Portal biliopathy is an abnormality of the bile duct caused by compression of the bile duct by the bile duct and peribiliary varices, most patients are asymptomatic and a few present with symptomatic portal biliopathy such as recurrent cholangitis, cholelithiasis, and biliary obstruction. This disease is rare, clinical diagnosis and treatment experience is scarce, and improper management can lead to serious consequences. In 1998, Chaudhary et al. reported 9 cases of portal cavernoma (PC) or portal cavernous lesions with abnormal biliary tract morphology, which was the first time portal biliopathy was proposed. The disease is rare and only small samples of cases have been reported both domestically and internationally. The pathophysiological mechanism of portal cholangiopathy has not been fully elucidated yet, but most scholars believe that its etiology is due to the formation of most of the hepatic collateral vessels in the hepatoduodenal ligament after portal vein spongiosis (mostly caused by extrahepatic portal vein obstruction, especially portal vein thrombosis), and these venous plexuses compress the common bile duct and cause bile duct obstruction. After portal vein obstruction, both the Petren vein, which runs close to the common bile duct but is separate from it, and the Saint plexus, which runs on the surface of the common bile duct, dilate, causing compression of the bile duct by Petren vein varices, and Saint vein varices cause changes in the luminal surface of the bile duct, resulting in irregular wall deformation. The combination of the two results in abnormal bile duct morphology. It has been suggested that the tortuous and dilated collateral veins due to portal spongiosis, which surround the bile ducts, together with the varicose veins of the bile ducts, impair the blood supply to the bile ducts and cause ischemic changes in the bile ducts, thus making ischemia the etiology of portal biliary disease. However, recent literature reported that the biliary obstruction symptoms of patients were reversed after portal shunt, thus excluding ischemia as the cause of portal biliary disease. 2. Clinical manifestations and morphological abnormalities of the biliary tract It is difficult to determine the proportion of symptomatic biliary disease in portal biliary disease because the definition of symptoms of portal biliary disease varies in different literature, with some studies including acute biliary colic as a possible symptom and others including only patients with jaundice or cholangitis. Five foreign publications have reported more than 20 patients with symptomatic portal cholangiopathy with a prevalence of 5 to 18 percent. The common clinical manifestations reported were jaundice, abdominal pain, recurrent cholangitis, and upper gastrointestinal bleeding. Laboratory tests show elevated total bilirubin and elevated AKP, ¡-GT. The literature reports that jaundice is rarely seen in children with portal vein obstruction, whereas jaundice is more frequent in adult patients with portal vein thrombosis causing portal spongiform change, suggesting that symptomatic portal biliary disease is closely related to the duration and extent of portal spongiform change. Imaging examinations mainly show features associated with portal spongiform change and biliary morphologic abnormalities, the former including splenomegaly, dilated splenic veins, most collateral veins in the splenic portal, peripancreatic and left perinephric areas, portal or superior mesenteric vein thrombosis, and cavernous tumors with tubular structures (collateral veins) in the hepatic portal area. The biliary morphology abnormalities are related to compression of the common bile duct by the variceal plexus of the hepatoduodenal ligament and fibrosis around the common bile duct, resulting in angulation and displacement of the bile ducts, with more complex manifestations reported in the literature: multiple collateral vessels on the dilated surface of the gallbladder, biliary stones in the intra- and extrahepatic bile ducts, common bile ducts not seen; dilated bile ducts in the hilar region, multiple sinuous collateral vessels around the bile ducts, one or more common bile ducts or common hepatic ducts of varying lengths stenosis, irregularity of extrahepatic bile duct lumen, dilatation of extrahepatic bile duct above stenosis, and metastasis, angulation, and dead branching of extrahepatic bile duct. In addition, some scholars reported that such patients had pseudo-cholangiocarcinoma signs: the caliber of the right and left hepatic ducts and common hepatic duct was larger than that of the common bile duct, most of the distal segments of the common bile duct were involved, and the bile duct was externally compressive defect and narrowed in a wavy and irregular shape, similar to the spread of cholangiocarcinoma along the bile duct, but there was no evidence of biliary tract and jugular belly tumor. One author studied 20 patients with extrahepatic portal vein obstruction of the bile duct by ERCP and all showed pseudosclerosing cholangitis changes, including stenosis, dilatation, and irregular duct walls resembling sclerosing cholangitis manifestations, with the vast majority (90%) of abnormalities limited to the middle bile duct, bile duct venous varices with external compression, and limited stenosis. Intraoperatively, the liver of such patients is normal, but the hepatoduodenal ligament is covered with tortuous veins, thickened and fibrotic, and the gallbladder is also densely covered with a layer of tortuous vessels, so it is difficult to reveal the bile duct or the common bile duct is difficult to identify, and the lateral branch vessels are often mistaken for the bile duct, and forced exposure may lead to hemorrhage. Treatment Treatment should take into account the treatment of upper gastrointestinal bleeding caused by portal hypertension and the removal of biliary obstruction. The varices in the hepatic portal area caused by extrahepatic portal vein obstruction, especially PC, are not only the cause of biliary obstruction, but also an obstacle to the safe implementation of surgical treatment. A common therapeutic error is the lack of awareness of this and performing biliary surgery according to general principles, which encounters abundant collateral veins in the hilar region and throughout the hepatoduodenal ligament, making surgery impossible or causing rupture of these varices with thin walls and high pressure during surgery, intraoperative hemorrhage, or even patient death. Therefore, portal shunts should be the first-line treatment of choice for symptomatic portal biliary disease. Portal shunts reduce the pressure in the bile ducts and surrounding varices, relieving the pressure on the bile ducts and making the symptoms disappear. Patients with this disease often have good liver function and can tolerate the procedure well, even in cases of biliary obstruction and jaundice combined with acute upper gastrointestinal bleeding, which can achieve hemostasis and release biliary obstruction and eliminate jaundice. Sclerotherapy injection should be used with caution in such cases to avoid aggravating the varices around the bile ducts and thus aggravating portal biliary disease. The first case of portal biliary disease treated by portal vein shunt was reported by Choudhuri et al. Splenectomy and proximal splenorenal shunt successfully cured the patient of upper gastrointestinal bleeding and jaundice. Recently Vibert et al. reported good clinical results in 10 cases of symptomatic portal biliary disease treated by splenorenal shunt via retroperitoneal approach with the advantage of less surgical risk (it is common for such patients to have undergone previous surgery) and also to reduce the formation of peritoneal adhesions and avoid subsequent biliary surgery becoming more complicated. If bile duct strictures persist after bypass, interventional treatment such as endoscopic supported dilatation or second-stage biliary diversion procedures such as bile-intestinal anastomosis may be used (biliary surgery is safer after bypass when the pressure in the varicose veins surrounding the bile duct is greatly reduced). If portal vein anastomosis is not possible due to the presence of extensive portal vein thrombosis, intrahepatic biliary bypass can be performed to avoid the risk of hemorrhage associated with dissection of the hepatic tissues including the undecompressed PC. For example, in China, He Zhenping et al [2] reported a case of emergency surgery in a patient with portal biliary tract who encountered a thick finger-like tortuous dilated vein surrounding the gallbladder and encircling the bile duct in the hilar region, and a segment III hepatic duct with T-tube placement and cholecystostomy was performed. A splenectomy and splenic vena cava anastomosis were later performed with a good prognosis. In addition, some interventions have been reported in the literature. In case of acute cholangitis, biliary drainage and antibiotic therapy can be given first. Percutaneous transhepatic route of drainage tube or plastic stent is preferred to relieve biliary strictures and obstruction symptoms, and metal stents are not advisable. Endoscopic treatment by placing drains or plastic stents through the duodenal papilla has the risk of causing severe bleeding and pancreatitis and should be used with caution.