Portal biliopathy is a condition characterized by abnormalities in the biliary tract due to portal hypertension, particularly affecting patients with extrahepatic portal vein obstruction. Symptoms can include abdominal pain, jaundice, and cholangitis, with diagnosis primarily via imaging techniques such as ERCP and MRCP. Treatment varies based on symptoms, ranging from medical management for asymptomatic cases to surgical interventions for symptomatic patients.

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
A. Compression theory
B. Ischemic theory
C. Infective theory
Chattopadhyay S et al. 2012

8. A. Compression theory
Long standing EHPVO
Replacement of PV by large
collaterals along CBD
Cavernomatous
transformation of PV
Compress pliable CBD
Long standing EHPVO
Vascular neogenesis and
formation of tumor like CT
Encase CBD/Cause
angulation of BD

9. B. Ischemic theory
Long standing portal thrombosis
Sclerosis of veins draining the BD
Damage to the capillaries and arterioles
Interruption of vascular supply
Ischemic strictures in bile duct

10. C. Infective theory
Infection/
cholangitis
Inflammation
Neogenesis
Deposition of
fibrous tissue
Stricture

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
• Recurrent fever with chills
• Jaundice
• Pruritus
• Biliary colic
• Recurrent 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.)
Abdomen ultrasound with Doppler
Endoscopic retrograde
cholangiopancreatography (ERCP)
Magnetic resonance
cholangiopancreatography (MRCP)
Endoscopic ultrasonography

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.)
Therapeutic role:
This includes-
• Removal of CBD stones,
• Relief of cholangitis, and
• Dilatation 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
Ursodeoxycholic 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:
1. Filling defects seen on imaging may be due to
varices → bleeding during attempted clearance
2. Venous collaterals in the region of the ampulla
of Vater → bleeding during papillotomy
3. 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:
1. Prevents variceal bleeding
2. Biliary bypass possible
Chattopadhyay S et al. 2012

36. Treatment (cont’d.)
Approached in a stepwise fashion:
Managing
biliary
strictures
and
stones
Portal
decompression
should be done
to reverse
biliary
abnormalities
Biliary
obstruction
should be
relieved by
biliary
diversion at
surgery
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