Portal biliopathy

Portal biliopathy is defined as abnormalities in the intrahepatic and extrahepatic biliary tract, gallbladder and cystic duct secondary to portal hypertension

1. Portal Biliopathy Presenter: Dr. Maimuna Sayeed Resident Phase B (year 4) Paediatric Gastroenterology and Nutrition BSMMU

2. Outline Definition Vascular supply of the bile duct Etiology Pathogenesis Classification Clinical feature Natural course Investigation Treatment Complication

3. Definition Portal biliopathy is define as abnormalities in the intrahepatic and extrahepatic biliary tract, gallbladder and cystic duct secondary to portal hypertension . Portal biliopathy refers to cholangiographic abnormalities which occur in patients with portal cavernoma. Khuroo MS et al. 2016 Chattopadhyay S et al. 2012

4. Incidence Extrahepatic portal vein obstruction (81%-100%) Non-cirrhotic portal fibrosis (9%-40 %) Cirrhosis of the liver (0%- 33%) Congenital hepatic fibrosis Chattopadhyay S et al. 2012, Suárez V et al. 2013

5. Vascular supply of the bile duct

6. Vascular supply of the bile duct (cont’d . )

7. Pathogenesis There are three main theories for the pathogenesis of portal biliopathy Compression theory Ischemic theory Infective theory Chattopadhyay S et al. 2012

8. A. Compression theory

9. B. Ischemic theory

10. C. Infective theory

11. Pathogenesis (cont’d.)

12. Pathogenesis (cont’d.)

13. Classification I II IIIa IIIb : Suárez V et al. 2013

14. Clinical features Partial or rarely, complete bile duct obstruction Asymptomatic(70%-95 %) Symptomatic( 5%-38 %) Recurrent abdominal pain R ecurrent fever with chills Jaundice Pruritus B iliary colic R ecurrent cholangitis Chattopadhyay S et al. 2012 , Suárez V et al. 2013

15. Natural course Natural course of portal biliopathy is progressive . Long term portal hypertension and significant biliary abnormalities leading to symptomatic biliopathy. Khuroo MS et al. 2016

16. Investigation

17. Investigation Liver function tests: Serum bilirubin-direct Serum alkaline phosphatase Serum aminotransferase Serum albumin Prothrombin time Biliary cirrhosis Biliary imaging ↑ ↑ ↑ ↓ ↑

18. Investigation(cont’d.)

19. Abdomen ultrasound with Doppler An excellent imaging modality in evaluation of portal cavernoma . Color Doppler has advantage of showing varices ( tortuous dilated vessels) around and in the wall of gallbladder. Bile duct wall may show collaterals within the thickened bile ducts. Ultrasound can detect bile duct dilatation with associated cholelithiasis and choledocholithiasis .

20. Abdomen ultrasound with Doppler (cont’d.) Figure: Ultrasound with Doppler in a patient with extrahepatic portal venous obstruction and portal biliopathy Khuroo MS et al. 2016

21. ERCP Gold standard for defining the biliary changes of portal biliopathy. It has both diagnostic and therapeutic role. Khuroo MS et al. 2016

22. ERCP (cont’d.) Diagnostic role: To see changes in the bile ducts, this includes- Single or multiple smooth strictures of varying length and degree, Saccular dilatations, Indentations , Dilated intrahepatic bile duct radicles, Displacement of bile ducts , and Filling defects in the CBD which may be due to stones or varices.

23. ERCP (cont’d.) T herapeutic role: This includes- R emoval of CBD stones, R elief of cholangitis, and D ilatation of dominant strictures with stenting.

24. ERCP ( cont’d.) Figure: Endoscopic retrograde cholangiograms in 8 patients of portal biliopathy depicting spectrum of cholangiographic abnormalities of portal biliopathy Khuroo MS et al. 2016

25. MRCP Typical biliary findings of biliopathy are well seen on MR cholangiography. The sensitivity of MRCP has been found to be similar to ERCP. It differentiates choledochal varices from stones, and identifies portal collaterals. Prominent findings are: biliary stricture, short dilated segments, localized saccular dilatation .

26. MRCP (cont’d.) Figure 4 Magnetic resonance imaging with Magnetic retrograde cholangiogram in a patient with portal biliopathy Khuroo MS et al. 2016

27. MRCP (cont’d.)

28. Endoscopic ultrasonography To the differentiation between CBD varices , stones , and tumors when other imaging modalities are not clear. Portal cavernoma and its anatomy can be well defined. These collaterals appear as multiple vascular channels in and/or around the extrahepatic biliary tract. Paracholedochal , epicholedochal , intra- choledochal and subepithelial varices can be well seen and differentiated.

29. Endoscopic ultrasonography (cont’d.) Figure: Radial endosonography images in portal biliopathy M. S. Sarma et al . 2018

30. Treatment

31. Treatment Asymptomatic patients: Need no active intervention.

32. Treatment (cont’d.) Symptomatic patients: Medical management U rsodeoxycholic acid (10-15mg/kg per day ) Suárez et al. 2013, Khuroo MS et al. 2016

33. Treatment (cont’d.) Endotherapy Indicated: CBD stones Cholangitis Shunt surgery is not feasible Chattopadhyay S et al. 2012

34. Treatment (cont’d.) Complications of endotherapy : Filling defects seen on imaging may be due to varices → bleeding during attempted clearance Venous collaterals in the region of the ampulla of Vater → bleeding during papillotomy Stents become blocked frequently requiring multiple changes → risk of bleeding Chattopadhyay S et al. 2012

35. Treatment (cont’d.) Surgical management: Portosystemic shunt surgery is the treatment of choice. Advantages: Prevents variceal bleeding Biliary bypass possible Chattopadhyay S et al. 2012

36. Treatment (cont’d.) Approached in a stepwise fashion: Khuroo MS et al. 2016

37. Complications Cholestasis Recurrent cholangitis Biliary sludge Gall stone Secondary biliary cirrhosis (2%-4 %) Suárez et al. 2013,

38. Figure: Algorithmic approach to management of portal biliopathy in patients with non-cirrhotic portal hypertension Chattopadhyay S et al. 2012

39. Key points Portal biliopathy (PB) is the late complication of portal hypertension . More commonly seen in patients with extrahepatic portal venous obstruction . External pressure over the bile ducts from biliary collaterals and/or ischaemic injury of bile ducts during portal vein thrombosis seems to be the main mechanism responsible for the development of PB .

40. Key points (cont’d.) Symptoms are associated with higher age , longer duration of disease. MRCP is the choice of investigation. Endotherapy is the preferred treatment for patients with CBD stones , cholangitis or patients with dominant biliary stricture, but without a shuntable vein. Portosystemic shunt should be performed in patients with dominant biliary strictures with a shuntable vein. Rarely, second stage biliary bypass may be required.

41. Thank you