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Background and aims: Information about the natural history of small duct primary sclerosing cholangitis (SDPSC) remains scant despite literature suggesting that it constitutes 6-16% of all cases of primary sclerosing cholangitis (PSC). We combined clinical data on SDPSC cases from two tertiary care institutions with liver transplantation programs with the aim of studying the natural history of SDPSC. Methods: Medical records of 25 individuals with SDPSC were reviewed. Diagnosis of SDPSC was based on liver biopsy findings consistent with PSC, a normal cholangiogram, and elimination of known causes of secondary sclerosing cholangitis. Demographic information, symptoms, past medical history, laboratory values, and histologic data were evaluated. Our primary outcome measure was liver transplantation or death. Secondary outcome measures included evidence of end-stage liver disease, development of cholangiocarcinoma, and/or the development of classic PSC on a repeat cholangiogram. Results: Data on 25 individuals (13 males, 12 females; mean age 40 ± 15 years) diagnosed with SDPSC were analyzed. Upon presentation, 11 patients had symptoms including abdominal pain, fatigue, and pruritus. Inflammatory bowel disease was present in 14 patients (56%) at diagnosis. On initial liver biopsy, 60% had early-stage disease (I or II) and none had cirrhosis. On follow-up (1-168 months, median 17 months), malignancy or progression to classic large duct PSC was not noted. Two (8%) patients had evidence of varices and one of the two also developed ascites; one of these patients underwent liver transplantation and the other one died due to sepsis. Conclusions: SDPSC, a mild disease at presentation typically runs a benign course and likely is not an early stage of classic PSC. Further studies with a control group of classic PSC and longer follow-up are needed to study the natural history of SDPSC. [ABSTRACT FROM AUTHOR]

Introduction: Multiple gallbladders represent a rare congenital disorder, and coexistence with carcinoma is extremely rare, leading to a high possibility of misdiagnosis and surgical complications. In this study, a case was reported and the literature was reviewed. Case presentation: An 80-year-old woman was diagnosed with acute cholecystitis via ultrasonography and was successfully treated with antibiotics. After the patient's biliary colic relapsed, she was referred to our hospital. Multiple imaging modalities revealed duplication of her gallbladder (H-type) and suggested coexistence with carcinoma. According to preoperative evaluations, we assumed the patient had stage IIIA disease, and cholecystectomy, cholangiography using a near-infrared ray vision system, and sectionectomy of segments 4a and 5 were performed. Contrary to the high standardized uptake values obtained by 18F-fluoro-2-deoxy-D-glucose positron emission tomography, gallbladder carcinoma was pathologically diagnosed as stage 0 mucosal cancer. Seven days after the operation, portal thrombosis of the posterior branch was revealed, and conservative therapy was indicated; satisfactory results were achieved. The patient was discharged 65 days after surgery. No recurrence was observed for 1 year after surgery. Conclusions: An extremely rare case of malignancy in a duplicated gallbladder was reported, and the literature was reviewed. Accurate estimations are feasible for diagnoses of multiple gallbladders, where correct evaluations are vital, especially in malignant cases. Because of the possibility of malignancy, resected accessory gallbladders should be scrutinized pathologically. [ABSTRACT FROM AUTHOR]

Spontaneous perforation of extrahepatic bile duct is rare. The cause is idiopathic once trauma and choledochal cyst are ruled out. The condition presents a diagnostic dilemma. Preoperative recognition is necessary as early surgical intervention gives excellent prognosis. We report clinical observations made in three cases with acute presentations. Diagnosis is to be suspected by the presence of jaundice after an initial anicteric period of good health with biliary ascites. This is confirmed by bilious abdominal paracentesis, signs of peritonitis and absent free gas on x ray. The constellation of these three findings was constant in three patients. The presented paper highlights the same as reliable clues to diagnosis. [ABSTRACT FROM AUTHOR]