1/239. 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. ( info) |
2/239. Pancreaticobiliary carcinoma associated with a large choledochal cyst: role of MRI and MR cholangiopancreatography in diagnosis and preoperative assessment. The role of magnetic resonance (MR) imaging and MR cholangiopancreatography is demonstrated in a case of pancreaticobiliary carcinoma associated with a large choledochal cyst. The size of the cyst presented considerable difficulty in evaluation with both endoscopic retrograde cholangiopancreatography and computed tomography. ( info) |
| Pulmonary lymphangitis carcinomatosa is an unusual cause of death in a young adult. This case describes an apparently healthy young woman who presented with severe acute pancreatitis, which is a recognized complication of a choledochal cyst. autopsy examination revealed advanced malignancy with poorly differentiated adenocarcinoma penetrating the wall of the choledochal cyst and metastatic adenocarcinoma in the lymph nodes, lungs and kidneys. This case emphasises the unusual presentation of a choledochal cyst with acute pancreatitis and the aggressive nature of malignancy associated with this congenital anomaly. ( info) |
| 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. ( info) |
5/239. Different types of congenital biliary dilatation in dizygotic twins. A pair of dizygotic twins who were both found to have congenital biliary dilatation, but of different types, is reported. This case is of academic interest from an etiologic point of view. ( info) |
6/239. 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. ( info) |
7/239. A case of carcinoma associated with the remained intrapancreatic biliary tract 17 years after the primary excision of a choledochal cyst. Although the incidence of biliary carcinoma associated with choledochal cyst is high, there are extremely rare reports about cancer development in the remaining intrapancreatic biliary tract in patients who underwent primary excision of the choledochal cyst. The authors present a case of carcinoma associated with the remnant intrapancreatic biliary tract in a 39 year-old woman, 17 years after the initial excision of a choledochal cyst with Roux-en-Y hepaticojejunostomy. Long-term follow-up may be recommended even in patients who have undergone excisional surgery for choledochal cyst, because it is possible that cancer associated with the intrapancreatic biliary tract may develop. ( info) |
8/239. 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. ( info) |
9/239. Laparoscope-assisted minimally invasive treatment for choledochal cyst. The principle of treatment of choledochal cysts is total cyst excision with hepaticojejunostomy because of the high rate of associated malignancy of the biliary system. The authors used a minimally invasive laparoscopic procedure to treat a patient with nonmalignant choledochal cyst. Although a large median laparotomy is usually used for cyst excision and hepaticoenterostomy, laparoscope-assisted total cystectomy and hepaticojejunostomy were performed with minimal skin incision. To avoid gas embolism during dissection around the hepatic hilus the surgical procedure was divided into two stages: CO2 insufflation and abdominal lifting without pneumoperitoneum. This combination of procedures was as safe and technically adequate as conventional surgery. No abnormalities were observed in liver function, and the patient could sit up in bed the first day postoperatively. Thirteen days after surgery, he was discharged from the hospital uneventfully. ( info) |
10/239. 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. ( info) |