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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. - PowerPoint PPT Presentation
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CONGENITAL ANOMALIES OF HEPATOBILIARY CONGENITAL ANOMALIES OF HEPATOBILIARY
TRACTTRACT
By: Maj Asrar AhmadBy: Maj Asrar Ahmad MBBS, FCPSMBBS, FCPS (Senior Registrar Paeds Surgery)(Senior Registrar Paeds Surgery)
SequenceSequence
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
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