CONGENITAL ANOMALIES OF HEPATOBILIARY CONGENITAL ANOMALIES OF HEPATOBILIARY

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By: Maj Asrar AhmadBy: Maj Asrar Ahmad MBBS, FCPSMBBS, FCPS (Senior Registrar Paeds Surgery)(Senior Registrar Paeds Surgery)

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AnatomyAnatomy EmbryologyEmbryology Congenital AnomaliesCongenital Anomalies Choledochal CystCholedochal Cyst Biliary AtresiaBiliary Atresia

AnatomyAnatomy

EmbryologyEmbryology

AnomaliesAnomalies

Choledochal CystCholedochal Cyst Biliary AtresiaBiliary Atresia Gallbladder Gallbladder HypoplasiaHypoplasia AgenisisAgenisis

Choledochal Cyst Choledochal Cyst

1/100,000-1/10001/100,000-1/1000 4:14:1 67 % before 10 years67 % before 10 years

ClassificationClassification

Alonsolej- 1959Alonsolej- 1959

Todani- 1977Todani- 1977

ClassificationClassification

(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%

PathophysiologyPathophysiology

PBMUPBMU Weakness of CBDWeakness of CBD ObstructionObstruction

PresentationPresentation

InfantsInfants ChildrenChildren

Classical TriadClassical Triad

AdultsAdults

InvestigationsInvestigations Complete Blood PictureComplete Blood Picture

TLC

Liver Function TestsLiver Function Tests Bilirubin ALT Alkaline Phsophtase

Serum AmylaseSerum Amylase

ImagingImaging

USGUSG Screening Choledocholithiases

CT ScanCT Scan MRCPMRCP ERCPERCP

ManagementManagement

Biliary AtresiaBiliary Atresia 1/10,0001/10,000 Syndromic (10%)Syndromic (10%) Non Syndromic (90%)Non Syndromic (90%)

Systemic ExaminationSystemic Examination

TypesTypes

85% 10% 5%

PresentationPresentation

NewbornNewborn

InvestigationsInvestigations

Liver Function TestsLiver Function Tests Direct and Indirect Bilirubin ALT Alkaline Phsophtase

USGUSG HIDA ScanHIDA Scan Liver BiopsyLiver Biopsy Operative CholangiogramOperative Cholangiogram

ManagementManagement

Phase-I Phase-I HEPATIC PORTOENTEROSTOMY (KASAI’s Procedure)

Phase-IIPhase-II LIVER TRANSPLANTATION

Kasai OperationKasai Operation

ComplicationsComplications

Cholangitis Cholangitis Antibiotics Steroids

Portal HypertensionPortal Hypertension Hepatopulmonary SyndromeHepatopulmonary Syndrome

Systemic ExaminationSystemic Examination

ResultsResults

Native LiverNative Liver 5-year Survival: 35-65% 10-year Survival: 27-53%

Liver TransplantationLiver Transplantation 3-year Survival: 85-95%

Systemic ExaminationSystemic Examination