Endoscopic treatment of biliary duct strictures in sclerosing cholangitis: follow-up assessment of a new therapeutic approach

Endoscopic sphincterotomy was performed on 10 patients with sclerosing cholangitis to improve biliary tract drainage and to remove bile duct sludge and stones. In addition, Gruentzig-type balloons were placed endoscopically to dilate severe biliary duct strictures in eight of these patients, and endoprostheses were inserted to bridge high grade strictures in three patients. In order to assess the effectiveness of endoscopic treatment, we compared the number of hospitalizations for clinical episodes of cholangitis in this patient group for similar periods of time before and after therapy. Episodes of cholangitis requiring hospitalization decreased from 2.5 +/- 0.4 per patient in the 12 months prior to endoscopic therapy to 0.2 +/- 0.2 episodes per patient in the year following treatment and to 0.33 +/- 0.2 episodes per patient in the additional follow-up period during the second year. Liver function tests have improved significantly over the follow-up period of 19.1 +/- 2.6 months from the time of endoscopic treatment. The serum bilirubin decreased from 6.9 +/- 2.0 mg/dl to 2.7 +/- 1.4 mg/dl; serum alkaline phosphatase decreased from 959 +/- 214 IU to 385 +/- 89 IU; and serum transaminase decreased from 117 +/- 17 IU to 77 +/- 12 IU. Endoscopic treatment appears to be effective in patients with severe sclerosing cholangitis.     

Endoscopic treatment of primary sclerosing cholangitis.

BACKGROUND/AIMS: Dominant stricture of an extrahepatic bile duct is responsible for symptoms and an exacerbation of cholestasis in 15-20% of patients with primary sclerosing cholangitis. The aim of this study was to evaluate the efficacy and safety of endoscopic treatment in this selected patient group. METHODS: Retrospectively, we evaluated 16 patients who were treated endoscopically due to elevation of serum biochemical liver tests and symptoms which were attributable to dominant bile duct strictures during the period 1990 to 2003. Symptoms and biochemical liver tests were compared before and after treatment. RESULTS: Sixteen patients underwent a total of 58 therapeutic endoscopic retrograde cholangiopancreatographies (ERCP). Sixteen endoscopic sphincterotomies, 15 balloon dilatations, 6 bougie dilatations, 3 stone/sludge extractions and 8 stentings were performed. Endoscopic therapy was technically successful in all patients (100%). Biochemical liver tests were significantly improved when compared with pretreatment values (p<0.001). Patients have been followed-up without stents except for the patients who had cholangiocarcinoma and cirrhosis at the beginning. Procedure-related early complications occurred in 8.6% of therapeutic endoscopic biliary procedures. There was no mortality due to endoscopic treatment. Two patients whose stents were changed every two to three months had cholangitis due to stenting during 13 stent periods. Four patients whose stents were changed in seven to 10 days developed suppurative cholangitis (total 6 stent periods). CONCLUSIONS: Endoscopic therapy of symptomatic dominant strictures in primary sclerosing cholangitis is safe and effective. The cholangitis seen in long-term stenting seems to be solved by short-term stenting.     

原发性硬化性胆管炎的内镜治疗及预后分析

目的　探讨原发性硬化性胆管炎（primary sclerosing cholangitis,PSC）患者行经内镜逆行胰胆管造影术（endoscopic retrograde cholangiopancreatography,ERCP）的诊疗效果及预后相关因素。方法　纳入2009年5月—2020年5月西京医院接诊的PSC病例。回顾性收集人口学、临床症状、实验室和影像学检查、ERCP诊疗等数据,探讨ERCP治疗人群特点及临床疗效,随访观察疾病进展、无移植生存期和总体生存期等。结果　共纳入74例PSC患者,中位年龄53岁,男性占54.1%（40/74）,合并胆管显性狭窄、炎症性肠病和其他自身免疫性肝病患者分别占32.4%（24/74）、18.9%（14/74）和17.6%（13/74）,接受ERCP患者占36.5%（27/74）。Logistic回归分析显示,高总胆红素（OR=12.33,95%CI：1.24~122.63,P=0.032）和合并胆管显性狭窄（OR=24.67,95%CI：3.40~178.88,P=0.002）是ERCP诊疗的独立危险因素。ERCP操作和临床成功率均为96.3%（26/27）。截至最后一次随访,患者进展为肝硬化、胆管癌、行肝移植及死亡比例分别为9.5%（7/74）、4.1%（3/74）、5.4%（4/74）和18.9%（14/74）。随访患者（n=54）的五年生存率为83.3%。接受ERCP患者与未接受ERCP患者相比,无移植生存期（P=0.933）和总体生存期（P=0.608）差异均无统计学意义。瘙痒患者 （HR=5.30,95%CI：1.50~18.90,P=0.010）无移植生存期更短。结论　PSC患者合并炎症性肠病较少而合并自身免疫性肝病的比例较高。高总胆红素或胆管显性狭窄患者接受ERCP治疗占比更高,其操作及临床成功率满意,但ERCP对长期预后无显著影响。瘙痒患者的无移植生存期较短。     

Biliary tract inflammatory disorders: Primary sclerosing cholangitis and primary biliary cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with endstage liver disease and yields excellent long-term survival in both groups.     

Biliary tract inflammatory disorders: Primary sclerosing cholangitis and primary biliary cirrhosis

Primary sclerosing cholangitis (PSC) and primary biliary cirrhosis (PBC) are chronic progressive cholestatic diseases that frequently lead to biliary cirrhosis. The exact pathogenesis of these diseases remains elusive but is likely immunologically based. Complications range from fatigue and pruritus to end-stage liver disease. The risk of developing hepatocellular carcinoma is low for patients with PBC, whereas cholangiocarcinoma is common in PSC and carries an ominous prognosis. Although ursodeoxycholic acid is effective in slowing the progression of PBC, no effective medical therapy exists for PSC. Liver transplantation is the only option for patients with end-stage liver disease and yields excellent long-term survival in both groups.     

内镜治疗术后胆漏和继发胆管狭窄

目的 探讨内镜治疗手术后并发胆漏和继发胆管狭窄的方法及效果。方法 胆漏患 者均先行内镜下十二指肠乳头切开,行鼻胆管引流术,继续保留原有胆道、腹腔引流。待胆道、腹腔引 流停止1-2周证实胆漏愈合后拔管,伴有胆道狭窄的患者在拔除鼻胆引流管后置入塑料内支架,持 续扩张2-3个月。结果 22例胆漏患者鼻胆引流3-4周后胆漏处均闭合,13例胆管狭窄置入内支 架者,10例支架取出后狭窄解除,2例合并肝总管狭窄者经重新置入双支架3个月后效果良好,1例 左肝管狭窄伴结石者,再置入单支架,术后仍有胆道感染症状反复出现。结论 内镜治疗可列为手术 后胆漏或继发胆管狭窄治疗的首选方法。     

Endoscopic therapy of sclerosing cholangitis

The efficacy of endoscopic treatment in primary sclerosing cholangitis has not been clearly established. This report presents endoscopic intervention in 53 consecutive patients with this disorder. Pertinent data were abstracted from the GI-TRAC database, medical records, and cholangiograms, and clinical follow-up was obtained by telephone interview of the subjects. Assessed treatment outcomes were clinical symptom, liver function test, and cholangiographic appearance. Between 1986 and 1993, 85 patients with primary sclerosing cholangitis underwent successful ERCP, of which 36 men and 17 women underwent 100 therapeutic endoscopic procedures. Forty-three of 50 dilations, 37 of 38 stentings, 8 of 8 nasobiliary tube placements, and 11 of 17 stone extractions were technically successful. These treatments were complicated by cholangitis or pancreatitis in 15 patients. Clinical follow-up was obtained in 50 of 53 patients who had undergone 85 procedures (median follow-up of 31 months): 28 patients felt better, 21 felt the same, and 1 felt worse. Liver function tests obtained within 3 months of the endoscopic treatment were significantly improved compared with pretreatment values (P < .001). Cholangiograms showed improvement in 36% of the patients, no change in 51%, and the effect of therapy could not be assessed in 13%. Overall, 41 of 53 patients (77%) had improvements of their clinical symptoms, liver function tests, or cholangiograms.     

Dominant strictures in patients with primary sclerosing cholangitis

BACKGROUND: Repeat endoscopic dilatations of dominant strictures (DS) have been reported to be of benefit in patients with primary sclerosing cholangitis (PSC). We aimed to determine the prevalence of DS in patients with PSC and the spontaneous course of ALP and bilirubin, up to a year from diagnosis in patients with and without DS. METHOD: Cholangiographies from 125 patients with PSC were reevaluated. DS was defined as a stenosis < or =1.5 mm in diameter of the common bile duct (CBD) and/or < or = 1.0 mm of right (RHD) or left hepatic duct (LHD). RESULTS: A dominant stricture in common bile duct and/or right hepatic duct or left hepatic duct was present in 56 out of 125 (45%) patients. Mean values for alkaline phosphatase were 16 and 15.2 microkat/L and bilirubin values were 42 and 35 micromol/L before cholangiography in patients with and without DS, respectively (NS). The change in ALP and bilirubin observed from the precholangiographic value up to 2 and 12 months afterward was not significantly different in those with and without DS. CONCLUSIONS: Cholestasis in patients with PSC does not seem to be related to the presence of DS. Endoscopic therapy of DS should not be routinely undertaken and randomized studies are needed to clarify its potential benefits.     

Benign biliary strictures: Endoscopic management

Over the past 2 decades, endoscopic retrograde cholangiopancreatography with stricture dilation and stent placement has gradually become the first-line treatment modality for the vast majority of benign biliary strictures (BBSs). Stricture remediation with progressive placement of multiple plastic stents with 3 months interval stent exchange during a period of one year has excellent long-term results in patients with postoperative BBSs. Covered self-expandable metal stents (SEMS) are a reasonable alternative to multiple plastic stenting, especially in patients with chronic pancreatitis. The use of covered SEMS should be limited to carefully selected cases of postoperative BBSs. Uncovered SEMS are contraindicated for any type of BBSs. Understanding of the etiology and exclusion of malignancy is essential for optimal treatment in some types of biliary strictures, especially in the case of autoimmune cholangiopathy and primary sclerosing cholangitis.     

Endoscopic stenting for biliary strictures.

A consensus is growing among units that have an experience in both endoscopic and percutaneous stenting techniques that the endoscopic approach of malignant biliary strictures is more comfortable for the patient and provides less complications. This article describes endoscopic biliary drainage in different malignant stenosis of the bile ducts and delineates the respective indications of percutaneous and endoscopic techniques together with the possible combination of these two methods in selected cases. It also tackles the question of the medical surgical approach of the patients, which might, thanks to a better selection, reduce the morbidity and mortality associated with surgery. The indications of biliary stenting in benign strictures, namely post operative or chronic pancreatitis associated biliary stenoses, are also discussed. Recently, new materials became available for endoscopic and percutaneous biliary drainage, and particularly metallic self expanding stents which might provide a better palliation among these patients. If these stents fulfill their promise on longer follow-up, they may replace the conventional stenting devices.     

Endoscopic removal of Ascaris lumbricoides from the biliary tract as emergency treatment for acute suppurative cholangitis

Ascariasis is the most common intestinal helminthiasis worldwide. Heavily infected individuals are prone to develop bowel obstruction or perforation as well as biliary disease. Nevertheless, the presence of roundworms in the biliary tree outside endemic areas is very uncommon. The migration of these worms to the biliary system can cause biliary colic, pancreatitis, or even acute suppurative cholangitis with hepatic abscesses and septicemia.We report here on 2 infants with 14 and 15 months and a 9-year-old boy who suffered from massive biliary ascariasis and who presented with acute suppurative cholangitis. All cases were successfully treated by endoscopic retrograde cholangiopancreatography with worm extraction and adjuvant medical therapy.Physicians should be aware of ascariasis in patients with pancreatobiliary symptoms who have traveled to endemic areas or in immigrants from these areas.     

Endoscopic management of primary sclerosing cholangitis

Introduction: Primary sclerosing cholangitis (PSC) is a rare but clinically important cholestatic liver disease. Histopathologically and cholangiographically, PSC is characterized by intra- and/or extra-hepatic bile duct inflammation and fibro-obliteration, which ultimately leads to biliary cirrhosis and related sequelae, including development of hepatobiliary and colorectal carcinomata. PSC can be diagnosed at essentially any age and carries a median survival of 15–20 years, regardless of age at diagnosis, and is a foremost risk factor for cholangiocarcinoma. Given the chronic and progressive nature of PSC, its inherent association with both neoplastic and non-neoplastic biliary tract complications, and the lack of effective pharmacotherapies, alimentary and biliary tract endoscopy plays a major role in the care of patients with PSC.

Areas covered: Here, we provide a narrative review on endoscopic management of PSC, including established and evolving applications to the diagnosis and treatment of both its benign and malignant complications.

Expert commentary: Due to the rarity of PSC and the considerable patient-years required to rigorously study major endpoints, there remains a paucity of high-quality evidence regarding its management. As the advanced endoscopic repertoire expands, so has the interest in developing best practices in PSC, which we discuss herein.     

Endoscopic treatment of biliary tract fistulas

Endoscopic therapy was attempted in 24 patients with spontaneous or postoperative persistent biliary fistulas. Endoscopic retrograde cholangiography demonstrated the site of the fistula in 22 cases. Sphincterotomy or biliary stent placement resulted in rapid resolution of the fistula in 16 of 24 patients. Failures were attributed to exclusion of the injured intrahepatic bile duct in two cases, insufficient dilation of a bile duct stricture in one, the large size of the bile duct defect in two, and associated lesions in three (cirrhosis, arterial trauma, subhepatic abscess). Endoscopic management of biliary fistulae requires: (1) visualization of the location of the fistula by retrograde cholangiography especially in case of an intrahepatic lesion, (2) prior percutaneous drainage of associated subhepatic or subphrenic abscesses, and (3) appropriate relief of distal biliary obstruction in order to reduce the intraductal biliary pressure. The outcome is uncertain when endoprostheses are used to bridge large bile duct defects.     

Medical treatment of primary biliary cirrhosis and primary sclerosing cholangitis.

Treatment of primary biliary cirrhosis (PBC) and primary sclerosing cholangitis (PSC) with ursodeoxycholic acid (UDCA) has been in common use since 1985. In PBC, treatment with UDCA improves laboratory data, liver histology, enables a longer transplantation-free interval and prolongs disease survival. Because UDCA is unable to cure the disease newer drugs or combination therapies are still needed. Studies with UDCA and immunosuppressants such as prednisone, budesonide and azathioprine have shown that in selected patients combination therapy may be superior to UDCA monotherapy. PSC is treated successfully with UDCA and endoscopic dilatation of the bile duct strictures. Treatment of extrahepatic manifestations of cholestatic liver disease such as pruritus, fatigue, osteoporosis and steatorrhea can be problematic and time-consuming.     

Endoscopic therapy in primary sclerosing cholangitis: outcome of treatment and risk of cancer

BACKGROUND/AIMS: Primary sclerosing cholangitis is a cholestatic liver disease characterized by multifocal strictures in the intra- and extrahepatic biliary tree. Dominant strictures may arise in the extrahepatic bile ducts, and in these circumstances, endoscopic therapy has been introduced to relieve cholestasis and perhaps also delay the development of liver cirrhosis. The experience of endoscopic treatment at this point in time is limited and the long-term benefit is not clear. Neoplastic transformation in primary sclerosing cholangitis is unpredictable, which is illustrated in the present study along with an evaluation of the efficacy of endoscopic treatment. METHODOLOGY: Endoscopic retrograde cholangiopancreatography was performed in 25 patients with primary sclerosing cholangitis. In 15 there were dominant strictures in the hilum of the liver and/or the distal bile duct and these patients were treated by dilation and/or endoprostheses. Four patients in the treatment group had just cholestatic biochemical test results and 11 were symptomatic. RESULTS: Endoscopic therapy was technically successful in all 15 patients. In 43 sessions, 5 patients were treated by dilation, 2 with endoprostheses, and 8 by both methods. Improvement was achieved radiologically in 12 patients, clinically in 8, and according to liver function tests in 7. Therapy was complicated by cholangitis in 5 patients. Complications were mild and there was no mortality related to the procedure. However, 6 patients in the treatment group died, 5 of cholangiocarcinoma and 1 of colon cancer. CONCLUSIONS: Endoscopic therapy in primary sclerosing cholangitis is indicated in selected patients. The cancer incidence is high, not least in patients with deteriorating disease. It is important to find techniques for identifying patients at risk in order to perform liver transplantation before malignant transformation.