Biliary Atresia kamal.pptxas,xasmdmasc dcaq

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1. Biliary Atresia Dr. Kamal Hamayel

2. Definitions Biliary atresia is characterized by idiopathic progressive fibroinflamatory and obliterative disease of extrahepatic biliary tree including the gallbladder .   Is the most common surgically treatable cause of  cholestasis  encountered during the newborn period . If not surgically corrected, secondary biliary cirrhosis invariably results. overall incidence in the United States of 1 per 10,000-15,000 live births.

3. Definitions Initial treatment is with the Kasai portoenterostomy in the newborn period. Subsequent liver transplantation is indicated for deteriorating liver function or other intractable complications.

4. Types of BA Postnatal biliary   atresia : isolated BA 65-90 %. Presented in age 2-8 weeks. Embryonic  biliary   atresia : Patients with associated situs inversus or  polysplenia / asplenia  with or without other congenital anomalies in 10-35% of cases . In first 2 weeks of life  

5. Kasai classification system

6. Clinical presentation Most infants are full-term , Appetite, growth, and weight gain may be normal . Typical symptoms include variable degrees of jaundice, dark urine, and light stools . Hepatomegaly , firm or hard liver Splenomegaly  is common, suggestting progressive cirrhosis with portal hypertension .

7. Clinical presentation Direct hyperbilirubinemia is always an abnormal finding and it is typically present from birth in the fetal/embryonic form. In the more common postnatal form, physiologic jaundice frequently merges into conjugated hyperbilirubinemia .   physiologic unconjugated hyperbilirubinemia rarely persists beyond 2 weeks . Using conjugated hyperbilirubinemia as a screening method can detect all cases of BA . Consider biliary atresia in all neonates with direct hyperbilirubinemia .

8. Laboratory Studies Serum  bilirubin  (total and direct): Conjugated hyperbilirubinemia , defined as any level exceeding either 1 mg/ dL (total bilirubin < 5 mg/ dL ) or 20% of total bilirubin (total bilirubin >5 mg/ dL ), is always abnormal. BA typically show only moderate elevations in total bilirubin , which is commonly 6-12 mg/ dL , with the direct (conjugated) fraction comprising 50-60% of total serum bilirubin .

9. Other labs Liver enzymes: to differentiate cholestatic vs hepatocellular jaundice. Serum alpha1-antitrypsin with: Alpha1-antitrypsin deficiency is the most common inherited liver disease that presents with neonatal cholestasis . Sweat chloride: Biliary tract involvement is a well-recognized complication of cystic fibrosis which associated with cholestatic jaundice.

10. Imaging Studies Ultrasonography : may demonstrate absence of the gallbladder and no dilatation of the biliary tree. the sensitivity and specificity of these findings are 80% only. Hepatobiliary scintiscanning : intestinal excretion of radiolabel confirms patency of the extrahepatic biliary system. associated with a 10% rate of false-positive or false-negative diagnostic errors.

11. Procedures (ERCP): for evaluation and management of patients with cholestatic jaundice. Percutaneous liver biopsy: has 90% sensitivity and specificity for diagnosing biliary atresia .  Intraoperative cholangiography : This procedure definitively demonstrates anatomy and patency of the extrahepatic biliary tract. The study is also indicated when biopsy results are equivocal or scintiscan fails to demonstrate clear evidence of duodenal bile excretion.

12. Treatment & Management No primary medical treatment is relevant in the management of extrahepatic biliary atresia . Once biliary atresia is suspected, surgical intervention is the only mechanism available for a definitive diagnosis ( intraoperative cholangiogram ) and therapy (Kasai portoenterostomy ).

13. Surgical management intraoperative cholangiography establishes the diagnosis of extrahepatic biliary atresia , the fibrotic biliary tract remnant is identified, and the patency of the biliary system is assessed. In cases of ductal hypoplasia , further surgical intervention is not indicated, and bile may be collected to evaluate for disorders of bile acid metabolism.

14. Kasai portoenterostomy

15. Post op Medication ursodeoxycholic acid: in patients with chronic cholestatic conditions and bile duct patency, it enhance bile flow.   long-term, high-dose treatment was associated with a higher rate of severe adverse events, including progression to cirrhosis.  to prevent cholangitis postoperatively, prophylaxis with trimethoprim-sulfamethoxazole has been used on a long-term basis. 

16. Post op high dose steroids high-dose methylprednisolone was used in the immediate postoperative period. Studies have shown high-dose corticosteroids following surgery did not result in statistically significant treatment differences in bile drainage at 6 months, Also, corticosteroid therapy was associated with earlier onset of serious adverse events in children with biliary atresia .  

17. Adult survivors of biliary atresia with their native liver 23% and 44% of children with BA survive to 20 years of age with their native liver. Cirrhosis is present in 49% to 79%. Portal hypertension is present in 46% to 97%. Bacterial cholangitis occurs in 21% to 37% during adulthood. Children with BA are at risk of hepatocellular carcinoma, with an incidence of 0.8% to 1.3%

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