Current treatment of EHBA is surgical. Hepatoportoenterostomy for the relief of biliary obstruction in these infants was initially reported in 1959 by Kasai.17 Now commonly called the Kasai procedure, it consists of mobilizing the extrahepatic biliary tree and anastomosing a jejunal Roux en-Y loop to the liver hilum. If successful, any patent intrahepatic bile ducts will drain into the roux limb allowing relief of the biliary obstruction. If possible, a Kasai procedure should be performed before 60 days of age when its short-term success is 80%.16,83,84 Efficacy of the procedure drops with age of the patient, decreasing to 60% by 90 days of age. Although the prognosis is worse and the need for transplantation is higher in infants who undergo the Kasai procedure after 3 months of age, most hepatologists feel the procedure should be attempted in most of these patients. Overall, 20% to 30% of patients who undergo a Kasai for EHBA enjoy long-term stability of their disease.84,85 However, even if the biliary obstruction is successfully treated via the Kasai operation, ongoing lifelong management of the disease is usually required, and aggressive nutritional support with correction of nutritional deficits is likely to improve outcome. 82,86–92
Several approaches have been used to attempt to enhance bile drainage after the Kasai procedure. These include the use of choleretics, such as ursodeoxycholic acid and phenobarbital, bile acid and binding resins, and anti-inflammatory drugs.93,94 Unfortunately, the clinical trials have been limited and it is not yet known whether these measures are beneficial. The use of steroids perioperatively, to decrease the inflammatory response that is known to play a role in the destructive biliary pathogenesis, and that could be exacerbated by the surgical intervention, is a current focus of multicentered research. Recent evaluation does not support the use of oral steroids95 but there are currently no placebo-controlled studies powered well enough to definitely answer the question of whether perioperative steroids are beneficial. BARC is currently performing the definitive study to answer this question (http://www.barcnetwork.org/).
Liver transplantation is performed when biliary flow is not restored and synthetic dysfunction or complications of biliary cirrhosis occur.96