Endoscopic Management of Portal Cavernoma Cholangiopathy: Practice, Principles and Strategy
2013; Elsevier BV; Volume: 4; Linguagem: Inglês
10.1016/j.jceh.2013.08.011
ISSN2213-3453
AutoresVivek A. Saraswat, Praveer Rai, Tarun Kumar, Samir Mohindra, Radha K. Dhiman,
Tópico(s)Pediatric Hepatobiliary Diseases and Treatments
ResumoPortal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied. Portal cavernoma cholangiopathy (PCC) is the presence of typical cholangiographic changes in patients with a portal cavernoma due to chronic portal vein thrombosis, in the absence of other biliary tract diseases. Probably due to biliary stasis related to the cavernoma, there is a high incidence of biliary sludge and calculi in PCC, which trigger symptoms that resolve with appropriate interventions. Persistent and troublesome symptoms are usually due to biliary stenoses or strictures, which may occur with or without biliary calculi and may be short or long, solitary or multifocal, extrahepatic or intrahepatic. Experience with endoscopic interventions in PCC over the last twenty years has shown that it is the procedure of choice for bile duct calculi. Plastic stenting with repeated, timely, stent exchanges is the first line intervention for jaundice or cholangitis due to biliary strictures. If biliary obstruction does not resolve, portosystemic shunt surgery (PSS) or transjugular intrahepatic portosystemic stent shunt (TIPS) is performed to decompress the portal cavernoma. However, for patients with non-shuntable veins or blocked shunts, repeated plastic stent exchanges are the only option though there are reports of the use of biliary self-expandable metal stents in this situation. If symptomatic biliary obstruction persists after successful PSS or TIPS, second stage biliary surgery may be necessary. Recent experience suggests that treating biliary strictures in PCC on the lines of postoperative benign biliary strictures with balloon dilatation and repeated exchanges of plastic stent bundles may be effective therapy. Endoscopic management appears to be associated with an increased frequency of hemobilia, which usually responds to standard management. Recurrent cholangitis with formation of sludge and concretions may be a problem with repeated stent exchanges, especially if patient compliance is poor. In conclusion, the current understanding is that symptomatic PCC is best managed jointly by the endoscopist and surgeon with sequential interventions designed initially to establish and maintain biliary drainage, then to decompress the portal cavernoma and, finally, if required, second stage biliary surgery or endotherapy for biliary strictures. Endoscopic therapy occupies a central role in management before, during and after surgical therapy. Paradigms of endoscopic therapy continue to evolve as knowledge of pathogenesis and natural history improves and newer approaches and techniques are applied. Biliary changes secondary to portal hypertension, especially in portal cavernoma secondary to extrahepatic portal vein obstruction, have long been described in literature under different names by various authors.1Chawla Y. Agrawal S. Portal cavernoma cholangiopathy – history, definition and nomenclature.J Clin Exp Hepatol. 2013; PubMed Google Scholar The Indian National Association for Study of Liver (INASL) working party defined portal cavernoma cholangiopathy (PCC) as abnormalities in the extrahepatic biliary system including the cystic duct and gallbladder, with or without abnormalities in the 1st and 2nd generation biliary ducts, in a patient with portal cavernoma in the absence of other causes of these biliary changes like bile duct injury, primary sclerosing cholangitis, cholangiocarcinoma etc.1Chawla Y. Agrawal S. Portal cavernoma cholangiopathy – history, definition and nomenclature.J Clin Exp Hepatol. 2013; PubMed Google Scholar Although an early report by Sarin2Chandra R. Tharakan A. Kapoor D. Sarin S.K. Comparative study of portal biliopathy in patients with portal hypertension due to different etiologies.Indian J Gastroenterol. 1997; 15 ([abstract]): A59Google Scholar described PCC, called portal biliopathy by the authors, in patients with liver cirrhosis and non-cirrhotic portal fibrosis as well as with extrahepatic portal venous obstruction (EHPVO), subsequent reports found that it occurred almost exclusively in patients with EHPVO with a portal cavernoma. The majority of patients with these abnormalities are asymptomatic and are incidentally detected to have biliary abnormalities on cholangiography. A minority of patients present with symptoms of chronic cholestasis with or without biliary pain or acute cholangitis, related most often to the presence of biliary strictures or stones.3Duseja A. Portal cavernoma cholangiopathy (PCC) – clinical characteristics.J Clin Exp Hepatol. 2013; PubMed Google Scholar Finding stricture with dilatation at cholangiography is associated with a high risk of developing symptoms of PCC. Symptomatic PCC is a late presentation in the natural history of the condition.4Kumar M. Natural history and prognosis of portal cavernoma cholangiopathy.J Clin Exp Hepatol. 2013; PubMed Google Scholar Asymptomatic patients with PCC do not require any treatment. In symptomatic PCC, treatment is focussed on relief of obstructive jaundice and the management of portal hypertension.5Dhiman R.K. Behera A. Chawla Y.K. Dilawari J.B. Suri S. Portal hypertensive biliopathy.Gut. 2007; 56: 1001-1008Crossref PubMed Scopus (122) Google Scholar The era of endoscopic management in PCC began in 1993, with the first report6Lohr J.M. Kuchenreuter S. Grebmeier H. Hahn E.G. Fleig W.E. Compression of the common bile duct due to portal vein thrombosis in polycythemia vera.Hepatology. 1993; 17: 586-592Crossref PubMed Scopus (25) Google Scholar of endoscopic biliary stenting in this condition, almost fifty years after symptomatic PCC was first reported in 1944.7Fraser J. Brown A.K. A clinical syndrome associated with a rare anomaly of vena portal system.Surg Gynecol Obstet. 1944; 78: 520-524Google Scholar Twenty years later, endotherapy has come to occupy a central place in the management of symptomatic PCC. Although therapeutic paradigms are still evolving, the debate for primacy between surgeon and endoscopist is giving way to the perception that close co-ordination between them with a careful, calibrated approach is needed to ensure that patients receive optimal therapy. Endoscopic intervention in PCC began as a fire-fighting exercise for managing cholestasis or cholangitis by establishing biliary drainage with plastic stents or nasobiliary drains. The first report of endoscopic extraction of common bile duct stones in PCC8Bhatia 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 was soon followed by other small series5Dhiman R.K. Behera A. Chawla Y.K. Dilawari J.B. Suri S. Portal hypertensive biliopathy.Gut. 2007; 56: 1001-1008Crossref PubMed Scopus (122) Google Scholar, 9Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar, 10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar, 11Khare 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 (44) Google Scholar, 12Sezgin O. Oguz D. Altintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 58: 602-608Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar, 13Dumortier J. Vaillant E. Boillot O. et al.Diagnosis and treatment of biliary obstruction caused by portal cavernoma.Endoscopy. 2003; 35: 446-450Crossref PubMed Scopus (45) Google Scholar reporting success without an increase in complications, though some workers reported hemobilia during the procedure.14Tighe M. Jacobson I. Bleeding from bile duct varices as unexpected hazard during therapeutic ERCP.Gastrointest Endosc. 1996; 43: 250-252Abstract Full Text Full Text PDF PubMed Scopus (32) Google Scholar, 15Mutignani M. Shah S.K. Bruni A. Perri V. Costamagna G. Endoscopic treatment of extrahepatic bile duct strictures in patients with portal biliopathy carries a high risk of hemobilia: report of 3 cases.Dig Liver Dis. 2002; 34: 587-591Abstract Full Text PDF PubMed Scopus (42) Google Scholar, 16Sharma M. Ponnusamy R.P. Is balloon sweeping detrimental in portal biliopathy? A report of 3 cases.Gastrointest Endosc. 2009; 70: 171-173Abstract Full Text Full Text PDF PubMed Scopus (18) Google Scholar, 17Tyagi P. Puri A.S. Sharma B.C. Balloon sweep in portal biliopathy.Gastrointest Endosc. 2010; 71 ([Lett]): 885-886Abstract Full Text Full Text PDF PubMed Scopus (3) Google Scholar In many centers, endoscopic management has remained confined to establishing drainage of the obstructed biliary system before surgery, in the expectation that either the obstruction would resolve after a period of endoscopic drainage or that portosystemic shunt surgery (PSS), with or without second-stage biliary surgery, would provide definitive management. However, as the complexity of patients with symptomatic PCC was realized, with biliary strictures, calculi or both being present in the extrahepatic, intrahepatic or both locations, and as the difficulties and limitations of surgical management became clear,11Khare 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 (44) Google Scholar, 18Chaudhary 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 (121) Google Scholar, 19Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (56) Google Scholar, 20Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of 39 surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (48) Google Scholar most workers accepted that the optimal management of symptomatic PCC required appropriate use of both endoscopic and surgical interventions. When surgery is unsuccessful, the only option available for patients is repeated stent exchanges for prolonged periods or lifelong. When biliary access is difficult, some workers have resorted to the placement of covered removable self-expanding metallic stents but their long-term outcome remains uncertain.10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar Recently, good results have been obtained with the use of plastic stent bundles as popularized for postoperative benign biliary strictures (personal data described below). Patients with PCC appear to have increased prevalence of biliary calculi (Table 1), though available data come from small series of non-consecutive patients treated in tertiary care centers and may be an overestimate. Eleven series published between 1992 and 20119Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar, 10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar, 12Sezgin O. Oguz D. Altintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 58: 602-608Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar, 13Dumortier J. Vaillant E. Boillot O. et al.Diagnosis and treatment of biliary obstruction caused by portal cavernoma.Endoscopy. 2003; 35: 446-450Crossref PubMed Scopus (45) Google Scholar, 18Chaudhary 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 (121) Google Scholar, 19Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (56) Google Scholar, 20Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of 39 surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (48) Google Scholar, 21Bayraktar Y. Balkanci F. Kayhan B. Ozenç A. Arslan S. Telatar H. Bile duct varices or "pseudocholangiocarcinoma sign" in portal hypertension due to cavernous transformation of the portal vein.Am J Gastroenterol. 1992; 87: 1801-1806PubMed Google Scholar, 22Condat 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 (157) Google Scholar, 23Dhiman R.K. Chawla Y. Duseja A. et al.Portal hypertensive biliopathy (PHB) in patients with extrahepatic portal venous obstruction (EHPVO).J Gastroenterol Hepatol. 2006; 21 ([abstract]): A504Google Scholar have reported a mean frequency of 26.3% (16–85%) for biliary calculi among a total of 331 patients, including 143 with symptomatic PCC. Prevalence of both, gallbladder (mean 13.6%, range 0–69%) as well as biliary ductal calculi (mean 17.8%, range 0–77%), is increased and prevalence is much higher in symptomatic PCC, being 60.8% overall in 143 symptomatic patients (35.1% for gallstones, 41.2% for choledocholithiasis).5Dhiman R.K. Behera A. Chawla Y.K. Dilawari J.B. Suri S. Portal hypertensive biliopathy.Gut. 2007; 56: 1001-1008Crossref PubMed Scopus (122) Google Scholar, 9Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar, 10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar, 11Khare 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 (44) Google Scholar, 18Chaudhary 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 (121) Google Scholar While increased prevalence of gallstones in PCC remains inadequately explained, it appears that biliary stasis due to collateral compression or stricture formation results in the high prevalence of biliary calculi and that formation of these calculi may precipitate biliary obstruction and trigger symptoms in patients with PCC.9Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar, 10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google ScholarTable 1Frequency of Biliary Calculi in Portal Cavernoma Cholangiopathy.Author yearSubjectsDurationBiliary calculi, N (%)Gallbladder calculi, N (%)Bile duct calculi, N (%)Bayraktar 199221Bayraktar Y. Balkanci F. Kayhan B. Ozenç A. Arslan S. Telatar H. Bile duct varices or "pseudocholangiocarcinoma sign" in portal hypertension due to cavernous transformation of the portal vein.Am J Gastroenterol. 1992; 87: 1801-1806PubMed Google ScholarPortal vein thrombosis—47–8 (19)08 (19)Chaudhary 199818Chaudhary 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 (121) Google ScholarSymptomatic PCC—9–2 (22)02 (22)Condat 200322Condat 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 (157) Google ScholarSymptomatic PCC—72 years4 (16)2 (8)2 (8)Sezgin 200312Sezgin O. Oguz D. Altintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 58: 602-608Abstract Full Text Full Text PDF PubMed Scopus (61) Google ScholarSymptomatic PCC—106 years2 (20)1 (10)1 (10) intrahepaticDumortier 200313Dumortier J. Vaillant E. Boillot O. et al.Diagnosis and treatment of biliary obstruction caused by portal cavernoma.Endoscopy. 2003; 35: 446-450Crossref PubMed Scopus (45) Google ScholarSymptomatic PCC—6–4 (66)2 (33)4 (66)Khare 200511Khare 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 (44) Google ScholarSymptomatic PCC—1310 years8 (61)6 (46)8 (61)Dhiman 200623Dhiman R.K. Chawla Y. Duseja A. et al.Portal hypertensive biliopathy (PHB) in patients with extrahepatic portal venous obstruction (EHPVO).J Gastroenterol Hepatol. 2006; 21 ([abstract]): A504Google ScholarPortal vein thrombosis—53–11 (20)7 (13)4 (7)Symptomatic PCC—13Vibert 200719Vibert E. Azoulay D. Aloia T. et al.Therapeutic strategies in symptomatic portal biliopathy.Ann Surg. 2007; 246: 97-104Crossref PubMed Scopus (56) Google ScholarPortal vein thrombosis—6420 years11 (58)04 (21)Symptomatic PCC—19Intrahepatic, 7 (37)Oo 200910Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google ScholarSymptomatic PCC—1313 years11 (85)9 (69)10 (77)Agarwal 201120Agarwal A.K. Sharma D. Singh S. Agarwal S. Girish S.P. Portal biliopathy: a study of 39 surgically treated patients.HPB (Oxford). 2011; 13: 33-39Abstract Full Text Full Text PDF PubMed Scopus (48) Google ScholarSymptomatic PCC—3911 years19 (48.7)12 (31)7 (18)Llop 20119Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google ScholarPCC—5212 years7 (50)6 (43)2 (14)Symptomatic PCC—14Saraswat 2013 (personal data)Symptomatic PCC—2016 years10 (50)6 (30)8 (40)Total351NA97 (27.7)51 (14.6)67 (19.1)Symptomatic PCC—163(59.9)(31.5)(41.3)PCC, portal cavernoma cholangiopathy; PVT, portal vein thrombosis. Open table in a new tab PCC, portal cavernoma cholangiopathy; PVT, portal vein thrombosis. Endoscopic clearance of CBD calculi in patients with PCC was first reported by Bhatia and Sarin in 1995.8Bhatia 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 In three years, they managed 4 patients with PCC and choledocholithiasis, two of whom developed cholangitis. Three of the 4 patients underwent ES with extraction of multiple small brown black calculi. No complications were reported, symptoms resolved and patients were well 4–8 months after the procedure. Khare et al11Khare 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 (44) Google Scholar found bile duct calculi in 8 of their 13 patients, including 5 who had stones above common bile duct strictures. Endoscopic clearance by sphincterotomy followed by repeated sweeps with balloon extractors and Dormia baskets was successful in 5 of the 6 patients in whom it was attempted. Multiple sessions (12 sessions in 3 patients) were necessary for successful clearance in patients with calculi above strictures, who needed biliary balloon dilatation and mechanical lithotripsy. Oo et al10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar found biliary calculi or sludge in 10 of 13 patients in their series. However, they reported successful CBD clearance after ES and balloon trawl in only 1 patient, though biliary drainage was achieved in 8. Llop et al9Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar reported a series of 52 patients with PCC followed over 12 years (1996–2008), including 14 who were symptomatic. Choledocholithaisis in 6 patients could be treated by sphincterotomy and stone extraction in 5, though repeat sphincterotomy was needed in one of them while the 6th patient needed biliary surgery. No calculi were detected in 3 others who presented with cholestasis and cholangitis and could be treated with sphincterotomy followed by ursodeoxycholic acid (UDCA). Interestingly, they also reported 5 patients with abdominal pain and cholestasis who did well on UDCA treatment alone. Since the first report of biliary stenting for choledochal stenosis in 1993,6Lohr J.M. Kuchenreuter S. Grebmeier H. Hahn E.G. Fleig W.E. Compression of the common bile duct due to portal vein thrombosis in polycythemia vera.Hepatology. 1993; 17: 586-592Crossref PubMed Scopus (25) Google Scholar endoscopic management of symptomatic PCC has been reported in at least 87 patients in 17 case series,6Lohr J.M. Kuchenreuter S. Grebmeier H. Hahn E.G. Fleig W.E. Compression of the common bile duct due to portal vein thrombosis in polycythemia vera.Hepatology. 1993; 17: 586-592Crossref PubMed Scopus (25) Google Scholar, 8Bhatia 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, 9Llop E. de Juan C. Seijo S. et al.Portal cholangiopathy: radiological classification and natural history.Gut. 2011; 60: 853-860Crossref PubMed Scopus (61) Google Scholar, 10Oo Y.H. Olliff S. Haydon G. Thorburn D. Symptomatic portal biliopathy: a single centre experience from the UK.Eur J Gastroenterol Hepatol. 2009; 21: 206-213Crossref PubMed Scopus (41) Google Scholar, 11Khare 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 (44) Google Scholar, 12Sezgin O. Oguz D. Altintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 58: 602-608Abstract Full Text Full Text PDF PubMed Scopus (61) Google Scholar, 13Dumortier J. Vaillant E. Boillot O. et al.Diagnosis and treatment of biliary obstruction caused by portal cavernoma.Endoscopy. 2003; 35: 446-450Crossref PubMed Scopus (45) Google Scholar, 22Condat 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 (157) Google Scholar, 24Thervet L. Faulques B. Pissas A. et al.Endoscopic management of obstructive jaundice due to portal cavernoma.Endoscopy. 1993; 25: 423-425Crossref PubMed Scopus (43) Google Scholar, 25Perlemuter G. Bejanin H. Fritsch J. et al.Biliary obstruction caused by portal cavernoma: a study of 8 cases.J Hepatol. 1996; 25: 58-63Abstract Full Text PDF PubMed Scopus (79) Google Scholar, 26Mörk H. Weber P. Schmidt H. Goering R.M. Scheurlen M. Cavernous 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 (33) Google Scholar, 27Solmi L. Rossi A. Conigliaro R. Sassatelli R. Gandolfi L. Endoscopic treatment of a case of obstructive jaundice secondary to portal cavernoma.Ital J Gastroenterol Hepatol. 1998; 30: 202-204PubMed Google Scholar, 28Hernandez I.G. Sandoval M.W. Méndez E.L. Calleros J.H. Tapia A.L. Uribe M. Biliary stricture caused by portal biliopathy: case report and literature review.Ann Hepatol. 2005; 4: 286-288PubMed Google Scholar, 29Ajayi A.O. Chandrasekar T.S.C. Hammed A.H. Portal biliopathy in a 13-year-old Asian girl: a case report and review of literature.Ann Afr Med. 2009; 8: 185-188PubMed Google Scholar, 30Layec S. D'Halluin P.N. Pagenault M. Bretagne J.F. Massive hemobilia during extraction of a covered self-expandable metal stent in a patient with portal hypertensive biliopathy.Gastrointest Endosc. 2009; 70: 555-556Abstract Full Text Full Text PDF PubMed Scopus (22) Google Scholar, 31Cantu P. Bezzio C. Role of a short-term stent-trial in a patient with biliary stricture and portal hypertensive biliopathy: long-term outcome result.Dig Dis Sci. 2011; 56: 1242-1244Crossref PubMed Scopus (2) Google Scholar, 32Costamagna G. Pandolfi M. Mutignani M. Spada C. Perri V. Long-term results of endoscopic management of postoperative bile duct strictures with increasing numbers of stents.Gastrointest Endosc. 2001 Aug; 54: 162-168Abstract Full Text Full Text PDF PubMed Scopus (350) Google Scholar nine of which involved fewer than 4 patients and the largest included 20 patients (Table 2). Results of endoscopic management for biliary stenoses are summarized below.Table 2Endoscopic Treatment for Portal Cavernoma Cholangiopathy.SeriesPCC patientsCholangiogram abnormalitiesEndoscopic treatmentDuration of therapyComplicationsOutcomeBhatia et al 19958Bhatia 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 ScholarSymptomatic PCC—4Stricture + stone—3; CBDS—4ES, extraction, ENBD3 yearsNilSymptoms—resolved; (FU 3–8 months)Condat et al 200322Condat 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 (157) Google ScholarAsymptomatic PCC—18; symptomatic PCC—7Stricture—2; stricture + stone—1; GS—2, CBDS—1Nil; UDCA—4NANAImproved on UDCA—3Sezgin et al 200312Sezgin O. Oguz D. Altintas E. Saritas U. Sahin B. Endoscopic management of biliary obstruction caused by cavernous transformation of the portal vein.Gastrointest Endosc. 2003; 58: 602-608Abstract Full Text Full Text PDF PubMed Scopus (61) Google ScholarSymptomatic PCC—10Stricture (CHD/CBD) 9; intrahepatic stone 1ES, BD, stenting3.3 years (range, 1–7)Hemobilia—1; cholangitis—5, death—1Normal
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