EXTRAHEPATIC BILIARY ATRESIA Introduction.pptx

EHBA

1. EXTRAHEPATIC BILIARY ATRESIA INTRODUCTION AND CLASSIFICATION DR. MAYANK RAJ KARN RESIDENT 1 ST YEAR ( GENERAL SURGERY)

2. CONTENT: 1) HISTORY 2) EMBROYOLOGY 3) ANATOMY 4) PHYSIOLOGY 5) EPIDEMIOLOGY 6) ETIOPATHOGENESIS 7) CLINICAL FEATURES 8) CLASSIFICATION 9) DIAGNOSTIC APPROACH

3. HISTORY JOHN THOMPSON, 1892 : 1 ST described Biliary Atresia. HOLMES, 1916 : Theoretically concluded surgical treatment was possible. LADD : 1 ST to report successful surgical correction of Biliary Atresia. KASAI, 1950 : Surgically connected porta hepatis to intestine.

4. EMBROYOLOGY Hepatic Diverticulum of foregut ( 4 th WOG) Cranial Caudal Intrahepatic ducts Extrahepatic ducts Recanalization of bile ducts oocurs at same period.

5. SURGICAL ANATOMY Left hepatic duct ( Drain from seg . I ,II, III, IV ) longer in course Bile Duct Right hepatic duct ( Drain from seg . V,VI,VII,VIII )

6. Portal Plate System

7. COMMON BILE DUCT Common hepatic duct and cystic duct join to form CBD CBD Supraduodenal part Retroduodenal part Infraduodenal part CBD join with pancreatic duct to form Ampulla of vater and opens into major duodenal papillae Posteromedial wall of 2 nd part of duodenum.

8. SPHINCTER OF ODDI COMPLEX Sphincter of Boyden 2 in number around CBD Sphincter Pancreaticus around pancreatic duct Sphincter of Oddi proper over Ampulla of Vater

9. CALOT’S TRIANGLE AND HEPATOCYSTIC TRIANGLE Calot’s Triangle Boundary Above: Cystic A rtery Below and right : Cystic Duct Below and left : Common H epatic Duct Hepatocystic Triangle Boundary Above : Inferior Margin of liver MOYNIHAN’S HUMP : ( Caterpiller Hump) Present in C alot’s triangle Seen when Right hepatic artery become t ortous

10. PHYSIOLOGY The smallest functional unit of liver is hepatic lobule. Created by 4 to 6 portal triads. Each hepatocyte is encircled by bile canaliculi. Which coalesce to form small bile ducts entering portal triad.

11. Bile is synthesized from cholesterol in liver. Cholic acid ( most abundant) and Chenodeoxycholic acid are primary bile acids . About 95% of Bile is reabsorbed from terminal ileum ( Enterohepatic circulation ). 5 % excreated in stool. Color of bile is due to bilirubin, breakdown product of hemoglobin. Bilirubin get excreted from urine (Urobilinogen) and stool ( Stercobilinogen )

12. EPIDEMIOLOGY Definition : Occlusive Panductular cholangiopathy , Usually presentation at birth. 1 in 10,000 to 1 in 16,700 live births. Female > Male ( 1:4 to 1:7). Higher incidence in Asia than in Europe or North America.

13. ETIOPATHOGENESIS Exact etiology unknown and likely multifactorial. Genetic , inflammatory and infectious causes have been presented but none has been proven. Progression results in Cholestasis, Hepatic Fibrosis and cirrhosis.

14. CLINICAL FEATURES Physiological Jaundice : Appears after 24hrs of birth. Increase in Unconjugated bilirubin. Bilirubin never exceeds > 15mg/dl. Disappears : 7 days in term and 14 days in preterm. Rate of rise < 5mg/dl /24hrs Pathological jaundice: Appears within 1 st day of life. Increase in both Unconjugated and Conjugated Bilirubin. Duration >14days Rate of rise > 5mg/dl /24 hrs

15. When to suspect Biliary Atresia? Neonate presents with yellow discoloration of body > 14 Days. Dark staining of Diapers. Pale color stool. On late presentation : Hepatomegaly, splenomegaly , Ascites , Failure to thrive and Malnutition

16. CLASSIFICATION Phenotypic Classification: Biliary Atresia Splenic Malformation Syndrome: Early trimester insult. Associated with Maternal Diabetes and Invitro Fertilization.

17. 2. Cystic Biliary Atresia : Caused by extrahepatic cystic formation in an obliterated Biliary Tract. Usually confused with Choledochal Malformation Postnatally. 3. Cytomegalovirus- associated Biliary Atresia : 4. Isolated Biliary Atresia: ( most common)

18. Japanese classification : ( Based on level of most proximal Obstruction)

19. Diagnostic Approach:

20. Reference: Pediatric Surgery 7 th edition : Arnold G.Coran Sabiston textbook of Surgery 21 st edition Biliary Atresia : Mark Davenport

21. Thank you.