• Infection (reovirus 3, cytomegalovirus [CMV])
• Vascular insufficiency
• Autoimmune obliteration of the bile ducts
• Pancreatic reflux leading to destruction of the biliary duct system

• Absence of the inferior vena cava with azygous continuation
• Preduodenal portal vein and symmetric liver
• Malrotation
• Situs inversus
• Bronchial anomalies
• Multiple spleens

• No clear genetic inheritance can be demonstrated, as indicated by discordance for both monozygotic and dizygotic twins.
• However, HLA-B12, HLA-A9-B5, and HLA-A28-B35 are found with a higher frequency in affected individuals.

• Biliary atresia
• Choledochal cyst
• Sclerosing cholangitis
• Bile duct stenosis
• Anomalies of the choledochopancreaticoductal junction
• Spontaneous perforation of the common bile duct
• Obstructing neoplasia or stone
• Inspissated bile or mucus plug

• Sepsis
• Urinary tract infection (UTI)
• TORCH
• Coxsackie B virus, echovirus

• Tyrosinemia
• Galactosemia
• Hereditary fructose intolerance
• Inborn errors of bile acid metabolism
• Zellweger syndrome
• Benign familial recurrent cholestasis
• a₁-Antitrypsin deficiency
• Neonatal iron-storage disease
• Cystic fibrosis
• Hypopituitarism
• Bile duct paucity (Alagille syndrome and nonsyndromic paucity)
• Trisomy 17, 18, 21 and Turner syndrome

• Medications
• Total parenteral nutrition (TPN)

• Post-shock
• Post-asphyxia
• Congestive heart failure

• Dubin-Johnson syndrome
• Rotor syndrome
• Idiopathic neonatal giant-cell hepatitis

• Jaundice is best visualized by examination of the hard palate, buccal mucosa, or sclera and may not be present until the bilirubin exceeds 5 to 7 dL in the newborn period and 2 dL in the older child.
• Acholic stools, hepatomegaly, and abnormal liver consistency are variable findings and do not need to be present to establish the diagnosis of biliary atresia.

• Conjugated hyperbilirubin is defined as a conjugated fraction greater than 2.0 dL or a conjugated/ total greater than 15%.
• Any child with a conjugated hyperbilirubinemia should undergo the following examinations:
  • Fractionated bilirubin
  • AST, ALT, PT/ PTT, total protein, albumin, CBC
  • Bacterial cultures (blood, urine, stool)
  • Viral studies (hepatitis B, hepatitis C, Epstein-Barr virus [EBV], TORCH, HIV, adenovirus, enterovirus)
  • a₁ -Antitrypsin with Pi typing
  • Urine and serum amino acids
  • Urine organic acids
  • Urine for reducing substance while the child is taking lactose-containing formula; if positive, assay of galactose-1-phosphate uridyl-transferase
  • X-ray studies to exclude evidence of congenital infections and Alagille syndrome (i.e., calcifications of brain and butterfly vertebrae)
  • Eye examination for congenital infections
  • Sweat test
  • HIV serology
  • Thyroid-function tests
  • Abdominal ultrasound
  • Hepatobiliary scintigraphy
  • Liver biopsy
  • Operative cholangiogram, if nonexcreting scintigraphy

• General approach
  • Once the diagnosis of EHBA is established, a surgical drainage procedure is performed.
  • Subsequent management is directed at providing nutrition and monitoring for common problems.
  • Although surgical intervention is helpful, liver disease progresses and the majority of patients will ultimately require liver transplantation.
• Surgical drainage
  • Correctable biliary atresia occurs in up to 10% of patients and is defined as obstruction limited to the distal common bile duct, cystic duct, and/ or gallbladder. These patients may undergo a more limited operation linking the patent distal portion of the tree to the intestine, bypassing the obstruction.
  • Noncorrectable biliary atresia occurs most frequently and is manifested as an absent extrahepatic biliary tree. The standard surgical procedure is the hepatoportoenterostomy (Kasai procedure).
• Nutrition
  • Malabsorption due to decreased concentration of bile acids within the duodenal lumen is common and leads to fat-soluble vitamin deficiency and malnutrition. Patients receive routine supplementation with vitamins A, D, E, and K. The diet should be enriched with medium-chain triglycerides to ensure adequate fat ingestion. Nasogastric tube feedings should be implemented.
• Pruritus is common and develops when there is increased serum bile acid concentration. Many approaches to treatment with only limited success have included ursodeoxycholic acid, antihistamines, cholestyramine, improved nutrition (e.g., nasogastric supplements), rifampin, phenobarbital, naloxone, and biliary diversion.
• Hyperlipidemia/xanthomas: Hyperlipidemia develops as a complication of chronic liver disease and can be treated with choleretic agents such as ursodeoxycholic acid as well as with improved nutrition.
• Ascites
  • Spironolactone, chlorothiazide, and furosemide are commonly used diuretics. Acute changes in fluid balance or a rapid diuresis can be achieved by the use of furosemide in conjunction with albumin replacement
• Liver transplantation: indications for transplantation include life-threatening hemorrhage from portal hypertension, failure to thrive, intractable pruritus, and liver failure.

• Poor growth
• Fat-soluble vitamin deficiency
• Cholangitis
• Portal hypertension
• Pruritus
• Progression of liver damage despite a surgical drainage procedure

• Keep notes in a flow chart.
• Monitor growth parameters and fat-soluble vitamins.
• Monitor liver span, liver texture, and spleen size to follow progress of disease.
• Follow liver-function tests and CBC.
• Watch closely for cholangitis: the most important findings suggestive of cholangitis are fever and elevated transaminases and GGT levels.

Q: When should a patient with neonatal jaundice have a fractionated bilirubin test?
A: If hyperbilirubinemia has not resolved by 6 weeks, fractionation should be performed to allow ample time for evaluation of neonatal cholestasis and the possible need for surgical intervention.

Q: Can the physician prioritize the diagnostic evaluation?
A: In general, the answer is no. Because the diagnosis must be made as early as possible and because many tests are performed only in special laboratories, the full workup should be complete within a few days to 2 weeks, depending on the age of the child.

Q: Will a Kasai operation hurt the chances of successful liver transplantation?
A: No. A review of children who underwent transplantation at Boston Children’s Hospital reported an 89% 10-year survival for patients who had the Kasai procedure.