Portal Cholangiopathy: An Uncommon Cause of Right Upper Quadrant Pain

Abstract

Portal cholangiopathy is one of the complications of the chronic portal vein thrombosis (PVT). Chronic PVT can occur in a patient with acute PVT that usually does not resolve regardless of the treatment. There is a development of collateral blood vessels that bring blood from the portal system towards the liver around the obstruction area, known as the cavernous transformation of the portal vein or portal cavernoma, in a patient with chronic PVT. The appearance and location of collateral channels depends on the extent and location of thrombus in the portomesenteric venous system. If the portomesenteric venous system is occluded near the formation of the portal vein, blood tends to flow through collateral channels that form varices in and around the common bile duct. Portal cholangiopathy (also referred to as portal biliopathy) is common in patients with long-standing chronic PVT. It is due to compression of the large bile ducts by the venous collaterals that form in patients with chronic PVT. Most of the patients with long-standing PVT have portal cholangiopathy. Typically, symptoms of portal cholangiopathy include jaundice, biliary colic, and pruritus. Portal cholangiopathy is a rare complication of chronic portal hypertension, and it is an important differential diagnosis of biliary colic secondary to cholelithiasis. The patient can also present with the sharp right upper quadrant pain, which is atypical by nature for biliary colic.

Keywords: portal cavernoma cholangiopathy; ruq pain.

Figure 1. CT scan.

(A) Axial CT scan through the gallbladder; gallbladder: two gallstones are present (green arrow). The gallbladder is not overly distended. There is pericholecystic fluid present (red arrows); pancreas: the pancreas is atrophic with parenchymal calcifications and prominence of the pancreatic duct. (B) Coronal reconstruction CT scan through porta hepatis shows normal caliber bile ducts. There is a cavernous transformation of the portal vein (black arrow). Numerous varices are identified, including perigastric and pericholecystic.

Figure 2. Hepatobiliary iminodiacetic acid (HIDA) scan

The initial radioangiogram shows an excellent flow to the liver (not pictured). (A) Dynamic images show a uniform distribution of the tracer in the liver — excretion in the gut present by 15 minutes. The gallbladder is not visualized 60 minutes into the study. (B) At this time, additional 1.1 mCi of technetium 99m choletec is administered. Approximately 2 mg of morphine sulfate is given intravenously, and imaging is continued. The gallbladder is visualized following morphine administration (red arrow). Impression: No cystic or common duct obstruction. The gallbladder is visualized (red arrow) after 60 minutes and after morphine administration as above, findings suggestive of chronic gallbladder disease.