Pediatric Surgery: Biliary Atresia

Biliary Atresia

Basics

  • Definition: Congenital Extrahepatic Bile Duct Obliteration
  • Exact Etiology Uncertain
  • Risk Factors:
    • Asian
    • Female
  • Most Common Indication for Liver TXP in Peds

Associations

  • 20% Are Associated with Other Congenital Malformations
  • Congenital Malformations:
    • Malrotation
    • Cardiac Malformations
    • Polysplenia
    • Preduodenal Portal Vein
    • Azygous Continuation
    • Interrupted IVC

Presentation

  • Progressive Jaundice Beyond 2 Weeks of Life
  • Acholic Stools
  • Dark Urine
  • Firm Hepatomegaly
  • Fat-Soluble Vitamin Deficiency (A/D/E/K)
  • Eventually Causes Cirrhosis & Liver Failure
    • Malnutrition
    • Failure to Thrive

Complete Extrahepatic Biliary Atresia 1

Acholic Stools 2

Classification

  • Japanese Classification
    • Type 1: Atresia at the CBD (10%)
    • Type 2: Atresia at the Hepatic Ducts (2%)
    • Type 3: Atresia at the Porta Hepatis (88% – Most Common)
  • French Classification
    • Type 1: Limited to CBD (3%)
    • Type 2: Hepatic Duct Atresia with Cyst of Liver Hilum Communicating with Dystrophic Intrahepatic Bile Ducts (6%)
    • Type 3: Porta Hepatis Atresia with Patent Gallbladder, Cystic Duct & CBD (19%)
    • Type 4: Porta Hepatis Atresia with Complete Extrahepatic Atresia (72%)

Biliary Atresia Japanese Classification 3

Diagnosis

  • Provisional Diagnosis Made on History & Preoperative Assessment
  • Definitive Diagnosis Always Confirmed at Surgical Exploration
  • Labs: Elevated Bilirubin (Direct & Indirect) & Elevated LFT’s
  • Imaging: US, MRCP or HIDA
    • Give Phenobarbital (5 mg/kg/Days) for 5 Days Prior to HIDA – Enhances Biliary Excretion of Conjugated Bilirubin
  • Liver Biopsy – Periportal Fibrosis & Bile Plugging
    • Most Accurate Nonoperative Diagnostic Test

Biliary Atresia, (a) MRCP, (b) IOC 4

Treatment

  • Primary Treatment: Kasai Procedure (Roux-en-Y Hepaticoportojejunostomy)
  • If Fails: Liver Transplant
  • Outcomes:
    • 1/3 Rule:
      • 1/3 Do Well Long-Term
      • 1/3 Do Well Initially but Need TXP Later
      • 1/3 (15-30%) Do Poor & Need TXP Early
    • 30-Day Mortality: 0-5%
    • 2-Year Survival Rate: 91%

Kasai Procedure 2

References

  1. Chardot C. Biliary atresia. Orphanet J Rare Dis. 2006 Jul 26;1:28. (License: CC BY-2.0)
  2. Wildhaber BE. Biliary atresia: 50 years after the first kasai. ISRN Surg. 2012;2012:132089. (License: CC Not Specified)
  3. Sinha CK, Davenport M. Biliary atresia. J Indian Assoc Pediatr Surg. 2008 Apr;13(2):49-56. (License: CC BY-2.0)
  4. Liu B, Cai J, Xu Y, Peng X, Zheng H, Huang K, Yang J. Three-dimensional magnetic resonance cholangiopancreatography for the diagnosis of biliary atresia in infants and neonates. PLoS One. 2014 Feb 5;9(2):e88268. (License: CC BY-4.0)