CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT By Maj Asrar
CONGENITAL ANOMALIES OF HEPATOBILIARY TRACT By: Maj Asrar Ahmad MBBS, FCPS (Senior Registrar Paeds Surgery)
Sequence o o o Anatomy Embryology Congenital Anomalies Choledochal Cyst Biliary Atresia
Anatomy
Embryology
Anomalies o o o Choledochal Cyst Biliary Atresia Gallbladder Hypoplasia Agenisis
Choledochal Cyst o 1/100, 000 -1/1000 o 4: 1 o 67 % before 10 years
Classification o Alonsolej- 1959 o Todani- 1977
Classification 50 -80% (Type-1) 1 -2% (Type-2) 3 -5% (Type-3) 15 -35% 15 -20% (Type-4) (Type-5)
Pathophysiology o PBMU o Weakness of CBD o Obstruction
Presentation o Infants o Children Jaundice Classical Triad Pain o Adults Mass
Investigations o Complete Blood Picture n TLC o Liver Function Tests n Bilirubin n ALT n Alkaline Phsophtase o Serum Amylase
Imaging o USG n Screening n Choledocholithiases o CT Scan o MRCP o ERCP
Management
Biliary Atresia o 1/10, 000 o Syndromic (10%) o Non Syndromic (90%)
Types 10% 5% 85%
Presentation o Newborn Acholic stools Jaundice Hepatomegaly
Investigations o Liver Function Tests n Direct and Indirect Bilirubin n ALT n Alkaline Phsophtase o o USG HIDA Scan Liver Biopsy Operative Cholangiogram
Management o Phase-I n HEPATIC PORTOENTEROSTOMY (KASAI’s Procedure) o Phase-II n LIVER TRANSPLANTATION
Kasai Operation
Complications o Cholangitis n Antibiotics n Steroids o Portal Hypertension o Hepatopulmonary Syndrome
Results o Native Liver n 5 -year Survival: 35 -65% n 10 -year Survival: 27 -53% o Liver Transplantation n 3 -year Survival: 85 -95%
- Slides: 21