Cases reported "Choledochal Cyst"

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1/115. Primary sclerosing cholangitis mimicking choledocal cyst type 1 in a young patient.

    A choledochal cyst type I was diagnosed in a 12-year-old boy in 1984. The diagnosis was made using ultrasound and confirmed using computed tomography (CT) and endoscopic retrograde cholangiopancreatography (ERCP). Instead of the usual surgical treatment, endoscopic balloon dilatation of the sphincter of oddi and the distal common bile duct was carried out using an endoscopic procedure. The patient experienced relief of symptoms, gained weight and felt healthy again. An ERCP performed in 1990, because of increasing levels of liver enzymes and clinical features of abdominal pain and fatigue, revealed typical cholangiographic findings associated with primary sclerosing cholangitis, including bile duct irregularities with diffuse narrowing and twisting of the bile ducts with localized ectatic and strictured areas. Percutaneous needle liver biopsy confirmed the diagnosis. We conclude that primary sclerosing cholangitis should be considered when interpreting ERCP films from patients who are supposed to have choledochal cysts type I.
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2/115. choledochal cyst associated with polycystic kidney disease: report of a case.

    We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48-year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.
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3/115. A rare malformation of the pancreaticobiliary junction long common channel choledochal cyst and pancreas divisum in a patient with pancreatic cancer.

    Congenital variants of the pancreaticobiliary junction are rare anomalies that are usually diagnosed in childhood because of recurrent abdominal pain and jaundice. These lesions are associated with several pancreaticobiliary diseases including pancreatitis and malignancy. We observed a rare anomaly of the pancreaticobiliary tract with a combination of several ductal malformations, i.e., choledochal cyst, long common channel and incomplete pancreas divisum in a patient with pancreatic cancer.
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4/115. Heterotopic pancreatic tissue associated with intra- and extrahepatic choledochal cysts.

    A case report of heterotopic pancreas in intra- and extrahepatic biliary tracts in a 36-year-old female who suffered from intra- and extrahepatic choledochal cysts with an anomalous pancreatobiliary duct system. Histologic examination of the resected specimen showed pancreatic tissues located along the wall of the biliary tract with choledochal cysts. The pancreatic tissue consisted of acinar cells and duct elements without Langerhans' islets; the acinar cells were positive immunohistochemically for alpha-amylase and negative for endocrine hormones. Ultrastructural study revealed zymogen granules in the acinar cells. In the present case the heterotopic exocrine pancreatic tissue seems to be etiologically related to choledochal cysts as well as to the anomalous arrangement of the pancreatobiliary duct.
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5/115. Incomplete pancreas divisum with anomalous choledochopancreatic duct junction with choledochal cyst.

    The coexistence of incomplete pancreas divisum, an anomalous choledochopancreatic duct junction, and a choledochal cyst is an extremely rare condition, described in only 3 patients in the available medical literature. The symptoms may be similar to any of these 3 distinct pathological conditions. Magnetic resonance cholangiopancreatography or endoscopic retrograde cholangiopancreatography is usually required for diagnosis. Management of symptomatic pancreas divisum may be accomplished with open accessory duct sphincteroplasty or endoscopic papillotomy with or without stenting. Treatment of choledochal cyst is by complete excision of the cyst whenever possible, with hepaticodochoenterostomy being the treatment of choice. Here, we describe a patient with this complex disorder who was successfully managed with endoscopic minor duct papillotomy with accessory pancreatic duct stenting and resection of the choledochal cyst with hepaticodochojejunostomy.
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6/115. Large choledochocoele: difficulties in radiological diagnosis.

    BACKGROUND: A choledochocoele is a dilatation of the intramural portion of the distal common bile duct within the duodenal wall. It usually presents with recurrent crises of right upper quadrant pain, hyperamylasaemia or jaundice. MATERIALS AND methods: We present a 12-year-old boy with a choledochocoele that was missed on several US examinations despite its large size. RESULTS: Findings on US, IV cholangiography, upper gastrointestinal contrast studies, ERCP and surgery are shown. The histological findings as well as the usual differential diagnosis are discussed.
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7/115. risk of bile duct carcinogenesis after excision of extrahepatic bile ducts in pancreaticobiliary maljunction.

    BACKGROUND: A reflux of pancreatic juice into the biliary tract caused by pancreaticobiliary maljunction (PBM) has been considered important in the development of biliary tract carcinogenesis in choledochal cysts. We excised extrahepatic bile ducts in patients with choledochal cysts to terminate the reflux of pancreatic juice. We investigated whether this surgery could stop the development of the residual bile duct carcinoma. methods: Fifty-six patients with a diagnosis of PBM with choledochal dilatation underwent surgical excision of extrahepatic bile ducts. We applied a person-year method to compare the relative risks (observed number/expected number) of biliary tract carcinoma before and after surgery. RESULTS: In 3 patients, bile duct carcinoma developed in residual dilated segments 19 years 6 months, 8 years 8 months, and 2 years 5 months, respectively, after surgery. Although the relative risk in the post-surgery group was slightly decreased by surgery, it was still high compared with that of the general population. CONCLUSIONS: The incidence of bile duct carcinoma is still high, even after excision of extrahepatic bile ducts in PBM patients with choledochal dilatation. For these patients, careful long-term follow-up is necessary, especially after operations that leave the dilated bile ducts, such as cases of Todani's type IV-A.
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8/115. Choledochocele demonstrated by computed tomographic cholangiography: report of a case.

    Choledochocele is a rare cystic congenital malformation of the distal common bile duct, the precise pathogenesis of which remains unclear. It is usually diagnosed in adulthood after many examinations for unclear digestive symptoms, as in the patient whose case is described herein. Of all the diagnostic techniques available, endoscopic retrograde cholangiography (ERCP) seems to be the best; however, it is an invasive procedure associated with some morbidity. Spiral computed tomographic cholangiography with three-dimensional reconstruction is also an interesting screening technique. Malignant transformation of a choledochocele occurs very rarely, compared with other cystic malformations of the biliary tract. If a choledochocele is small and symptomatic, the lesion can be treated by endoscopic sphincterotomy, but larger cysts are more effectively removed by a surgical cystoduodenostomy. We present herein the case of a 25-year-old woman admitted to our hospital in March 1998 with upper right quadrant abdominal pain caused by a choledochocele, who was successfully treated by a cystoduodenostomy.
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9/115. Choledochocele imaged with magnetic resonance cholangiography.

    Choledochal cysts are rare developmental malformations of the biliary tree. Percutaneous and endoscopic ultrasound, as well as endoscopic retrograde cholangiopancreatography, are recommended diagnostic tools. Magnetic resonance cholangiography may also contribute to the workup and treatment plan of patients with choledochal cysts. We report a 25-yr-old white woman with episodic nausea and moderate epigastric discomfort. Magnetic resonance cholangiography showed a rather spherical, cyst-like, fluid-filled structure in continuity with the prepapillary segment of the common bile duct, thus making a choledochocele (type III choledochal cyst) likely. The patient was treated by endoscopic sphincterotomy and, after a 6-month follow-up, is without complaints.
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10/115. Cystic dilatation of common bile duct. Case report and review.

    The cystic dilatation of the common bile duct is a rare disease in europe and in the USA; even rarer in adults. In fact less than 30% of cases are described in the over 20's. There are some reports in the elderly. When observed in adults the diagnosis is usually due to the onset of symptoms of associated pathologies or to complications such as cholangitis or cancer. Ultrasound, CT and ERCP allow, in the majority of cases, an adequate pre-operative characterization of the lesion. Intra-operative cholangiography verifies the completeness of surgery--always resective--and excludes residual biliary pathologies. The treatment in patients who already underwent conservative surgery is more complex, in fact, in symptomatic patients a second observation and resection is mandatory while in asymptomatic patients a careful and complete follow-up is adequate without underestimating the cancer risk. Septic and inflammatory complications, especially if chronic or recurring, can damage the liver; here resection or liver transplantation can be necessary. The case here reported is a 24-year-old woman with recurring cholangitis for at least 2 years in which US, CT and ERCP showed a Todani's type I cystic dilatation of the CBD. The total excision of the cyst with hepatic-jejunal anastomosis was carried out. A two-year follow up demonstrated no symptoms and normal laboratory findings; cholangioscintigraphy showed a good hepatic-biliary and anastomotic function.
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