80% at 5 -years. Long term mortality is very rare.
We, in Singapore, are fortunate to have a Liver Transplant programme. Currently, biliary atresia with cirrhosis accounts for more than 72% of the patients who have undergone a liver transplant. Of these, almost 60% of the children received part of their parents' livers as living related transplantation.
*For infants with Biliary Atresia, the current standard of care is to offer Kasai procedure as the initial option so that the child can live with his native liver and as a continuum of treatment liver transplantation may become necessary for chronic decompensated liver disease