POST-OPERATIVE COMPLICATIONS OF BILIARY ATRESIA

HEPATOPORTOENTEROSTOMY “Kasai procedure” - The most effective surgical treatment to promote bile flow into the intestine Kasai noticed that the fibrous tissue at the portia hepatis invests microscopically patent biliary ductules, which in turn communicate with the intrahepatic ductal system. Transecting this fibrous tissue (encountered cephalad to the bifurcating portal vein), opens these channels and establishes bile flow into a surgically constructed intestinal conduit, usually a Roux-en-Y (surgically created (end-to-side) anastomosis) limb of jejunum. Remove the atretic biliary ducts outside the liver and attach the small intestine directly to the liver at the spot 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.” A liver biopsy is performed at the time of surgery to determine degree of hepatic fibrosis that is present. Likelihood of surgical success is increased if the procedure is accomplished before the infant attains the age of 8 weeks. Source: Schwartz’s Manual of Surgery

HEPATOPORTOENTEROSTOMY A third of patients remain symptom-free after the procedure, but a remainder require liver transplantation because of progressive liver failure. Source: Schwartz’s Manual of Surgery

COMPLICATIONS AFTER KASAI PROCEDURE: ASCENDING CHOLANGITIS Most common complication A bacterial infection of the biliary tree Bacteria normally found in the small intestine moves up the Roux-en-Y causing infection. - It has a strongly negative influence on prognosis after successful surgical repair, being the cause of progressive cirrhosis and death in a considerable number of cases. Source: http://www.classkids.org/library/biliaryatresia.htm Case Report: Late Cholangitis After Kasai Procedure Detected with Magnetic Resonance Cholangiopancreaticography. Journal of Pediatric Gastroenterology and Nutrition.

ASCENDING CHOLANGITIS COMPLICATIONS AFTER KASAI PROCEDURE: Lighter stools Signs of sepsis Increased jaundice ASCENDING CHOLANGITIS Intravenous and long-term oral antibiotics are used to prevent and treat ascending cholangitis. Source: http://www.classkids.org/library/biliaryatresia.htm

COMPLICATIONS AFTER KASAI PROCEDURE: PORTAL HYPERTENSION Formation of scar tissue eventually leads to portal hypertension in many children. Complications of portal hypertension include problems with bleeding and clotting; enlarged weak veins in the esophagus and stomach; and ascites. The most common localisation of the varices are: esophagus, stomach, Roux en Y loop, rectum. Portal hypertension refers to increased pressure in the veins between the intestines and spleen to the liver. In case of failed Kasai operation, portal hypertension is treated by liver transplantation, but often require sclerotherapy or variceal ligation before liver replacement. Source: http://www.classkids.org/library/biliaryatresia.htm http://www.md.ucl.ac.be/pedihepa/BILIARYATRESIA.htm

Cystoenterostomy or liver transplantation may eventually be required. COMPLICATIONS AFTER KASAI PROCEDURE: INTRAHEPATIC BILIARY CAVITIES Large intrahepatic biliary cysts may develop several months to years after the Kasai operation, even in patients with complete clearance of jaundice. These cavities may become infected and/or compress portal vein, requiring external drainage. Portal hypertension refers to increased pressure in the veins between the intestines and spleen to the liver. Cystoenterostomy or liver transplantation may eventually be required. Source: http://www.md.ucl.ac.be/pedihepa/BILIARYATRESIA.htm

COMPLICATIONS AFTER KASAI PROCEDURE: POOR GROWTH AND MALNUTRITION Adequate bile flow is needed for the digestion and absorption of dietary fats and fat-soluble vitamins, including vitamins A, D, E, and K. Hence, when bile flow is reduced, poor growth and malnutrition may result. Special formulas containing medium-chain triglycerides (an easily digested form of dietary fat) and water-soluble vitamin supplements are often prescribed to maximize the child’s growth and development. Source: http://www.classkids.org/library/biliaryatresia.htm