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• W2081531185 abstract "The pathogenesis of portal cavernoma cholangiopathy (PCC) is important as it can impact the choice of treatment modalities. PCC consists of a reversible component, which resolves by decompression of collaterals as well as a fixed component, which persists despite the decompression of collaterals. The reversible component is due to compression by large collaterals located adjacent to the bile duct as well as possibly intracholedochal varices. The fixed component is likely to be due to ischemia at the time of portal vein thrombosis, local ischemia by compression as well as encasement by a solid tumor-like cavernoma comprising of fibrous hilar mass containing multiple tiny collateral veins rather than markedly enlarged portal collaterals. Although cholangiographic abnormalities in portal hypertension are common, the prevalence of symptomatic PCC is low. This is likely to be related to the cause of portal hypertension, the duration of portal hypertension and possibly the pattern of occlusion of the splenoportal axis. There may possibly be higher prevalence of symptomatic PCC in extension of the thrombosis to the splenomesentric veins. The pathogenesis of portal cavernoma cholangiopathy (PCC) is important as it can impact the choice of treatment modalities. PCC consists of a reversible component, which resolves by decompression of collaterals as well as a fixed component, which persists despite the decompression of collaterals. The reversible component is due to compression by large collaterals located adjacent to the bile duct as well as possibly intracholedochal varices. The fixed component is likely to be due to ischemia at the time of portal vein thrombosis, local ischemia by compression as well as encasement by a solid tumor-like cavernoma comprising of fibrous hilar mass containing multiple tiny collateral veins rather than markedly enlarged portal collaterals. Although cholangiographic abnormalities in portal hypertension are common, the prevalence of symptomatic PCC is low. This is likely to be related to the cause of portal hypertension, the duration of portal hypertension and possibly the pattern of occlusion of the splenoportal axis. There may possibly be higher prevalence of symptomatic PCC in extension of the thrombosis to the splenomesentric veins. The biliary changes seen in extrahepatic portal vein occlusion (EHPVO) are referred to as portal cavernoma cholangiopathy (PCC). While these biliary changes are seen on imaging in the majority of patients with EHPVO, they can become symptomatic in some of these patients. The pathogenesis of PCC has been a subject of debate. The pathogenesis is important as it can impact choice of treatment modalities. While external pressure by portal cavernoma and collaterals appear to be an obvious possible cause, the lack of reversal by decompression of the collaterals has led to the consideration that ischemia may play a role. Any theories regarding the pathogenesis of PCC must take into account both the reversible component after decompression of collaterals as well as the fixed cholangiographic abnormalities, which persist despite decompression. The bile duct and the liver have a unique dual blood supply and the development of portal venous thrombosis results in changes in their blood supply and the development of collaterals, which have implications in the development of PCC. The liver has a peculiar dual afferent blood supply with 75% of the blood being supplied by the deoxygenated blood from the portal vein and the remaining 25% being supplied by the well-oxygenated blood from the hepatic artery. Unlike the portal vein, which exclusively supplies the sinusoids, the hepatic artery does not directly supply blood to the sinusoids, but rather supplies the liver parenchyma through arterioportal communications at different anatomic locations before its blood reaches the sinusoids. The artery supplies three compartments within the portal tract: the peribiliary vascular plexus, the portal tract interstitium and the portal vein vasa vasorum. These drain into the hepatic artery-derived portal system, which joins the regular portal system or inlet venule or perfuse the lobule. The artery outside the portal tract, the isolated artery, supplies 2 compartments: the Glisson's capsule and the vasa vasorum of the hepatic veins.1Shah V. Garcia-Cardena G. Sessa W.C. Groszmann R.J. The hepatic circulation in health and disease: report of a single-topic symposium.Hepatology. 1998; 27: 279-288Crossref PubMed Scopus (58) Google Scholar, 2Ekataksin W. The isolated artery: an intrahepatic arterial pathway that can bypass the lobular parenchyma in mammalian livers.Hepatology. 2000; 31: 269-279Crossref PubMed Scopus (51) Google Scholar The hepatic arterial buffer response (HABR) is a unique mechanism by which the hepatic artery can produce compensatory increase in arterial flow in response to a decrease in portal venous flow. The HABR can be explained by the adenosine hypothesis. Adenosine is released at a constant rate in the space of Mall (the thin fluid space surrounding the portal triad). Adenosine is regulated by washout by the portal vein and the hepatic artery. If the portal vein blood flow is reduced, less adenosine is washed away from the space of Mall and the elevated adenosine level leads to hepatic artery dilatation and increased hepatic arterial flow. Because the portal vein cannot control its blood flow, there is no reciprocal HABR seen in cases of hepatic arterial thrombosis.3Schmidt R. Regulation of hepatic blood flow: the hepatic arterial buffer response revisited.World J Gastroenterol. 2010; 16: 6046-6057Crossref PubMed Scopus (13) Google Scholar This may explain why acute portal vein thrombosis is well tolerated while acute arterial thrombosis causes significant liver dysfunction. The hepatic artery has historically been considered to be the main provider of blood and oxygenation of the biliary ducts.4Takasaki S. Hano H. Three-dimensional observations of the human hepatic artery (arterial system in the liver).J Hepatol. 2001; 34: 455-466Abstract Full Text Full Text PDF PubMed Scopus (63) Google Scholar, 5Elias H. Petty D. Terminal distribution of the hepatic artery.Anat Rec. 1953; 116: 9-17Crossref PubMed Scopus (42) Google Scholar However, the portal venous blood flow may have a significant contribution through hepatic artery-portal venous anastomoses. Animal studies have shown the presence of arterioportal communications in the peribiliary plexus.6Mitra S.K. The terminal distribution of the hepatic artery with special reference to arterio-portal anastomosis.J Anat. 1966; 100: 651-663PubMed Google Scholar, 7Cho K.J. Lunderquist A. The peribiliary vascular plexus: the microvascular architecture of the bile duct in the rabbit and in clinical cases.Radiology. 1983; 147: 357-364PubMed Google Scholar The bile ducts are supplied blood by a peribiliary vascular plexus, which is fed by the hepatic artery, the portal vein and the branches of the gastroduodenal artery. Slieker et al8Slieker J.C. Farid W.R.R. van Eijck C.H.J. et al.Significant contribution of the portal vein to blood flow through the common bile duct.Ann Surg. 2012; 255: 523-527Crossref PubMed Scopus (32) Google Scholar analyzed the microvascular blood flow in the common bile duct in 15 patients who underwent pancreaticoduodenectomy with a combination of laser Doppler flowmetry and reflectance spectrometry. Compared to baseline measurements, the microvascular flow through the common bile duct was decreased to 62% after clamping the portal vein, 51% after clamping the hepatic artery and 31% after clamping both. This study emphasized the contribution of portal venous blood flow to the common bile duct. It must however be mentioned that the conditions of this study were far from physiological. The measurements reflected an acute change in microvascular blood flow through the CBD and it is possible that, in time, redistribution may occur within the microcirculation. Besides, in these patients who underwent pancreaticoduodenectomy, neovascularization or alterations in the region due to cancer of pancreatic head that affect microvascular blood flow in the hepatoduodenal ligament could have influenced results. The venous drainage of the bile duct and gallbladder consists of two venous plexuses, namely, epicholedochal venous plexus of Saint9Saint O.A. The epicholedochal venous plexus and its importance as a mean of identifying the common bile duct during operations on extrahepatic biliary tract.Br J Surg. 1971; 46: 489-498Google Scholar and paracholedochal veins of Petren.10Petren T. Die extrahepatischen gallenwegsvenen and ihre. Pathologischanatomische Bedeutum.Verh Anat Ges. 1932; 41: 139-143Google Scholar The epicholedochal venous plexus of Saint forms a fine reticular network on the outer surface of the common bile duct and hepatic ducts. In contrast, the paracholedochal venous plexus of Petren runs parallel to the bile duct. The right sided paracholedochal plexus can communicate with the gastrocolic trunk and pancreaticoduodenal vein and drain into the cystic duct or directly into the liver while the left-sided plexus can communicate with the first jejunal trunk, left and right gastric veins and the left portal vein.11Sharma M. Rameshbabu C.S. Collateral pathways in portal hypertension.J Clin Exp Hepatol. 2012; 2: 338-352Abstract Full Text Full Text PDF Scopus (86) Google Scholar, 12Crabo L.G. Conley D.M. Graney D.O. Freeney P.C. Venous anatomy of the pancreatic head: normal CT appearance in cadavers and patients.AJR. 1993; : 1039-1045Crossref PubMed Scopus (40) Google Scholar The venous drainage of the bile duct is depicted in Figure 1. The patient usually has a silent presentation at the time of portal thrombosis despite the loss of portal venous blood flow, which accounts for 50% of the oxygen requirement of the liver. This silent presentation is likely to be due to two factors. The loss of portal blood flow after portal vein occlusion is compensated initially by the dilatation of the hepatic arterial system in response to the decrease in portal venous blood flow by the HABR. Subsequently there is a rapid development of venous collaterals with formation of cavernoma as well as varices. An immediate increase in arterial blood flow has been seen after portal vein embolization.13Nagino M. Nimura Y. Kamiya J. Kanai M. Hayakawa N. Yamamoto H. Immediate increase in arterial blood flow in embolized hepatic segments after portal vein embolization: CT demonstration.AJR. 1998; 171: 1037-1039Crossref PubMed Scopus (67) Google Scholar In animal studies, portal branch ligation was produced an initial microcirculatory failure with liver atrophy followed by a hepatic arterial buffer response, microvascular remodeling, normoxygenation and hepatocellular proliferation.14Kollmar O. Corsten M. Scheur C. Vollmar B. Schilling M.K. Menger M.D. Portal branch ligation induces a hepatic arterial buffer response, microvascular remodeling, normo-oxygenation, and cell proliferation in portal blood-deprived liver tissue.Am J Physiol Gastrointest Liver Physiol. 2007; 292: G1534-G1542Crossref PubMed Scopus (49) Google Scholar There is scarce data on the effects of decreased portal blood flow on the biliary system. An experimental study of portal branch ligation in rabbits showed only slight dilatation of the bile ducts in the affected lobes after 6–12 months.15Saji Y. The effect of decreased portal blood flow on the biliary system.Jpn J Surg. 1988; 18: 558-568Crossref PubMed Scopus (15) Google Scholar However PCC has been shown to occur early after acute portal vein thrombosis in humans. In a study that included 22 patients with acute portal vein thrombosis, 30% developed significant changes of PCC within 1 year.16Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (65) Google Scholar The dilated biliary system may act as a risk factor for development of biliary stones, which may contribute to symptoms in PCC. Symptoms in PCC are seen to occur in 5–38% of cases of EHPVO. Unlike variceal bleed, which usually occurs in the first two decades of life in EHPVO, the patients with PCC develop symptoms at a higher age and longer duration of disease. The symptoms in EHPVO may occur either due to chronic cholestasis from biliary strictures/compression or due to biliary stones which can cause biliary colic or cholangitis. Patients with prolonged duration of PCC can develop clinical and biochemical evidence of hepatic dysfunction.17Dhiman R.K. Behera A. Chawla Y.K. Dilawari J.B. Suri S. Portal hypertensive biliopathy.Gut. 2007; 56: 1001-1008Crossref PubMed Scopus (124) Google Scholar Collaterals form following portal vein thrombosis, which have been implicated in the reversible component of PCC that may resolve after decompression of these collaterals. In PCC, the dilated venous collaterals cause external pressure and protrusion over the common bile duct and the hepatic ducts, which are thin and pliable.18Dhiman R.K. Puri P. Chawla Y. et al.Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic.Gastrointest Endosc. 1999; 50: 646-652Abstract Full Text PDF PubMed Scopus (112) Google Scholar, 19Bayraktar Y. Balkanci F. Ozenc A. et al.The “pseudo-cholangiocarcinoma sign” in patients with cavernous transformation of the portal vein and its effect on the serum alkaline phosphatase and bilirubin levels.Am J Gastroenterol. 1995; 90: 2015-2019PubMed Google Scholar, 20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholar, 21Dhiman R.K. Singh P. Duseja A. et al.Pathogenesis of portal hypertensive biliopathy (PHB): is it compression by collaterals or ischemia?.J Gastroenterol Hepatol. 2006; 21 ([abstract]): A506Google Scholar In PCC, the dilatation of the plexus of Saint causes fine irregularities in the biliary tract while the dilatation of the plexus of Petren causes extrinsic compression over bile duct.18Dhiman R.K. Puri P. Chawla Y. et al.Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic.Gastrointest Endosc. 1999; 50: 646-652Abstract Full Text PDF PubMed Scopus (112) Google Scholar The left hepatic duct is more commonly involved in PCC, which could be related to the formation of prominent collateral veins where the umbilical vein joins the left branch of the portal vein.22Dhiman R.K. Singh P. Behera A. et al.Diagnosis of portal hypertensive biliopathy (PHB) in patients with extrahepatic portal venous obstruction (EHPVO): endoscopic retrograde cholangiography versus MR cholangiography.J Gastroenterol Hepatol. 2006; 21 ([abstract]): A507Crossref Google Scholar In addition to extrinsic collaterals causing compression, intra bile duct collaterals have also been shown which may contribute to the biliary abnormalities. On EUS paracholedochal varices have been shown to perforate the wall of the bile duct and lie in the subepithelial layer of the bile duct.23Sharma M. Pathak A. Perforators of common bile duct wall in portal hypertensive biliopathy.Gastrointest Endosc. 2009; 70: 1041-1043Abstract Full Text Full Text PDF PubMed Scopus (20) Google Scholar, 24Sharma M. Pathak A. Intracholedochal varices in portal hypertensive biliopathy.Eur J Radiol. 2009; 72: e119-e123Crossref Scopus (14) Google Scholar There are rare case reports of portal biliopathy characterized localization of the portal cavernoma in the wall of the bile duct causing thickening of the walls of the bile duct along with dilatation of the intrahepatic biliary ducts with no evidence of extrinsic compression by collaterals on magnetic resonance (MR) imaging.25Novellas S. Chevallier P. Peroux J.L. Bruneton J.-N. Rare localization of a portal cavernoma in the wall of the common bile duct.J Clin Imaging. 2004; 28: 132-134Abstract Full Text Full Text PDF PubMed Scopus (5) Google Scholar The evidence in favor of compression by dilated venous collaterals comes in the form of:a)Imaging evidence of collaterals impinging on bile duct lumen. Collaterals impinging on bile duct lumen can cause cholangiographic changes. MR cholangiographic studies coupled with MR portography studies have demonstrated that the cholangiographic abnormalities resulted from impingement on the bile duct lumen by the dilated veins of the portal cavernoma.20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholarb)Resolution of cholangiographic abnormalities by decompression of the collaterals. Partial or complete resolution of these changes of PCC on endoscopic retrograde cholangiography (ERC) has been shown after shunt surgery18Dhiman R.K. Puri P. Chawla Y. et al.Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic.Gastrointest Endosc. 1999; 50: 646-652Abstract Full Text PDF PubMed Scopus (112) Google Scholar, 26Khare R. Sikora S.S. Srikanth G. et al.Extrahepatic portal venous obstruction and obstructive jaundice: approach to management.J Gastroenterol Hepatol. 2005; 20: 56-61Crossref PubMed Scopus (45) Google Scholar, 27Chaudhary A. Dhar P. Sarin S.K. et al.Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management.Br J Surg. 1998; 85: 326-329Crossref PubMed Scopus (123) Google Scholar, 28Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of 39 surgically treated patients.HPB. 2011; 13: 33-39Abstract Full Text Full Text PDF Scopus (50) Google Scholar, 29Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar, 30Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB. 2012; 14: 441-447Abstract Full Text Full Text PDF Scopus (27) Google Scholar and after transjugular intrahepatic portosystemic shunt.31Bayraktar Y. Ozturk M.A. Egesel T. et al.Disappearance of “pseudocholangiocarcinoma sign” in a patient with portal hypertension due to complete thrombosis of left portal vein and main portal vein web after web dilatation and transjugular and intrahepatic portosystemic shunt.J Clin Gastroenterol. 2000; 31: 328-332Crossref PubMed Scopus (15) Google Scholar, 32Gorgul A. Kayhan B. Dogan I. et al.Disappearance of pseudocholangiocarcinoma sign after TIPSS.Am J Gastroenterol. 1996; 91: 150-154PubMed Google Scholar The evidence of resolution of PCC by decompression of the collaterals is summarized in Table 1.Table 1Studies Showing Reversibility of Portal Biliopathy After Decompression of Collaterals.AuthorNumber of patientsModality of decompressionResolution of biliary stricturesDhiman et al18Dhiman R.K. Puri P. Chawla Y. et al.Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic.Gastrointest Endosc. 1999; 50: 646-652Abstract Full Text PDF PubMed Scopus (112) Google Scholar5Shunt surgeryPartial reversal – 3Complete reversal – 1No reversal – 1Chaudhary et al27Chaudhary A. Dhar P. Sarin S.K. et al.Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management.Br J Surg. 1998; 85: 326-329Crossref PubMed Scopus (123) Google Scholar7Shunt surgery5Khare et al26Khare R. Sikora S.S. Srikanth G. et al.Extrahepatic portal venous obstruction and obstructive jaundice: approach to management.J Gastroenterol Hepatol. 2005; 20: 56-61Crossref PubMed Scopus (45) Google Scholar4Shunt surgery3Agarwal et al28Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of 39 surgically treated patients.HPB. 2011; 13: 33-39Abstract Full Text Full Text PDF Scopus (50) Google Scholar37Shunt surgery23Vibert et al29Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar10Shunt surgery7Chattopadhyay et al30Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB. 2012; 14: 441-447Abstract Full Text Full Text PDF Scopus (27) Google Scholar43Portosystemic shunting or splenectomy-devascularization38 Open table in a new tab The persistence of changes of PCC after shunt surgery has led to the conclusion that ischemia may play a significant role in the pathogenesis of PCC. However, it is well known that occasionally, the portosystemic collaterals may persist despite effective portal decompression in patients with intra- or extrahepatic portal hypertension.33Sharma B.C. Singh R.P. Narasimhan K.L. Rao K.L.N. Mitra S.K. Dilawari J.B. Effect of shunt surgery on spleen size, portal pressure and oesophageal varices in patients with non-cirrhotic portal hypertension.J Gastroenterol Hepatol. 1997; 12: 582-584Crossref PubMed Scopus (36) Google Scholar, 34Huston D.G. Pereiras R. Zeppa R. Levi J. Schiff E.R. Fink P. The fate of esophageal varices following selective distal splenorenal shunt.Ann Surg. 1976; 183: 496-501Crossref PubMed Scopus (21) Google Scholar Thus, despite shunt surgery for portal hypertension, it would be no surprise that the shunt surgery may not result in complete disappearance of the collaterals and thus the cholangiographic changes may persist.c)There are no cases reported of bile duct stenosis developing before the development of a cavernoma.20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholar The resolution of abnormalities cholangiographic abnormalities is not universal and is often partial which suggests that mere compression by collaterals or portal cavernoma is a simplistic view of the etiology. Smooth strictures open after shunt surgery and proximal dilatation disappear in most patients. The indentations and caliber irregularities disappear after shunt surgery, whereas angulations and ectasias of biliary ducts persist.18Dhiman R.K. Puri P. Chawla Y. et al.Biliary changes in extrahepatic portal venous obstruction: compression by collaterals or ischemic.Gastrointest Endosc. 1999; 50: 646-652Abstract Full Text PDF PubMed Scopus (112) Google Scholar Rigidity of strictures, which has been observed, also supports the ischemic etiology.17Dhiman R.K. Behera A. Chawla Y.K. Dilawari J.B. Suri S. Portal hypertensive biliopathy.Gut. 2007; 56: 1001-1008Crossref PubMed Scopus (124) Google Scholar, 35Mork H. Weber P. Schmidt H. Goerig R.M. Scheurlen M. Cavernomatous transformation of the portal vein associated with common bile duct strictures: report of two cases.Gastrointest Endosc. 1998; 47: 79-83Abstract Full Text Full Text PDF PubMed Scopus (34) Google Scholar While some authors have shown imaging evidence of collaterals causing biliary compression, this is not seen in all the patients. Dhiman et al21Dhiman R.K. Singh P. Duseja A. et al.Pathogenesis of portal hypertensive biliopathy (PHB): is it compression by collaterals or ischemia?.J Gastroenterol Hepatol. 2006; 21 ([abstract]): A506Google Scholar evaluated the relationship of biliary changes with portal cavernoma using MR cholangiography coupled with MR portography in 18 patients with EHPVO and found that in nine patients with dominant stricture of the bile duct, only five (55%) of them were caused by compression from adjacent collaterals and no such relationship was seen in the remaining four (44%); and hence, presumed an ischemic etiology. The various causes of the persistent component of PCC are – (i) ischemia at the time of portal vein thrombosis, (ii) local ischemia due to prolonged local wall compression by collaterals, and (iii) encasement by a fibrous “solid tumor-like cavernoma” which encases the bile duct. Extension of the thrombotic process to small venules of the bile ducts has been proposed to cause ischemia of the bile ducts.36Khuroo M.S. Yattoo G.N. Zargar S.A. et al.Biliary abnormalities associated with extrahepatic portal venous obstruction.Hepatology. 1993; 17: 807-813Crossref PubMed Scopus (182) Google Scholar Historically, the hepatic artery has been considered mainly responsible for biliary blood flow. However, the contribution of the portal vein to the microvascular blood flow through the bile duct is being recognized. Slieker et al8Slieker J.C. Farid W.R.R. van Eijck C.H.J. et al.Significant contribution of the portal vein to blood flow through the common bile duct.Ann Surg. 2012; 255: 523-527Crossref PubMed Scopus (32) Google Scholar have shown that clamping the portal vein decreased the microvascular flow through the common bile duct by 62%. The reciprocal hepatic arterial response, which causes a dilatation of the hepatic arterial system in response to a decrease in the portal venous flow, is an adaptive protective phenomenon that results in a diversion of blood flow toward the sinusoids. It is possible that this may result in reduced supply toward the bile ducts. The importance of portal venous blood flow in ischemic-type biliary lesions (ITBL) after liver transplantation is also being recognized. Following segmental portal vein thrombosis, patients with liver transplant have been seen to develop ITBL in the affected segments in the presence of normal arterial blood flow.37Farid W.R.R. de Jonge J. Slieker J.C. et al.The importance of portal venous blood flow in ischemic-type biliary lesions after liver transplantation.Am J Transpl. 2011; 11: 857-862Crossref PubMed Scopus (34) Google Scholar There may be formation of new vessels and connective tissue resulting in solid tumor-like cavernoma comprising of fibrous hilar mass containing multiple tiny collateral veins. Laparoscopy may reveal compression by a fibrous hilar mass containing multiple tiny collateral veins rather than markedly enlarged portal collaterals.20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholar, 27Chaudhary A. Dhar P. Sarin S.K. et al.Bile duct obstruction due to portal biliopathy in extrahepatic portal hypertension: surgical management.Br J Surg. 1998; 85: 326-329Crossref PubMed Scopus (123) Google Scholar, 37Farid W.R.R. de Jonge J. Slieker J.C. et al.The importance of portal venous blood flow in ischemic-type biliary lesions after liver transplantation.Am J Transpl. 2011; 11: 857-862Crossref PubMed Scopus (34) Google Scholar, 38Nyman R. al-Suhaibani H. Kagevi I. Portal vein thrombosis mimicking tumour and causing obstructive jaundice. A case report.Acta Radiol. 1996; 37: 685-687Crossref PubMed Scopus (11) Google Scholar MR has shown this “solid tumor-like cavernoma” to be characterized by a uniform mass-like structure with a solid appearance mimicking a tumor in which the venous collaterals could not be individualized clearly.20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholar Histology and morphological studies have shown that the cavernous transformation is not only a dilatation of periportal collaterals but also involves neogenesis and formation of connective tissue with increasing duration of thrombosis.39Bechtelsheimer H. Conrad A. Morphology of cavernous transformation of the portal vein. [author's translation].Leber Magen Darm. 1980; 10: 99-106PubMed Google Scholar In such cases having a frozen portal hilum, decompression of portal collaterals may not relieve biliary obstruction. Cholelithiasis in the setting of PCC may be due to chronic cholestasis, changes in the lithogenicity of bile or other factors such as reduced portal flow and associated liver atrophy.40Harmanci O. Bayraktar Y. How can portal vein cavernous transformation cause chronic incomplete biliary obstruction?.World J Gastroenterol. 2012; 18: 3375-3378Crossref PubMed Scopus (24) Google Scholar Reduced contractile function of the gallbladder has also been proposed to contribute to development of gallstones stones due to the presence of gallbladder wall collaterals. Patients with asymptomatic PCC may not have a critical level of extrahepatic biliary obstruction resulting in abnormal emptying of gallbladder and bile lithogenicity.41Dhiman R.K. Sharma A.L.A. Kohli K.K. Chawla Y. Effect of portal biliopathy and gallbladder varices on gallbladder motility and bile lithogenicity in patients with extrahepatic portal venous obstruction [abstract].J Gastroenterol Hepatol. 2004; 19: A736Crossref Scopus (8) Google Scholar Animal studies have shown decreased bile acid synthesis and bile flow following interruption of portal flow with an increased tendency toward formation of stones.42Adachi Y. Kamisako T. Yamamoto T. The effects of temporary occlusion of the superior mesenteric vein or splenic vein on biliary bilirubin and bile acid excretion in rats.J Lab Clin Med. 1991; 118: 261-268PubMed Google Scholar, 43Eto T. Gallstone formation in dogs after selective occlusion of the portal vein branches.Jpn J Surg. 1988; 18: 268-275Crossref PubMed Scopus (8) Google Scholar Pigment stones have been seen to develop in patients with ductal stenosis without stones in the gallbladder.20Condat B. Vilgrain V. Asselah T. et al.Portal cavernoma associated cholangiopathy: a clinical and MR cholangiography coupled with MR portography imaging study.Hepatology. 2003; 37: 1302-1308Crossref PubMed Scopus (163) Google Scholar, 44Bhatia V. Jain A.K. Sarin S.K. Choledocholithiasis associated with portal biliopathy in patients with extrahepatic portal vein obstruction: management with endoscopic sphincterotomy.Gastrointest Endosc. 1995; 42: 178-181Abstract Full Text Full Text PDF PubMed Scopus (43) Google Scholar Although biliary abnormalities in portal hypertension are extremely common, symptomatic PCC is seen only in a small fraction. The low prevalence of symptomatic PCC is likely to be related to the cause of portal hypertension, the duration of portal hypertension and the pattern of occlusion of the splenoportal axis. Development of PCC is related to the duration of portal hypertension. PCC is a rare cause of childhood cholangiopathy.45El-Matary W. Roberts E.A. Kim P. Temple M. Cutz E. Ling S.C. Portal hypertensive biliopathy: a rare cause of childhood cholestasis.Eur J Pediatr. 2008; 167: 1339-1342Crossref PubMed Scopus (6) Google Scholar It usually takes two or three decades to develop PCC usually in adulthood.46Rao K.L.N. Thapa B.R. Portal hypertensive biliopathy: can we prevent it.J Indian Assoc Pediatr Surg. 2010; 15: 1Crossref PubMed Scopus (2) Google Scholar On long term follow up of 82 children with extrahepatic portal vein obstruction, three patients (3.6%) developed symptomatic intrahepatic and extrahepatic biliary dilatation or stenosis (2 at 12 years of age and other at 14 years).47Maksoud-Filho J.G. Gonçalves M.E.P. Cardoso S.R. Gibelli N.E. Tannuri U. Long- term follow-up of children with extrahepatic portal vein obstruction: impact of an endoscopic sclerotherapy program on bleeding episodes, hepatic function, hypersplenism, and mortality.J Paediatr Surg. 2009; 44: 1877-1883Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar Similar results have been shown by Zargar et al48Zargar S.A. Yattoo G.N. Javid G. et al.Fifteen-year follow up of endoscopic injection sclerotherapy in children with extrahepatic portal venous obstruction.J Gastroenterol Hepatol. 2004; 19: 139-145Crossref PubMed Scopus (53) Google Scholar who followed up 69 children with EHPVO for an average of 15 years, three (4%) of whom developed biliary obstruction secondary to PCC. It is likely that this time lag between development of portal hypertension and development of symptomatic PCC is related to progression in size of collaterals around the bile duct, development of fibrosis in the cavernoma, development of local ischemic necrosis of the bile duct due to prolonged pressure by the enlarged collaterals or development of biliary stones in the dilated biliary tract. Llop et al16Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (65) Google Scholar have shown that the appearance of PCC is an early event in the natural history of portal vein thrombosis (PVT) and once PCC appears, the biliary tree abnormalities remain stable. They followed up 67 consecutive patients of PVT, which included 22 patients with acute PVT, for progression of cholangiographic abnormalities development of symptomatic PCC. They graded the cholangiographic changes of PCC as grade I (minimal irregularities), grade II (stenosis without dilatation) and grade III (stenosis with dilatations). They found that symptomatic PCC occurred only in patients with grade III PCC. They found that PCC developed early after acute PVT in 30% and the capacity of biliary abnormalities to progress after 1 year was extremely low. They also found that despite early development of significant PCC, the prevalence of symptomatic PCC increases only with longer period of time and development of biliary stones was a trigger of symptoms in 50% of their cases. That significant/symptomatic PCC may never develop in many patients with PVT may be explainable by the pattern of development of collaterals. Walser et al49Walser E.M. Runyan B.R. Heckman M.G. et al.Extrahepatic portal biliopathy: proposed etiology on the basis of anatomic and clinical features.Radiology. 2011; 258: 146-153Crossref PubMed Scopus (57) Google Scholar compared the anatomy and clinical features of 19 patients of PVT with PCC to 41 patients of PVT without PCC. They found that patients without PCC were more likely to have cirrhosis. On the other hand, patients with PCC were more likely to have extension of the thrombus into the splenomesentric venous thrombosis (95% vs. 2%), lower gastroesophageal varices (16% vs. 49%), smaller coronary vein diameter and acute angulation of the common bile duct at the superior border of the pancreatic head (associated with compression by pancreaticoduodenal veins). They also found that PCC patients had higher incidence of hypercoagulable states. They hypothesized that in patients without PCC, there was a gradual decrease in hepatopedal flow, which causes the coronary vein to dilate. Therefore, when the portal vein finally thromboses, this results in portal decompression into gastroesophageal varices rather than into peripancreatic venous structures. In contrast, when patients with a hypercoagulable state and a normal liver develop an acute thrombosis of the portal and mesenteric veins, there are no portosystemic shunts from chronic portal hypertension and the first large collateral may be the gastrocolic trunk off the superior mesenteric vein. The blocked superior mesenteric vein may result in retrograde flow via gastrocolic trunk into the pancreaticoduodenal veins. The posterior pancreaticoduodenal vein joins the portal vein near the hepatic hilum. This is more likely to cause a different collateral pathway in the setting of PCC where the portomesentric occlusion results in preferential peribiliary collateral flow (retrograde gastrocolic trunk to pancreaticoduodenal venous flow) rather than the commoner gastroesophageal variceal. It is possible that this peribiliary venous pathway may be responsible for bile duct compression.49Walser E.M. Runyan B.R. Heckman M.G. et al.Extrahepatic portal biliopathy: proposed etiology on the basis of anatomic and clinical features.Radiology. 2011; 258: 146-153Crossref PubMed Scopus (57) Google Scholar PCC consists of both a reversible component, which resolves by decompression of collaterals as well as a fixed component, which persists despite the decompression of collaterals. The reversible component is due to compression by large collaterals located strategically close to the bile duct as well as possibly intracholedochal varices. The fixed component is likely to be due to ischemia at the time of portal vein thrombosis, local ischemia by compression as well as encasement by a solid tumor-like cavernoma comprising of fibrous hilar mass containing multiple tiny collateral veins rather than markedly enlarged portal collaterals." @default.
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• W2081531185 title "Pathogenesis of Portal Cavernoma Cholangiopathy: Is it Compression by Collaterals or Ischemic Injury to Bile Ducts During Portal Vein Thrombosis?" @default.
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