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

2. Sequence Sequence  Anatomy  Embryology  Congenital Anomalies  Choledochal Cyst  Biliary Atresia

3. Anatomy Anatomy

4. Embryology Embryology

5. Anomalies Anomalies  Choledochal Cyst  Biliary Atresia  Gallbladder  Hypoplasia  Agenisis

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

7. Classification Classification  Alonsolej- 1959  Todani- 1977

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

9. Pathophysiology Pathophysiology  PBMU  Weakness of CBD  Obstruction

10. Presentation Presentation  Infants  Children Classical Triad Classical Triad  Adults Jaundice Mass Pain

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

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

13. Management Management

14. Biliary Atresia Biliary Atresia  1/10,000  Syndromic (10%)  Non Syndromic (90%)  Systemic Examination

15. Types Types 85% 10% 5%

16. Presentation Presentation  Newborn Jaundice Hepatomegaly Acholic stools

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

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

19. Kasai Operation Kasai Operation

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

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