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- W1999402387 abstract "The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5–38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure. The majority of patients with portal cavernoma cholangiopathy (PCC) are asymptomatic, however some (5–38%) present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis. Most patients with extrahepatic portal venous obstruction (EHPVO) present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portosystemic system to reverse the biliary obstruction. It is important to realize that though endoscopic drainage has been proposed as a non-surgical approach to the management of PCC it is successful in only certain specific situations like those with bile duct calculi, cholangitis, etc. A small proportion of such patients will continue to have biliary obstruction and these patients are thought to have a mechanical ischemic stricture. These patients will require a second stage procedure in the form of a bilioenteric bypass to reverse the symptoms related to PCC. In the absence of a shuntable vein splenectomy and devascularization may resolve the PCC in a subset of patients by decreasing the portal pressure. Biliary abnormalities associated with portal hypertension, referred to as portal cavernoma cholangiopthy (PCC) have been reported since the 1980s. These changes were reported mainly in patients with extrahepatic portal venous obstruction (EHPVO) where the incidence was as high as 80–100%.1Dilawari J.B. Chawla Y.K. Pseudosclerosing cholangitis in extrahepatic portal venous obstruction.Gut. 1992; 33: 272-276Crossref PubMed Scopus (145) Google Scholar, 2Khuroo 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, 3Nagi B. Kochhar R. Bhasin D. Singh K. Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances.Acta Radiol. 2000; 41: 612-615Crossref PubMed Scopus (53) Google Scholar, 4Sarin S.K. Bhatia V. Makwane U. Portal biliopathy in extrahepatic portal vein obstruction.Indian J Gastroenterol. 1992; 2: A82Google Scholar, 5Chandra R. Kapoor D. Tharakan A. Chaudhary A. Sarin S.K. Portal biliopathy.J Gastroenterol Hepatol. 2001; 16: 1086-1092Crossref PubMed Scopus (149) Google Scholar, 6Malkan G.H. Bhatia S.J. Bashir K. et al.Cholangiopathy associated with portal hypertension: diagnostic evaluation and clinical implications.Gastrointest Endosc. 1999; 49: 344-348Abstract Full Text Full Text PDF PubMed Scopus (78) Google Scholar The majority of patients with PCC are asymptomatic, however some present with obstructive jaundice, cholangitis, or even biliary pain due to bile duct stones which form as a result of stasis or repeated infections. Only 5–38% of patients with portal hypertension will have symptomatic PCC.4Sarin S.K. Bhatia V. Makwane U. Portal biliopathy in extrahepatic portal vein obstruction.Indian J Gastroenterol. 1992; 2: A82Google Scholar Most patients with EHPVO present with variceal bleeding and hypersplenism and these are the usual indications for surgery. Those who present with PCC may also need decompression of their portal venous system to reverse the biliary obstruction. In most patients the biliary changes are reversed by a portal decompressive procedures like a surgically created shunt because the distended varices in and around the bile duct then collapse. However there is a small group (28–50%) of patients in whom PCC is not relieved after a shunt procedure and these will need drainage to reverse their biliary obstruction either via a stent or a surgical drainage procedure like a hepaticojejunostomy.7Chaudhary 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, 8Khare 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, 9Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar, 10Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar, 11Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB (Oxford). 2012; 14: 441-447Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar These patients present late in life and have usually had repeated episodes of cholangitis and stent exchanges. 2–4% of patients in whom the biliary obstruction has been present for a long time may go on to develop secondary biliary cirrhosis.12Condat 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 Patients with EHPVO who develop symptomatic PCC (5–38%), present with jaundice, pruritus and fever with chills and sometimes abdominal pain if there is associated stone disease. These patients present later, usually in the fourth decade, than those who are seen with variceal bleeding or hypersplenism where the mean age is younger.1Dilawari J.B. Chawla Y.K. Pseudosclerosing cholangitis in extrahepatic portal venous obstruction.Gut. 1992; 33: 272-276Crossref PubMed Scopus (145) Google Scholar, 2Khuroo 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, 3Nagi B. Kochhar R. Bhasin D. Singh K. Cholangiopathy in extrahepatic portal venous obstruction. Radiological appearances.Acta Radiol. 2000; 41: 612-615Crossref PubMed Scopus (53) Google Scholar, 13Sezgin O. Oguz D. Attintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 68: 602-608Abstract Full Text Full Text PDF Scopus (62) Google Scholar These patients may also have repeated episodes of cholangitis and a history of having had repeated endotherapy in the form of stenting, sphincterotomy, etc. A subgroup of patients who go on to develop secondary biliary cirrhosis present with ascites, a decreased serum albumin level and a deranged coagulation profile apart from a raised bilirubin.12Condat 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 primary indications for surgery in these patients with PCC may either be the biliary symptoms such as recurrent cholangitis which have necessitated repeated admissions for endoscopic therapy or for the usual indications for operation in patients with EHPVO i.e. recurrent gastrointestinal bleeding, hypersplenism, abdominal discomfort due to massive splenomegaly and growth retardation in children. Occasionally both sets of symptoms, PCC and bleeding etc. may coexist at presentation. Apart from the altered biochemical parameters, a cholangiogram is mandatory to diagnose PCC. Previously, ERCP was used, but now with the availability of non-invasive tests like the MRCP, ERCP is only used when a bile duct decompression or stone extraction becomes necessary.12Condat 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 diagnostic modalities have two main goals—the first is to diagnose PCC and the second to identify which available vein will be most suitable to construct the portosystemic shunt procedure. This may be done using ultrasonography but now a CT angiography or an MR portovenography is used more frequently. These provide better visualization of the portal venous system as well as the systemic veins such as the renal vein and inferior vena cava which may be used in the anastomosis.14Dhiman 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 Imaging also provides information about the architecture of the liver and the presence of ascites which indicates hepatic decompensation. Once the decision has been taken to perform a portosystemic shunt its type is decided by the preference of the surgeon but, should be a non-selective shunt so that the whole portal venous system pressure is decompressed more effectively leading to regression of the pericholedochal collaterals. Portal decompression as a treatment for PCC was first described by Choudhuri et al in 1988 from India.15Choudhuri G. Tandon R.K. Nundy S. Misra N.K. Common bile duct obstruction by portal cavernoma.Dig Dis Sci. 1988; 33: 1626-1628Crossref PubMed Scopus (40) Google Scholar Later several authors published their experience on the effectiveness of portosystemic shunts in PCC. Although the majority of these patients will benefit from portal decompression alone and not require biliary drainage there is a subset (30–50%) who do not improve and require a biliary drainage procedure such as a Roux-en-Y hepaticojejunostomy.7Chaudhary 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, 9Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar, 10Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (50) Google Scholar Direct biliary drainage has also been attempted but such procedures are often associated with torrential bleeding from the collaterals around the bile duct. It was then proposed that biliary drainage should be done at a later stage after the portal decompression as the pressure in the collaterals around the bile duct is reduced making the procedure less hazardous.9Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar, 16Bejanin H. Baumann R. Choury A. Fritsch J. Buffet C. Portal cavernoma compressing the bile duct. Apropos of three cases.Gastroenterol Clin Biol. 1993; 17: 134-138PubMed Google Scholar It is also apparent that not all patients would require a second procedure and that portal decompression alone was effective in reversing the biliary obstruction in the majority of these patients.7Chaudhary 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, 17Dhiman 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 Those in whom the biliary obstruction was not reversed by a shunt procedure which was demonstrably patent, it was hypothesized that there was an element of ischemia which was responsible for the formation of irreversible mechanical strictures which would ultimately require a biliary drainage.2Khuroo 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 Thus these patients who had a patent shunt and persistent jaundice, had either choledocholithiasis which had developed because of stasis, or had a dominant stricture. Those with bile duct stones can be treated with endotherapy and stenting if there was associated cholangitis. Those with strictures would require biliary drainage as a second stage procedure.17Dhiman 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, 18Chattopadhyay S. Nundy S. Portal biliopathy.World J Gastroenterol. 2012; 18: 6177-6182Crossref PubMed Scopus (27) Google Scholar With advances in endotherapy, patients with PCC are increasingly being managed with multiple stents for prolonged periods of time. One of the strong arguments in favor of operative management has been the fact that surgery is a onetime procedure, does not require repeated hospital visits, which is particularly relevant for the majority of EHPVO patients who come from areas where specialized medical help and access to endotherapy may not be available.11Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB (Oxford). 2012; 14: 441-447Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar In many surgical series, there are a group of about 20–47% of patients who are deemed as nonshuntable due to a lack of any suitable veins. This is because the entire portal venous system is obliterated and replaced by extensive collaterals.9Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google Scholar, 19Poddar U. Thapa B.R. Singh K. Endoscopic sclerotherapy in children: experience with 257 cases of extrahepatic portal venous obstruction.Gastrointest Endosc. 2003; 57: 683-686Abstract Full Text Full Text PDF PubMed Scopus (33) Google Scholar, 20Dhiman R.K. Chhetri D. Behera A. et al.Management of biliary obstruction in patients with portal hypertensive biliopathy (PHB) [abstract].J Gastroenterol Hepatol. 2006; 21: A505Google Scholar In such patients it might be worth performing an oesophagogastric devascularization procedure. Chattopadhyay et al reported that patients who underwent these nonshunt procedures also had a significant reduction in their serum bilirubin levels.11Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB (Oxford). 2012; 14: 441-447Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar Splenic artery ligation leads to reduction in the portal blood flow and portal pressure, which may lower the pressure in the pericholedochal collaterals leading to symptomatic improvement in PCC. There are other surgeons who believe that no patient can have nonshuntable veins and they have performed a portosystemic anastomosis with any patent vein available like the inferior mesenteric vein, or even a large collateral vein to decompress the portal system with success. There have been few patients with PCC and nonshuntable veins, who have been offered liver transplantation.21Filipponi F. Urbani L. Catalano G. et al.Portal biliopathy treated by liver transplantation.Transplantation. 2004; 77: 326-327Crossref PubMed Scopus (27) Google Scholar, 22Hajdu C.H. Murakami T. Diflo T. et al.Intrahepatic portal cavernoma as an indication for liver transplantation.Liver Transpl. 2007; 13: 1312-1316Crossref PubMed Scopus (29) Google Scholar, 23Gupta S. Singhal A. Goyal N. Vij V. Wadhawan M. Portal biliopathy treated with living donor liver transplant: index case.Exp Clin Transplant. 2011; 9: 145-149PubMed Google Scholar In them it is important to identify a portal inflow for the graft. However one needs to discuss the need for long term immunosuppression after transplant vis a vis other options available with these patients. A living related transplant has been done and justified in a patient who had both intrahepatic and extrahepatic biliary strictures which could not be corrected by a surgical biliary bypass.21Filipponi F. Urbani L. Catalano G. et al.Portal biliopathy treated by liver transplantation.Transplantation. 2004; 77: 326-327Crossref PubMed Scopus (27) Google Scholar, 22Hajdu C.H. Murakami T. Diflo T. et al.Intrahepatic portal cavernoma as an indication for liver transplantation.Liver Transpl. 2007; 13: 1312-1316Crossref PubMed Scopus (29) Google Scholar, 23Gupta S. Singhal A. Goyal N. Vij V. Wadhawan M. Portal biliopathy treated with living donor liver transplant: index case.Exp Clin Transplant. 2011; 9: 145-149PubMed Google Scholar There has also been a report of a case with EHPVO and PCC who did not have any shuntable vein and repeated attempts at biliary decompression led to life threatening bleeding from the pericholedochal collaterals. Percutaneous biliary drainage was performed for cholangitis, but an attempt at internalization into the duodenum via the common bile duct led to bleeding from periportal collaterals. Hence a novel approach was used by deploying a self-expanding metal stent between the stomach and the segment II bile duct.24Mistry J.H. Varma V. Mehta N. Kumaran V. Nundy S. Gupta A. Percutaneous transhepatic hepaticogastrostomy for portal biliopathy: a novel approach.Trop Gastroenterol. 2012; 33: 140-143Crossref PubMed Scopus (5) Google Scholar The literature is silent on the role of early portal decompression as a prophylactic procedure in EHPVO patients so that they do not develop symptomatic PCC.25Rao 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 Authors believe that there is no role of prophylactic portosystemic shunt surgery in patients with asymptomatic PCC. Surgical options in a patient with PCC are aimed at decompression of the portal system to reduce the pressure in the collaterals which would take care of the biliary obstruction apart from treating the variceal hemorrhage, hypersplenism etc. This might be the only procedure required in the majority. Decompression of the portal system is achieved by performing a non-selective portosystemic shunt. The type of shunt depends upon the preference of the surgeon, however the commonly performed shunts are proximal splenorenal shunt or a mesocaval shunt. A side-to-side splenorenal shunt is performed by some surgeons to preserve the spleen in young patients. Other makeshift shunts which are less frequently performed are meso-gonadal vein shunt, meso-renal shunt, or between a portal varix and cava.26Kim H.B. Pomposelli J.J. Lillehei C.W. et al.Mesogonadal shunts for extrahepatic portal vein thrombosis and variceal hemorrhage.Liver Transpl. 2005; 11: 1389-1394Crossref PubMed Scopus (12) Google Scholar, 27Drews J.A. Castagna J. Inferior mesorenal shunt as a second procedure for portal decompression.Surg Gynecol Obstet. 1976; 142: 84-86PubMed Google Scholar, 28Warren W.D. Salam A. Smith R.B. The meso–spleno–renal shunt procedures: a comprehensive approach to portasystemic decompression.Ann Surg. 1974; 179: 791-798Crossref PubMed Scopus (12) Google Scholar, 29Camerlo A. Fara R. Barbier L. Grégoire E. Le Treut Y.P. Which treatment to choose for portal biliopathy with extensive portal thrombosis?.Dig Surg. 2010; 27: 380-383https://doi.org/10.1159/000314610Crossref PubMed Scopus (8) Google Scholar A Rex bypass (between the mesenteric vein and the left portal vein) is also performed in children with EHPVO. Portosystemic shunt and Rex bypass may be the only procedure required in majority of the patients with PCC. It might lead to regression of the pericholedochal collaterals and the PCC may not require any further treatment.30Luoto T. Pakarinen M. Mattila I. Rintala R. Mesoportal bypass using a constructed saphenous vein graft for extrahepatic portal vein obstruction–technique, feasibility, and outcomes.J Pediatr Surg. 2012; 4: 688-693https://doi.org/10.1016/j.jpedsurg.2011.10.065Abstract Full Text Full Text PDF Scopus (25) Google Scholar, 31Poddar U. Borkar V. Management of extra hepatic portal venous obstruction (EHPVO): current strategies.Trop Gastroenterol. 2011; 32: 94-102PubMed Google Scholar However in a small subset of patients, a second stage procedure is required in the form of a biliary bypass (Roux-en-Y hepaticojejunostomy) to decompress the obstructed biliary system. There would still remain a subset of patients who would not have a suitable shuntable vein. In this group it has been an observation by some that splenectomy devascularization alone will not only take care of the variceal bleed and hypersplenism but would also reduce the pressure in the pericholedochal collaterals (decreases the portal inflow as well as pressure) and might help in those having symptomatic PCC.11Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB (Oxford). 2012; 14: 441-447Abstract Full Text Full Text PDF PubMed Scopus (27) Google Scholar The other rare surgical option for these patients having EHPVO and PCC is liver transplant.21Filipponi F. Urbani L. Catalano G. et al.Portal biliopathy treated by liver transplantation.Transplantation. 2004; 77: 326-327Crossref PubMed Scopus (27) Google Scholar, 22Hajdu C.H. Murakami T. Diflo T. et al.Intrahepatic portal cavernoma as an indication for liver transplantation.Liver Transpl. 2007; 13: 1312-1316Crossref PubMed Scopus (29) Google Scholar, 23Gupta S. Singhal A. Goyal N. Vij V. Wadhawan M. Portal biliopathy treated with living donor liver transplant: index case.Exp Clin Transplant. 2011; 9: 145-149PubMed Google Scholar This option is applicable only for those patients who are troubled more by the PCC than hypersplenism or variceal bleed. If there is extensive porto-mesenteric thrombosis then the portal inflow to the graft is through the systemic circulation and that would in the form of either a cavoportal hemi-transposition or a reno-portal inflow. This procedure unfortunately does not decompress the splanchnic bed and the patients would continue to have symptoms related to hypersplenism and esophagogastric varices. Follow-up duration in various series has been between 14 months and 12 years. Resolution of PCC has been defined variously—symptomatic relief (itching, cholangitis and jaundice), biochemical resolution (reduction in bilirubin and alkaline phosphatise), and radiological improvement (resolution of biliary obstruction on cholangiogram). Doppler study and shunt patency may not correlate with outcome. Chaudhary et al7Chaudhary 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 in 1998 reviewed their experience in the management of patients with symptomatic PCC (Table 1). Out of 210 patients with EHPVO, 9 (4%) were referred for the surgical management of PCC. Eight of these patients presented with jaundice, two of them with abdominal pain and one patient had repeated episodes of cholangitis. On ERCP, eight patients had a stricture of the bile duct and two had bile duct calculi (one with a stricture, one without a stricture). A direct attempt at biliary drainage in the early period resulted in excessive bleeding from which one patient died, another patient had repeated endotherapy and was advised a shunt procedure which he refused and was lost to follow-up.Table 1Summary of Published Series on the Surgical Management of Portal Cavernoma Cholangiopathy.AuthorsNumbers/ageClinical presentationCholangiographySurgical procedureOutcomeChaudhary et al 1998, 1987–19957Chaudhary 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 ScholarEHPVO-PB9/210 (4%)Mean age—21 yearsRange: 18–36 yearsJaundice-8Abdominal pain-2Recurrent cholangitis-1ERCPStricture-8Bile duct calculi-2Choledochojejunostomy-2Proximal splenorenal shunt-7Anastomotic narrowing-1aLost to follow-up.Died-1Recovered-3Persistent jaundice-4Staged HJ-2Endotherapy-2Khare R et al 2005, 1992–20028Khare 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 ScholarEHPVO-PB-13Medain age—21 yearsRange: 12–50 yearsObstructive jaundice-13A—Biliary stricture-5B—CBD stones-3C—Both-5A—Splenorenal shunt-4B—Endotherapy-2Splenorenal shunt → HJ-1C—Failed endotherapy-4Splenorenal shunt → HJ-1Devascularisation-1Choledochoduodenostomy-1Abandoned-2Perioperative bleeding- 4 Mortality-1Vibert et al 2007, 1980–20009Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (57) Google ScholarSymptomatic PB-19/64(29%)Cholestasis-19Cholangitis-7PSS group-10IHBRD-9EHBRD-7Intrahepatic calculi-3NPSS group-9EHBRD-7Intrahepatic calculi-4Extrahepatic calculi-4PSS groupPTBD & stone extraction-1Splenorenal shunt-10NPSS groupEndoscopic biliary drainage-3Stone extraction-1Stenting-2PTBD-6Biliodigestive anastomosis-4PSS group, Mortality-nilEarly resultsResolution of jaundice-7Long termBiliodigestive bypass-5NPSS groupPersistent jaundice & repeated endotherapy-2Combined endo & percutaneous drainage-2Repeated percutaneous cholangioscopies & stone removal-4Agarwal et al 2010, 1996–200710Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (50) Google ScholarSymptomatic EHPVO-PB39/177 (22%)Mean age—29.6 ± 12.5 yearsRange: 13–56 yearsJaundice-39Cholangitis-24Abdominal pain-24Variceal bleed-33Hypersplenism-7Dominant stricture-15CBD calculi-7Gallstones-12IHBRD-39Proximal splenorenal-37Hepaticojejunostomy-1bExtensive splenoportal venous thrombosis.Devascularisation & HJ-1cShrunken nodular liver, was considered unfit for shunt surgery.Second stage procedure-13dPatients with persistently abnormal LFTs who required surgery.HJ-11Choledochojejunostomy-1Cholecystectomy-1eEndoscopic CBD clearance was done and then had cholecystectomy.Abnormal LFTs-14Second stage-13dPatients with persistently abnormal LFTs who required surgery.Cholangitis-12Endotherapy-10Recurrent cholangitis-4CBD calculi-3Both-3Failed endotherapy-8Mortality-nilChattopadhyay et al 2012, 1996–201011Chattopadhyay S. Govindasamy M. Singla P. et al.Portal biliopathy in patients with non-cirrhotic portal hypertension: does the type of surgery affect outcome?.HPB (Oxford). 2012; 14: 441-447Abstract Full Text Full Text PDF PubMed Scopus (27) Google ScholarOverall PB-56/155(36%)EHPVO-PB-41/113NCPF-PB-15/42Median age—26 yearsRange: 13–68 yearsSymptomatic-24/155(15%)Asymptomatic-32/155(21%)UGI bleed-34/56Jaundice-24/56Cholangitis-7/56Hypersplenism-8/56IHBRD-14GB stones-11CBD stones-7Multiple strictures-5Dominant stricture-3Proximal splenorenal-37Distal splenorenal-1Mesocaval-1Leinoadrenal-1Splenectomy devascularisation-16Persistent jaundice-7CBD stone removal-2Multiple stent exchange-2Blocked shunt-1Mesocaval shunt-1Dominant stricture-2HJ-2Mortality-nilEHPVO—extrahepatic portal vein obstruction, PB—portal biliopathy, HJ—hepaticojejunostomy, ERCP—endoscopic retrograde cholangiopancreatography, CBD—common bile duct, PSS—portosystemic shunt, NPSS—non-portosystemic shunt, IHBRD—intrahepatic biliary radical dilatation, EHBRD—extrahepatic biliary radical dilatation, PTBD—percutaneous transhepatic biliary drainage, LFT—liver function tests, NCPF—non cirrhotic portal fibrosis, UGI—upper gastrointestinal, GB—gall bladder.a Lost to follow-up.b Extensive splenoportal venous thrombosis.c Shrunken nodular liver, was considered unfit for shunt surgery.d Patients with persistently abnormal LFTs who required surgery.e Endoscopic CBD clearance was done and then had cholecystectomy. Open table in a new tab EHPVO—extrahepatic portal vein obstruction, PB—portal biliopathy, HJ—hepaticojejunostomy, ERCP—endoscopic retrograde cholangiopancreatography, CBD—common bile duct, PSS—portosystemic shunt, NPSS—non-portosystemic shunt, IHBRD—intrahepatic biliary radical dilatation, EHBRD—extrahepatic biliary radical dilatati" @default.
- W1999402387 created "2016-06-24" @default.
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- W1999402387 date "2014-02-01" @default.
- W1999402387 modified "2023-09-28" @default.
- W1999402387 title "Surgical Management of Portal Cavernoma Cholangiopathy" @default.
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