Dominant Stenoses Research Articles

Transpapillary Intraductal Ultrasound (IDUS) in the Evaluation of Dominant Bile Duct Stenoses in Patients with Primary Sclerosing Cholangitis

Background & Aims: Primary sclerosing cholangitis (PSC) is associated with the development of cholangiocarcinoma (CC) in approximately 9% of patients. Cholangiography or endoscopic tissue sampling does not reliably allow to distinguish CC from a benign dominant bile duct stenosis. The aim of the present study was to assess the value of intraductal ultrasonography (IDUS) in discriminating between benign and malignant dominant stenoses in PSC patients. Patients & Methods: Fourty PSC patients with dominant bile duct stenoses were prospectively studied. Transpapillary IDUS and endoscopic tissue sampling were performed in addition to endoscopic retrograde cholangiography (ERC). Cholangiography as well as the IDUS findings were classified as malignant or benign by the investigators. Final diagnosis of malignant stenosis was based on positive histology and/or cytology, whereas benign character was assumed in cases of negative tissue sampling and uneventful extended clinical follow-up. Results: Eight PSC patients (20%) had dominant bile duct stenoses caused by CC, whereas 32 of 40 patients (80%) had benign dominant bile duct stenoses. IDUS was significantly superior to ERC for detection of malignancy in terms of sensitivity (87.5% vs 62.5%, p = 0.05), specificity (90.6% vs 53.1%, p < 0.001), accuracy (90% vs 55%, p < 0.001), positive predictive value (70% vs 25%, p < 0.001), and negative predictive value (96.7% vs 85%, p = 0.049). Conclusion: Transpapillary IDUS significantly increases the ability to distinguish malignant from benign dominant bile duct stenoses in patients with PSC.

The role of dominant stenoses in bacterial infections of bile ducts in primary sclerosing cholangitis

Primary sclerosing cholangitis (PSC) is characterized by progressive fibrotic inflammation and strictures of the biliary system. We studied the role of dominant stenoses in bacterial biliary infections and the effect of routine antibiotic administration with cholangiography. A prospective clinical trial without blinding or randomization. The endoscopy unit in a university hospital. Fifty patients with PSC entered and finished the study. A total of 103 endoscopic retrograde cholangiographies (ERC) was performed in 37 PSC patients with dominant stenosis and 13 controls with PSC but no dominant stenosis. After selective cannulation of the bile duct, bile samples were obtained during each procedure. All patients received systemic antibiotic treatment with ciprofloxacin for one week after ERC. Enteric bacteria were detected in the bile specimens of 15 out of 37 PSC patients (40.5%) with dominant stenosis but never in the absence of dominant stenosis (P=0.004). Positive cultures for enteric bacteria were associated with elevated serum C-reactive protein, high leukocyte counts in bile (P<0.05) and the deterioration of liver function assessed by increasing bilirubin levels during the follow-up period lasting a median of 7 months (P=0.06). Despite the high rate of susceptibility in vitro, ciprofloxacin treatment eradicated enteric bacteria in only two out of 12 cases. Bacterial infection of the bile ducts with dominant stenosis is a frequent finding and may play a role in the progression of PSC. Short-course antibiotic treatment is not very effective in eradicating acteria from the bile ducts.

Primary Sclerosing Cholangitis: The Role of Endoscopic Therapy

Primary sclerosing cholangitis is characterized by progressive fibrosing inflammation of the bile ducts leading to their obliteration, resulting in cholestasis, and finally, cirrhosis of the liver. The majority of patients with advanced disease with time develop dominant stenoses of major bile ducts. Such dominant stenoses may be recognized by magnetic resonance cholangiopancreatography with good specificity and sensitivity, but for definite diagnosis and especially for therapeutic decisions, endoscopic retrograde cholangiography still represents the gold standard. Ursodeoxycholic acid treatment does not prevent the development of such stenoses. Endoscopic measures allow the opening of short- and long-segment stenoses of the common bile duct. Short- but not long-segment stenoses of the hepatic ducts near to the bifurcation also may be treated endoscopically. Repeated balloon dilatations over years appear to be the treatment of choice. In patients treated by early endoscopic dilatation of dominant stenoses, survival may be significantly improved compared with the predicted survival.

Impact of Dominant Stenoses on the Serum Level of the Tumor Marker CA19-9 in Patients with Primary Sclerosing Cholangitis

Patients with primary sclerosing cholangitis (PSC) have an increased risk of developing hepatobiliary tumors. The tumor marker CA19-9 was claimed to indicate the occurrence of bile duct carcinoma. This study aimed to assess whether increased serum levels of CA19-9 in PSC patients with dominant stenoses indicate bile duct carcinoma. The study cohort comprised 106 patients treated over a median time of 5.0 years (range 0.5 - 13 years). All patients were treated with ursodeoxycholic acid (UDCA) and whenever they developed dominant stenoses by endoscopic dilatation of these stenoses. In endoscopically treated patients, CA19-9 levels were measured before and 3, 6, 12 and 24 months after endoscopic dilatation. Of the 106 patients, 22 carcinoma-free patients and 3 patients with bile duct carcinoma had elevated CA 19 - 9 levels. In 14 out of 25 patients with elevated CA19-9 levels, dominant stenoses were diagnosed and treated by endoscopic dilatation. In 71.4 % of the endoscopically treated patients, CA19-9 levels decreased following the endoscopic intervention. In PSC patients, increased serum levels of CA19-9 are rarely due to the development of bile duct carcinoma. In patients with dominant stenoses, the relief of biliary obstruction by endoscopic dilatation may lead to a decrease of the serum levels of CA19-9.

Characterization of dominant bile duct stenoses in patients with primary sclerosing cholangitis using cholangioscopy

Background & Aims: Primary sclerosing cholangitis (PSC) is associated with the development of cholangiocarcinoma (CC) in around 9% of patients. Cholangiography or endoscopic tissue sampling does not reliably allow to distinguish CC from a benign dominant bile duct stenosis. The aim of the present study was to assess the value of cholangioscopy in discriminating between benign and malignant dominant stenoses in PSC patients.

Endoscopic Treatment of Dominant Stenoses in Patients With Primary Sclerosing Cholangitis

Primary sclerosing cholangitis is characterized by progressive fibrosing inflammation of the bile ducts, leading to their obliteration, which results in cholestasis and, finally, cirrhosis of the liver. Over time, the majority of patients with advanced disease develop dominant stenoses of major bile ducts. Ursodeoxycholic acid (UDCA) treatment does not prevent the development of such stenoses. Endoscopic measures allow the opening of short- and long-segment stenoses of the common bile duct and also of short segment stenoses of the hepatic ducts. Inpatients treated by early endoscopic dilatation of dominant stenoses, as well as with UDCA,survival may be significantly improved (compared with the predicted survival).

Endoscopic Therapy in Primary Sclerosing Cholangitis (PSC)—Does the Complication Risk Outweigh Therapeutic Benefit?

Endoscopic therapy in PSC has the potential to dilate dominant stenoses and remove concomitant pigment stones with subsequent improvement in cholestasis and secondary bacterial cholangitis. Recent publications suggest a high relative risk to benefit ratio because of chronic bacterial exposure to the biliary tree. Accordingly, we reviewed our 20-year experience using endoscopic therapy to treat symptomatic PSC. Materials and Methods: 110 PSC patients referred to our multi-specialty clinic for symptomatic PSC and treated endoscopically over a 20-year period were reviewed. Data collected included patient demographics, treatment indications, number of ERCPs performed, incidence and type of complications, and whether there was improvement in liver function tests (decreased AST / ALT / bilirubin≥50%, alk phos≥20%) and/or episodes of bacterial cholangitis over the subsequent 6-month period. Results: 110 of 117 patients, mean age 56 years (19-87), referred for symptomatic PSC underwent 393 ERCPs (median 3 [1-32]) over a 20-year period. Data were subsequently analyzed for patients with (80) and without (30) underlying inflammatory bowel disease to define whether active bowel inflammation was associated with changes in outcomes following endotherapy. Data were statistically analyzed using Fisher′s Exact Test. Of the 110 patients, 60 underwent brush cytology, 74 endoscopic sphincterotomy, 40 stone extraction, 42 temporary stent placement, and 51 balloon dilation. Complications were noted in 22 ERCPs (5.6%) and included 1 post-sphincterotomy bleed, 12 cases of pancreatitis, 4 cholangitis exacerbations, 3 duct perforations, 1 sepsis, and 1 liver abscess. LFTs decreased and/or attacks of cholangitis resolved in 63/88 (72%; confidence interval 61% to 81%) evaluable patients with complete follow up after endotherapy. There was no difference in complications (p=0.85), decrease in LFTs (p=0.19), or reduction in cholangitis (p=0.27) in patients with or without underlying IBD. Conclusions: 1. In contrast to recently published papers, endoscopic therapy appears to demonstrate a favorable benefit risk ratio when outcomes to include improvement in LFTs and decrease in bacterial cholangitis are considered. 2. The presence or absence of underlying IBD does not appear to affect response to endotherapy. 3. Whether such therapy is associated with decreased incidence of subsequent cirrhosis, decreased need for liver transplant, or change in mortality remains to be defined.

Development of dominant bile duct stenoses in patients with primary sclerosing cholangitis treated with ursodeoxycholic acid: outcome after endoscopic treatment

Background / Aims : Primary sclerosing cholangitis is characterized by progressive fibrotic inflammation and obliteration of intra- and/or extrahepatic bile ducts. Methods : In a prospective study of 106 patients treated for up to 13 years with ursodeoxycholic acid, the development of major bile duct stenoses and the efficacy of endoscopic measures have been evaluated. Results : Of 106 patients ten had major duct stenoses at entry, and during a median follow-up period of 5.0 years another 43 developed a dominant stenosis. Fifty-two patients with dominant stenoses were treated endoscopically by repeated balloon dilatations and five patients were temporarily stented. Complications of endoscopic procedures were pancreatitis (5.2%), bacterial cholangitis (3.3%) and bile duct perforation (0.5%). Five years after the first dilatation of a dominant stenosis the Kaplan–Meier survival rates free of liver transplantation were 100% in stage 2, 72% in stage 3 and 50% in stage 4 disease. The actuarial survival free of liver transplantation of the whole group at 3, 5 and 7 years were 0.987, 0.935 and 0.891 and the corresponding survival rates predicted with the Mayo multicenter survival model were 0.860, 0.775 and 0.737 ( P <0.001). Conclusions : In advanced disease, occlusion of major bile ducts with time occurs in the majority of patients. Endoscopic opening of dominant stenoses is effective and appears to be a valuable addition to the medical treatment of such patients.

4681 Endoscopic management of psc patients suffering from dominant bile duct strictures.

Introduction: In general, PSC patients are in good condition for many years until progressive bile duct stenoses lead to cholestasis deteriorating physical condition. Extrahepatic bile duct stenoses contributing to symptomatic cholestasis in PSC patients are referred to as dominant stenoses. So far, reported techniques and outcome of endoscopic dilation therapy of dominant stenoses are contradictory. Aim: We estimated the benefit of endoscopic dilation of dominant stenoses in patients with PSC. Methods: Our PSC patient pool of the last 9 years (Nov. 90 - Oct. /99) was studied retrospectively. Dominant stenoses were treated by balloon dilation and bouginage. Stenting of dominant stenoses was only carried out in case of unsuccessfull dilation therapy. Results: 72 patients suffering from symptomatic PSC were investigated by ERC. 40% (29/72) of all PSC patients presented dominant stenoses. The mean age was 40.9±14.2 years, 65% (19/72) were male and 35% (10/72) female. Patients suffering from dominant stenoses were treated by repeated dilation procedures (98 interventions in 29 patients, mean: 3.4 interventions per patient). Endoscopic therapy improved physical condition as well as decreased cholestatic parameters. AST, ALT, AP, gGT and bilirubin (78% from 6.1±6.4 down to 1.4±0.6 mg/dl). 98 interventions were accompanied by 8.2% of complications in terms of post-ERC pancreatitis (2.0%), CBD perforation (3.1%) and post-ERC cholangitis (3.1%). All complications were considered as mild to moderate since all disappeared within a few days following conservative therapy. Discussion: Many PSC patients suffer from extrahepatic dominant bile duct stenoses. Dilation therapy was able to widen dominant stenoses as well as to relieve cholestasis thus improving the general state of health. Conclusively, we recommend to screen symptomatic PSC patients for dominant bile duct stenoses. Dominant bile duct stenoses should be repeatedly treated by endoscopic dilation therapy since most afflicted PSC patients benefit significantly.

Primär sklerosierende Cholangitis – Therapieoptionen

The natural course of primary sclerosing cholangitis (PSC) is highly variable. There is no correlation between the PSC activity and the severity of the bowel disease which occurs simultaneously in more than half of the patients. With this variability in the natural history of the disease, the clinician is faced with difficult decisions regarding the timing of medical and interventional therapy as well as of orthotopic liver transplantation. Immunosuppression is unlikely to be effective in patients with advanced liver disease with an irreversible bile duct Ioss. Ursodeoxycholic acid (UDCA) is now commonly prescribed in PSC since the drug is safe in most patients. High-dose UDCA treatment looked promising in a small pilot study. Therefore this approach probably in combination with immunosuppressive treatment should be investigated in prospective long term controlled trials in patients in the prefibrotic stage. Treatment of the PSC symptoms (itching, steatorrhoea, osteoporosis, bacterial cholangitis) is of paramount importance for the quality of life of the patients. Relapsing cholangitis must be treated aggressively with appropriate antibiotics e. g. ciprofloxacine or mezlocillin plus metronidazole. Balloon dilation and short term stenting will help patients with dominant stenoses of the extrahepatic bile ducts. In late-stage PSC liver transplantation is the only therapeutic option. The 5-year survival rate is good (about 80%). Orthotopic liver transplantation should be performed early in the course of the disease when the operative risk is less. At this stage patients are also less likely to have developed hepatobiliary malignancy. The incidence of colorectal carcinoma in PSC patients with ulcerative colitis is high. Therefore, annual surveillance colonoscopy is recommended in this group of patients.