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CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT

CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT. By: Maj Asrar Ahmad MBBS, FCPS (Senior Registrar Paeds Surgery). Sequence. Anatomy Embryology Congenital Anomalies Choledochal Cyst Biliary Atresia. Anatomy. Embryology.

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CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT

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  1. CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT By: MajAsrar Ahmad MBBS, FCPS (Senior Registrar Paeds Surgery)

  2. Sequence • Anatomy • Embryology • Congenital Anomalies • Choledochal Cyst • BiliaryAtresia

  3. Anatomy

  4. Embryology

  5. Anomalies • Choledochal Cyst • BiliaryAtresia • Gallbladder • Hypoplasia • Agenisis

  6. Choledochal Cyst • 1/100,000-1/1000 • 4:1 • 67 % before 10 years

  7. Classification • Alonsolej- 1959 • Todani- 1977

  8. Classification (Type-1) (Type-2) (Type-3) (Type-4) (Type-5) 50-80% 1-2% 3-5% 15-35% 15-20%

  9. Pathophysiology • PBMU • Weakness of CBD • Obstruction

  10. Presentation • Infants • Children Classical Triad • Adults

  11. Investigations • Complete Blood Picture • TLC • Liver Function Tests • Bilirubin • ALT • Alkaline Phsophtase • Serum Amylase

  12. Imaging • USG • Screening • Choledocholithiases • CT Scan • MRCP • ERCP

  13. Management

  14. BiliaryAtresia • 1/10,000 • Syndromic (10%) • Non Syndromic (90%) • Systemic Examination

  15. Types 10% 5% 85%

  16. Presentation • Newborn

  17. Investigations • Liver Function Tests • Direct and Indirect Bilirubin • ALT • Alkaline Phsophtase • USG • HIDA Scan • Liver Biopsy • Operative Cholangiogram

  18. Management • Phase-I • HEPATIC PORTOENTEROSTOMY (KASAI’s Procedure) • Phase-II • LIVER TRANSPLANTATION

  19. Kasai Operation

  20. Complications • Cholangitis • Antibiotics • Steroids • Portal Hypertension • Hepatopulmonary Syndrome • Systemic Examination

  21. Results • Native Liver • 5-year Survival: 35-65% • 10-year Survival: 27-53% • Liver Transplantation • 3-year Survival: 85-95% • Systemic Examination

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