CT appearance of sclerosing cholangitis

Six patients with laryngoceles, two internal and four of the mixed type, were studied with CT. Uncomplicated laryngoceles appear on CT as air-filled structures lying in the paralaryngeal space (internal), lateral neck (external), or in both locations (mixed). Obstruction of the neck of the laryngocele by either tumor or chronic inflammation can result in a fluid-filled structure, producing on CT a well circumscribed mass of either near water or soft-tissue density, depending on its composition. CT proved useful in establishing the definitive diagnosis of a laryngocele and mapping its total extent for treatment planning.     

Inflammatory pseudotumor from sclerosing cholangitis

We report the computed tomographic (CT) findings in a case of primary sclerosing cholangitis. A "mass" demonstrated on CT and ultrasound examination in the region of the common bile duct and extending into the head of the pancreas was shown to be diffuse fibrosis and thickening of the common bile duct at surgery.     

Floxuridine-associated sclerosing cholangitis. A dog model

The authors developed a dog model for the biliary sclerosis that occurs as a severe complication of protracted hepatic arterial floxuridine (FUDR) infusions (using implanted drug delivery systems) in patients with hepatic cancers. Infusaid pumps attached to hepatic arterial catheters were used for protracted infusions in ten mixed breed hounds. To allow repeated cholangiograms, the animals' gallbladders were removed and catheters connected to subcutaneous infusion ports were positioned in the cystic ducts. Five treated dogs received FUDR 0.3 mg/kg/day through the pump for a total of 30 days. Five control dogs received only saline through the pump. Cholangiograms were obtained before and after treatment in all animals. In the control group, serum liver function test results and the cholangiographic appearance of the biliary tree remained within normal limits. By contrast, in the FUDR-treated group, serum glutamic-pyruvic transaminase and alkaline phosphatase progressively rose above normal, starting 2-3 weeks into FUDR infusion, followed by hyperbilirubinemia (7-28 mg/dl peak levels) beginning 4 to 6 weeks after initiation of the drug infusion. Cholangiograms revealed focal strictures involving the central bile ducts (five dogs) and diffuse attenuation of the intrahepatic ducts (four dogs). Thus, the liver function abnormalities and the cholangiographic findings in this dog model mimic the hepatobiliary toxicity in sensitive patients receiving similar treatment.     

Gallbladder disease in patients with primary sclerosing cholangitis

We evaluated the gallbladders of 121 patients who had well-documented primary sclerosing cholangitis. Sonograms, cholangiograms, and CT scans were reviewed, and the findings were correlated with surgical or autopsy findings, when available. Pathologic examination of the gallbladder was available in 55 (45%) of the 121 patients; of these, 49 (89%) had abnormal gallbladders. Ninety-three of the 121 patients had one or more radiologic examinations of the gallbladder: 77 had sonograms, 80 had cholangiograms, and 18 had CT scans. Seventy-five (62%) of the 121 patients had abnormal gallbladders on histologic examination or had positive findings on one or more imaging study. By excluding 25 patients who had histologic changes of borderline significance and/or patients who had thick-walled gallbladders attributable to end-stage liver disease, we concluded that 50 (41%) of the 121 patients had intrinsic abnormalities of the gallbladder. Thirty-two (26%) had gallstones, 18 (15%) had probable primary sclerosing cholangitis involving the gallbladder, and five (4%) had benign or malignant neoplasms. Our study indicates that gallbladder abnormalities are common among patients with primary sclerosing cholangitis, and sonography is the most useful technique for evaluating these conditions.     

Current update on primary and secondary sclerosing cholangitis

Sclerosing cholangitis can be idiopathic (primary) or secondary to an identifiable cause. Irrespective of cause, sclerosing cholangitis usually progresses to end-stage liver disease and warrants orthotopic liver transplantation. Recent studies provide new insights into the etiopathogenesis, natural history, diagnosis, and management of these different entities.     

Iatrogenic sclerosing cholangitis following hepatic arterial chemotherapy infusion

A case of common hepatic duct stricture secondary to hepatic artery chemotherapy infusion is described. CT and endoscopic retrograde cholangiopancreatography (ERCP) may be used in concert to differentiate this entity from other causes of jaundice--namely, hepatic replacement by tumor, porta hepatis adenopathy, and chemotherapy hepatotoxicity.     

原发性硬化性胆管炎的临床及影像诊断

目的：探讨原发性硬化性胆管炎（PSC）的临床及影像诊断。方法：回顾性分析15例PSC患者的临床表现、实验室检查及各种影像学检查特征。结果：15例PSC患者中，男性19例，女性6例，平均年龄19岁。临床表现以进行性黄疸、皮肤瘙痒、乏力、纳差为主，病程0．5～19年。碱性磷酸酶（ALP）Md=280U／L，谷氨酰胺基转肽酶（GGT）Md=300U／L。超声检查主要表现为胆管壁增厚、回声增强，胆管内径变细。CT检查表现为不同程度的肝内外胆管扩张、胆管壁增厚、钙化。核磁共振胰胆管造影术（MRCP）检查表现为肝外胆管狭窄及肝内胆管周围部狭窄伴扩张。内窥镜逆行胰胆管造影术（ERCP）检查13例患者均为阳性，表现为肝内胆管串珠样或剪枝样改变和肝外胆管憩室样凸出。8例纤维结肠镜检查6例合并溃疡性结肠炎。结论：原发性硬化性胆管炎好发于中年男性，临床表现以梗阻性黄疸，血清ALP、GGT明显升高为其显著特点，往往伴有溃疡性结肠炎。超声及CT检查对本病诊断具有提示和辅助诊断作用，确诊以ERCP为主，MRCP可弥补ERCP检查的不足并作为病变随访的有效方法。     

Cholangiocarcinoma complicating primary sclerosing cholangitis: cholangiographic appearances

Cholangiograms from 104 patients (and serial cholangiograms in 66 patients) with primary sclerosing cholangitis (PSC) were reviewed. In 13 patients the additional diagnosis of cholangiocarcinoma was made at biopsy or autopsy. Cholangiograms from patients with both PSC and carcinoma were compared with cholangiograms from patients with PSC alone. Marked dilatation of ducts or ductal segments (100% vs. 24%) and the appearance of a polypoid mass (46% vs. 7%) were common findings in the group of patients whose disease was complicated by malignancy. In the malignant group, polypoid masses were larger, measuring 1 cm or greater in diameter. On serial cholangiograms, four of 15 patients with progressive stricture formation and four of five with progressive ductal dilatation proved to have carcinomas. The frequent occurrence of bile duct carcinoma as a complication of PSC in this group of patients indicates that PSC has a strong tendency to undergo malignant degeneration. Cholangiographic findings which suggest malignant degeneration include markedly dilated ducts or ductal segments, presence of a polypoid mass 1 cm or greater in diameter, and progressive stricture formation or ductal dilatation.     

原发性硬化性胆管炎的影像学研究进展

原发性硬化性胆管炎(PSC)以肝内外胆管进行性炎症、增生和纤维化为主要特征,是自身免疫性肝病的一种.其发病机制不明,预后不佳.影像学检查对于诊断 PSC 具有重要作用,经内镜逆行胰胆管造影(ERCP)及磁共振胆胰管成像(MRCP)是常用的影像诊断技术.就 PSC 影像诊断方面的研究进展作一综述,以期提高对本病的认识.     

原发性硬化性胆管炎13例诊治体会

目的探讨原发性硬化性胆管炎诊断和治疗方法。方法对我院外科1999～2007年经影像学和穿刺活检病理检查的原发性硬化性胆管炎13例患者的诊断、药物治疗结果进行回顾性研究。结果患者均有阻塞性黄疸病史,7例应用了个体化的糖皮质激素治疗方案,6例采用了脱氧熊胆酸（ursodeoxycholicacid UDCA）治疗,无胆道手术的病例及肝移植病例,有4例患者获得长期缓解,而3例进展至胆汁性肝硬化,其中死亡1例。结论原发性硬化性胆管炎发病机制不明,临床表现不典型,药物治疗效果不理想。亦无法阻止病程的发展,肝移植可能是其相对有效的治疗方法。     

Nonoperative dilatation of dominant strictures in primary sclerosing cholangitis

While most cases of primary sclerosing cholangitis are characterized by multifocal bile duct strictures, a few have a localized high-grade stricture (dominant stricture) superimposed on diffuse disease. This dominant stricture may cause jaundice or bacterial cholangitis in some patients. Dominant strictures were percutaneously dilated in 14 patients with primary sclerosing cholangitis and jaundice or bacterial cholangitis. Stricture dilatation produced a significant decrease in the frequency of cholangitis and a significant decrease in serum bilirubin in those with recent onset of jaundice. The only complication was bacteremia or cholangitis in five patients. Three of nine patients with successful dilatations developed recurrent strictures at 6-18 months. Balloon dilatation should be considered the treatment of choice for dominant strictures in symptomatic patients with primary sclerosing cholangitis who have recent onset of jaundice (less than 6 months' duration) or recurrent episodes of bacterial cholangitis.     

Primary sclerosing cholangitis: evaluation with MR cholangiography-a case-control study

PURPOSE: To determine the accuracy of magnetic resonance (MR) cholangiography for detection of primary sclerosing cholangitis (PSC) and localization of PSC in the biliary tract. MATERIALS AND METHODS: In a prospective case-control study involving 102 patients, the MR cholangiograms obtained in 34 patients with PSC established with endoscopic retrograde cholangiopancreatography (ERCP) were compared with the MR cholangiograms obtained in 68 age-matched control patients with hepatobiliary diseases other than PSC. Two abdominal radiologists conducted an independent, blinded random review of the MR cholangiograms to assess for the presence or absence of PSC and determine the location of PSC in the biliary tract, and then compared the findings with those at ERCP. RESULTS: MR cholangiography was found to be accurate in detecting PSC and in defining the extent of disease. In the detection of PSC, the sensitivities were 88% and 85%; specificities, 97% and 92%; positive predictive values, 94% and 85%; and negative predictive values, 94% and 93% for readers 1 and 2, respectively. Interobserver agreement was excellent (kappa = 0.79). In the localization of extrahepatic PSC, the sensitivities were 83% and 89%; and specificities, 83% and 83% for readers 1 and 2, respectively. Interobserver agreement was good (kappa = 0.62). In the localization of intrahepatic PSC, the sensitivity was 87% for both readers; interobserver agreement was good (kappa = 0.71). CONCLUSION: MR cholangiography enables accurate detection and localization of PSC.