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Published on: August 23, 2022
Biliary atresia
1Department of Pediatric Surgery, King's College Hospital, Denmak Hill, London SE5 9RS UK.
Insights
Biliary atresia (BA) is a serious liver disease where bile ducts are destroyed. The Kasai operation offers a chance for recovery, with about 60% of infants clearing jaundice and 40-65% surviving with their native liver.
Area of Science:
- Pediatric Gastroenterology
- Hepatology
- Surgical Innovation
Background:
- Biliary atresia (BA) is a severe cholangiodestructive disease leading to cirrhosis and liver failure.
- Incidence varies globally, with higher rates in Asian countries.
- Associated congenital anomalies define Biliary Atresia Splenic Malformation (BASM) syndrome.
Purpose of the Study:
- To outline the etiology, diagnosis, and management of biliary atresia.
- To describe the surgical approach and outcomes of the Kasai operation.
- To highlight the role of liver transplantation as a second-stage treatment.
Main Methods:
- Diagnosis involves ultrasonography, liver function tests, viral serology, and liver biopsy.
- Classification is based on the level of extrahepatic duct obliteration (Types I, III).
- Management includes the Kasai operation (portoenterostomy) followed by potential liver transplantation.
Main Results:
- The Kasai operation aims to restore bile flow and prevent progression to cirrhosis.
- Post-Kasai, approximately 60% of infants achieve jaundice clearance.
- Five-year survival with native liver ranges from 40% to 65%.
Conclusions:
- Biliary atresia requires timely intervention to prevent irreversible liver damage.
- The Kasai operation is the primary surgical treatment, with variable success rates.
- Liver transplantation remains crucial for cases unresponsive to or complicated by the Kasai procedure.
Abstract:
Biliary atresia (BA) is a cholangiodestructive disease affecting biliary tract, which ultimately leads to cirrhosis, liver failure and death if not treated. The incidence is higher in Asian countries than in Europe. Up to 10% of cases have other congenital anomalies, such as polysplenia, asplenia, situs inversus, absence of inferior vena cava and pre-duodenal portal vein, for which we have coined the term Biliary Atresia Splenic Malformation (BASM) syndrome. For these infants the aetiology lies within the first trimester of gestation. For others affected with BA, aetiology is more obscure and perinatal destruction of fully-formed ducts perhaps by the action of hepatotropic viruses has been suggested. Whatever the cause, the lumen of the extrahepatic duct is obliterated at a variable level and this forms the basis for the commonest classification (Types I, II, III). All patients with BA present with varying degree of conjugated jaundice, pale non-pigmented stools and dark urine. Key diagnostic tests include ultrasonography, biochemical liver function tests, viral serology, and (in our centre) a percutaneous liver biopsy. In some centres, duodenal intubation and measurement of intralumenal bile is the norm. Currently BA is being managed in two stages. The first stage involves the Kasai operation, which essentially excises all extrahepatic biliary remnants leaving a transected portal plate, followed by biliary reconstruction using a Roux loop onto that plate as a portoenterostomy. If bile flow is not restored by Kasai procedure or life-threatening complications of cirrhosis ensue then consideration should be given to liver transplantation as a second stage. The outcome following the Kasai operation can be assessed in two ways: clearance of jaundice to normal values and the proportion who survive with their native liver. Clearance of jaundice (<2 mg/dL or <34 micromol/L) after Kasai has been reported to be around 60%, whereas five years survival with native liver ranges from 40% to 65%.
