Portal Cavernoma Cholangiopathy: Indian Perspective

Abstract

BaCKGrOUnDPortal cavernoma cholangiopathy (PCC) refers to the imaging, endoscopic, and clinically noted changes in the intrahepatic extrahepatic and biliary ducts and gallbladder (GB) abnormalities in patients with extrahepatic portal vein (PV) obstruction (EHPVO) with portal cavernoma formation. The portal cavernoma is formed as a result of recanalization of a thrombosed PV, which results in the formation of multiple collateral channels. PCC is seen in 80% to 100% of patients with EHPVO and is largely asymptomatic. The diagnosis is based on biliary tract imaging based on magnetic resonance (MR) imaging (MRI), such as MR cholangiography (MRC) or endoscopic retrograde cholangiography (ERC). Commonly noted are intrahepatic biliary radicle dilatation (IHBRD), smooth indentations, or impressions on the common bile duct (CBD), duct angulations, ectasias, strictures, stones, filling defects, and GB and pericholedochal varices. 1 There are several synonyms for PCC, and multiple differential diagnoses need to be considered as shown in Fig. 1. Changes like PCC can be identified in cirrhosis (0%-33%) and noncirrhotic portal hypertension (9%-40%), which are denoted as "portal biliopathy," but these changes are limited to the intrahepatic biliary radicles (IHBRs).

Updates in noncirrhotic portal hypertension

“…[15] The development of portal cavernoma cholangiopathy (PCC) due to both the mechanical impact of cavernoma and chronic ischemia deserves special mention. [16,17] PCC is defined as abnormalities in the extrahepatic biliary system, including the gallbladder and cystic duct, with or without abnormalities in the I and II generation biliary ducts in a patient with portal cavernoma. It is seen in 80%-100% of the cases, and a subgroup becomes symptomatic with jaundice, biliary colic, and recurrent cholangitis.…”

Warren Shunt- Positive Outcomes for Cavernous Portal Vein Transformation in a Young Patient

“…Taking into consideration that thrombosis may be a long-term complication of the Warren Shunt, this is a condition that has to be prevented and can be which can be easily detected using abdominal Doppler ultrasonography, as it allows direct visualisation of the shunt, and can reveal splenofugal drainage of blood into the left renal vein. [3,7,8]. Doppler sonography is a trustworthy procedure to accomplish the challenge of monitoring a cavernous portal vein transformation in a young patient, due to its noninvasive, generally painless, as well as the fact that it does not use radiation.…”

“…Abdominal Doppler ultrasonography is the gold standard for long-term monitoring a cavernous portal vein transformation in a young patient. It has a high degree of accuracy in detecting and monitoring portal cavernomas due to its reliable, non-invasive technique, generally painless, and inde nite repetitions, compared to other radiation-based imaging techniques [3,4]. We want to lay emphasis on the importance of colour and /or pulsed Doppler sonography in order to evaluate the blood ow in the cavernous portal vein transformation, in its segmental branches, in the hepatic vein and examine the spleno-renal shunt, and the importance of realizing a Warren shunt as a reliable treatment in order to prevent further decompensations of the liver [4,5].…”

“…Studies show that CTPV in cases of children may occur because of inherited or acquired prothrombotic disorders, abdominal infections. Portal cavernomas usually consists of the development of multiple venous channels within and around a previously stenotic or occluded portal vein, acting as porto-portal collateral vessels like dilated cystic veins, dilated epicholedocal plexus (of Saint), dilated paracholedocal plexus (of Petren), dilated gastric venous branches (left and right gastric veins) [3,4].…”

An Introduction to Liver Disease in India

“…Portal cavernous cholangiopathy, rarely reported outside India, is associated with EHPVO. 10 The challenges to manage patients with advanced liver disease are similar in India as in the rest of world, but the confounding factors are illiteracy, faith in traditional medicine, lack of health care facilities in rural areas, the absence of universal health care and a high prevalence of underlying malnutrition. 11,12 Sarcopenia is very common in Indian patients with advanced cirrhosis, and the presence of sarcopenia is known to increase liver disease-related mortality.…”