Which of the following are histopathological features of Extra hepatic Biliary Atresia -a) Bile lakesb) Hepatocyte ballooning degenerationc) Marked bile duct proliferationd) Fibrosis of hepatic ducte) Parenchymal cholestasis
• Characterized by progressive obliteration of the extrahepatic and intrahepatic bile ducts.
• Etiology is unknown; incidence 1 in 12,000 live births.
• Presently, there is no medical therapy to reverse the obliterative process
• Patients who are not offered surgical treatment uniformly develop biliary cirrhosis, portal hypertension, and death by 2 years of age.
• MC indication for pediatric liver transplantation
Pathology
• Bile duct proliferation, severe cholestasis with plugging, and inflammatory cell infiltrate are the pathologic hallmarks of this disease.
• Over time, these changes progress to fibrosis with end-stage cirrhosis.
• Positive for neural cell adhesion molecule (CD56) staining
Variants of biliary atresia
• Patency to the level of CBD
• Patency to the level of common hepatic duct
• Left and right hepatic duct at porta involved, solid porta hepatis (90%)
Clinical Features
• Infants with biliary atresia present with jaundice at birth or shortly thereafter.
• Infants with biliary atresia characteristically have acholic, pale gray stools, secondary to obstructed bile flow.
• With passage of time, progressive failure to thrive and, if untreated, develop stigmata of liver failure and portal hypertension (splenomegaly and esophageal varices)
• Associated malformations in 25%: Polysplenia, malrotation, preduodenal portal vein, and intrahepatic vena cava.
Diagnosis
• USG of the liver and GB is important in the evaluation of the infant with cholestasis.
• USG: GB is shrunken and CBD is not visible. A triangle cord sign found on ultrasound has a predictive accuracy of 95%, the gallbladder ghost triad in which the gallbladder is short (<1.9 cm) and irregular and lacks an echogenic inner lining also got good sensitivity.
• Next diagnostic step: Percutaneous liver biopsy if the hepatic synthetic function is normal (diagnostic accuracy 90%).
• Hepatobiliary scintigraphy: In cases in which the USG and biopsy findings are inconclusive (absent excretion into the intestine)
Treatment
• Exploratory laparotomy: If the needle biopsy or abdominal ultrasound is consistent with BA
• Intra-operative cholecystocholangiography: To confirm the diagnosis, demonstration of the fibrotic biliary remnant and definition of absent proximal and distal bile duct patency
• Treatment of choice: Kasai hepatoportoenterostomy (Roux-en-Y hepaticojejunostomy)
Postoperative Management
• Ursodeoxycholic acid (facilitate bile flow) + Methylprednisolone (anti-inflammatory agent) + TMP-SMX (antimicrobial prophylaxis)
• Cholangitis is the MC post-operative complication.
Prognosis
• About 30% of infants undergoing hepatoportoenterostomy before 60 days of age will have a long-term successful outcome and not require liver transplantation.
• Liver transplantation in the patients who develop progressive hepatic fibrosis with resultant portal hypertension and progressive cholestasis.
• Serum bilirubin at 3 months after surgery seems to be strongly predictive of long-term survival.
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