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Portal Biliopathy [DT]

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Portal Biliopathy and Extrahepatic Portal Venous Obstruction (EHPVO)

Portal Biliopathy:

  • Portal biliopathy refers to biliary obstruction caused by collaterals and portal cavernoma formation due to longstanding portal hypertension.
  • Occurs frequently in patients with Extrahepatic Portal Venous Obstruction (EHPVO), with a frequency of 81-100%.
    • Cirrhosis: Portal biliopathy seen in 0-33%.
    • Non-cirrhotic portal fibrosis (NCPF): Seen in 9-40%.

Pathophysiology:

  • Longstanding portal hypertension leads to the development of large collaterals in the biliary region, forming a typical portal cavernoma.
  • The left hepatic duct is more commonly and severely involved, where prominent collateral veins form at the junction of the umbilical vein and the left branch of the portal vein.

Types of Portal Biliopathy:

  • Type I: Only extrahepatic bile duct involvement.
  • Type II: Only intrahepatic bile duct involvement.
  • Type IIIA: Both extrahepatic and unilateral intrahepatic duct involvement.
  • Type IIIB: Both extrahepatic and bilateral intrahepatic duct involvement.

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Case Scenario - Extrahepatic Portal Vein Obstruction (EHPVO) with Fever and Jaundice

Initial Presentation:

A 30-year-old male with extrahepatic portal vein obstruction (EHPVO) and on endoscopic therapy for varices presents with fever and jaundice. Evaluation reveals narrowing at the lower end of the common bile duct (CBD) without a stone.

MCQ 1 - Initial Management

Question: What is the next line of management?

a) Hydration and antibiotics

b) ERCP and sphincterotomy without stenting

c) ERCP and stenting

d) ERCP and ENBD placement

Answer: A

  • The next line of management is hydration and antibiotics. This approach addresses the fever and infection aspect while preparing for more definitive treatment. In such cases, invasive procedures like ERCP are deferred initially until the infection is under control.

MCQ 2 - Preferred Definitive Treatment

Question: What is the preferred definitive treatment for this patient?

a) ERCP and stenting

b) Hepaticojejunostomy

c) Portosystemic shunt alone

d) Shunt followed by hepaticojejunostomy if jaundice does not resolve

Answer: D

  • The preferred definitive treatment is a shunt followed by hepaticojejunostomy if jaundice does not resolve. The shunt relieves portal hypertension, which often causes the narrowing of the bile duct, and if the jaundice persists, a hepaticojejunostomy is performed to bypass the obstruction.

MCQ 3 - Treatment of CBD Stone

Question: What is the preferred treatment for CBD stone in this patient?

a) ERCP and stone removal

b) PTBD and percutaneous stone extraction

c) Laparoscopic CBD exploration

d) Open CBD exploration

Answer: A

  • The preferred treatment of CBD stone in this patient is ERCP and stone removal. ERCP is the least invasive and most effective method for managing CBD stones in patients with EHPVO.

Summary:

  • Initial management focuses on hydration and antibiotics.
  • Definitive treatment involves a shunt followed by hepaticojejunostomy if jaundice persists.
  • If a CBD stone is present, ERCP and stone removal is the preferred treatment.

MCQ Discussion - Portal Biliopathy

Question: Which of the following is not true regarding Portal Biliopathy?

a) Strictures could be reversible or irreversible

b) More than 80% are asymptomatic

c) ERCP is the investigation of choice as it could be therapeutic as well, if suspicion is high with USG and Doppler

d) Following shunt two thirds may not require further biliary bypass

Answer: C

  • ERCP is not the investigation of choice for portal biliopathy. Although it can be therapeutic, the investigation of choice is MRCP (Magnetic Resonance Cholangiopancreatography), as it is non-invasive and provides excellent visualization of both the biliary and venous systems. ERCP is often reserved for therapeutic purposes if a biliary intervention is required.

Key Points:

  • Strictures in portal biliopathy can be reversible or irreversible due to the compression of the bile ducts by collateral veins (Answer A is true).
  • More than 80% of cases are asymptomatic, with symptomatic biliary complications occurring in a minority (Answer B is true).
  • Following a shunt, about two-thirds of patients may not require further biliary bypass as the shunt relieves portal hypertension, which can reduce compression on the bile ducts (Answer D is true).