Choledochal Cyst: A congenital anatomic malformation of a bile duct, including cystic dilatation of the extrahepatic bile duct or the large intrahepatic bile duct. Classification is based on the site and type of dilatation. Type I is most common.Jejunostomy: Surgical formation of an opening through the ABDOMINAL WALL into the JEJUNUM, usually for enteral hyperalimentation.Cysts: Any fluid-filled closed cavity or sac that is lined by an EPITHELIUM. Cysts can be of normal, abnormal, non-neoplastic, or neoplastic tissues.Common Bile Duct Diseases: Diseases of the COMMON BILE DUCT including the AMPULLA OF VATER and the SPHINCTER OF ODDI.Cholangiopancreatography, Magnetic Resonance: Non-invasive diagnostic technique for visualizing the PANCREATIC DUCTS and BILE DUCTS without the use of injected CONTRAST MEDIA or x-ray. MRI scans provide excellent sensitivity for duct dilatation, biliary stricture, and intraductal abnormalities.Anastomosis, Roux-en-Y: A Y-shaped surgical anastomosis of any part of the digestive system which includes the small intestine as the eventual drainage site.Common Bile Duct: The largest bile duct. It is formed by the junction of the CYSTIC DUCT and the COMMON HEPATIC DUCT.Hepatic Duct, Common: Predominantly extrahepatic bile duct which is formed by the junction of the right and left hepatic ducts, which are predominantly intrahepatic, and, in turn, joins the cystic duct to form the common bile duct.Biliary Tract Surgical Procedures: Any surgical procedure performed on the biliary tract.Duodenostomy: Surgical formation of an opening into the DUODENUM.Cholangiography: An imaging test of the BILIARY TRACT in which a contrast dye (RADIOPAQUE MEDIA) is injected into the BILE DUCT and x-ray pictures are taken.Imino AcidsCholangiopancreatography, Endoscopic Retrograde: Fiberoptic endoscopy designed for duodenal observation and cannulation of VATER'S AMPULLA, in order to visualize the pancreatic and biliary duct system by retrograde injection of contrast media. Endoscopic (Vater) papillotomy (SPHINCTEROTOMY, ENDOSCOPIC) may be performed during this procedure.Bile Ducts, Extrahepatic: Passages external to the liver for the conveyance of bile. These include the COMMON BILE DUCT and the common hepatic duct (HEPATIC DUCT, COMMON).Biliary Atresia: Progressive destruction or the absence of all or part of the extrahepatic BILE DUCTS, resulting in the complete obstruction of BILE flow. Usually, biliary atresia is found in infants and accounts for one third of the neonatal cholestatic JAUNDICE.Adenomyoma: A benign neoplasm of muscle (usually smooth muscle) with glandular elements. It occurs most frequently in the uterus and uterine ligaments. (Stedman, 25th ed)Dilatation, Pathologic: The condition of an anatomical structure's being dilated beyond normal dimensions.Pancreatic Ducts: Ducts that collect PANCREATIC JUICE from the PANCREAS and supply it to the DUODENUM.Cyst Fluid: Liquid material found in epithelial-lined closed cavities or sacs.Biliary Tract Diseases: Diseases in any part of the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Biliary Tract: The BILE DUCTS and the GALLBLADDER.Pancreatic Diseases: Pathological processes of the PANCREAS.LymphangitisTechnetium Tc 99m Disofenin: A radiopharmaceutical used extensively in cholescintigraphy for the evaluation of hepatobiliary diseases. (From Int Jrnl Rad Appl Inst 1992;43(9):1061-4)Biliary Fistula: Abnormal passage in any organ of the biliary tract or between biliary organs and other organs.Bile Ducts: The channels that collect and transport the bile secretion from the BILE CANALICULI, the smallest branch of the BILIARY TRACT in the LIVER, through the bile ductules, the bile ducts out the liver, and to the GALLBLADDER for storage.Gallbladder: A storage reservoir for BILE secretion. Gallbladder allows the delivery of bile acids at a high concentration and in a controlled manner, via the CYSTIC DUCT to the DUODENUM, for degradation of dietary lipid.Portoenterostomy, Hepatic: Operation for biliary atresia by anastomosis of the bile ducts into the jejunum or duodenum.Enterostomy: Creation of an artificial external opening or fistula in the intestines.Ovarian Cysts: General term for CYSTS and cystic diseases of the OVARY.Common Bile Duct Neoplasms: Tumor or cancer of the COMMON BILE DUCT including the AMPULLA OF VATER and the SPHINCTER OF ODDI.Bile Duct Neoplasms: Tumors or cancer of the BILE DUCTS.Epidermal Cyst: Intradermal or subcutaneous saclike structure, the wall of which is stratified epithelium containing keratohyalin granules.Pancreaticojejunostomy: Surgical anastomosis of the pancreatic duct, or the divided end of the transected pancreas, with the jejunum. (Dorland, 28th ed)Pancreatitis, Chronic: INFLAMMATION of the PANCREAS that is characterized by recurring or persistent ABDOMINAL PAIN with or without STEATORRHEA or DIABETES MELLITUS. It is characterized by the irregular destruction of the pancreatic parenchyma which may be focal, segmental, or diffuse.Pancreatic Juice: The fluid containing digestive enzymes secreted by the pancreas in response to food in the duodenum.Biliary Tract Neoplasms: Tumors or cancer in the BILIARY TRACT including the BILE DUCTS and the GALLBLADDER.Rupture, Spontaneous: Tear or break of an organ, vessel or other soft part of the body, occurring in the absence of external force.Encyclopedias as Topic: Works containing information articles on subjects in every field of knowledge, usually arranged in alphabetical order, or a similar work limited to a special field or subject. (From The ALA Glossary of Library and Information Science, 1983)Omentum: A double-layered fold of peritoneum that attaches the STOMACH to other organs in the ABDOMINAL CAVITY.Laparoscopy: A procedure in which a laparoscope (LAPAROSCOPES) is inserted through a small incision near the navel to examine the abdominal and pelvic organs in the PERITONEAL CAVITY. If appropriate, biopsy or surgery can be performed during laparoscopy.Bile Ducts, Intrahepatic: Passages within the liver for the conveyance of bile. Includes right and left hepatic ducts even though these may join outside the liver to form the common hepatic duct.Retrospective Studies: Studies used to test etiologic hypotheses in which inferences about an exposure to putative causal factors are derived from data relating to characteristics of persons under study or to events or experiences in their past. The essential feature is that some of the persons under study have the disease or outcome of interest and their characteristics are compared with those of unaffected persons.Treatment Outcome: Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series.Copyright: It is a form of protection provided by law. In the United States this protection is granted to authors of original works of authorship, including literary, dramatic, musical, artistic, and certain other intellectual works. This protection is available to both published and unpublished works. (from Circular of the United States Copyright Office, 6/30/2008)ArchivesEquipment Reuse: Further or repeated use of equipment, instruments, devices, or materials. It includes additional use regardless of the original intent of the producer as to disposability or durability. It does not include the repeated use of fluids or solutions.

Pulmonary lymphangitis carcinomatosa and acute pancreatitis: a rare presentation of choledochal cyst. (1/107)

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)

Choledochal cyst associated with polycystic kidney disease: report of a case. (2/107)

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)

Dilatation of the biliary tree in children: sonographic diagnosis and its clinical significance. (3/107)

We evaluated sonographically 162 children who met the criteria for biliary tract dilatation in the past 18 years. Of these, 131 patients were diagnosed as having anomalous dilatations of the biliary tree (including 112 with choledochal cysts and 19 with biliary duct dilatation and biliary atresia). Biliary tract dilatations in the other 31 patients were due to secondary causes or normal variants. All cases of intrahepatic biliary tree dilatation and those with both intra- and extrabiliary duct dilatations were anomalous. In 117 cases of extrahepatic biliary tract dilatation only, the mean diameter was widest in cases of choledochal cyst (21.4 +/- 12.1 mm, compared with cases of biliary tract dilatation with biliary atresia (10 +/- 2.4 mm), secondary biliary duct dilatation (8.5 +/- 1.5 mm), and normal variants (4.4 +/- 1.2 mm) (P < 0.001). Of the 43 infants with biliary tree dilatation, 24 (56%) had choledochal cysts and 19 (44%) had biliary tract dilatation associated with biliary atresia. Excluding cases associated with biliary atresia, the accuracy of diagnosing choledochal cysts in extrahepatic biliary tract dilatation was 71% and 97% using cutoffs of 7 mm and 10 mm as the minimum diameter, respectively.  (+info)

Congenital choledochal cyst with pancreatitis. (4/107)

OBJECTIVE: To understand the relationship among congenital choledochal cyst, anomalous junction of pancreaticobiliary duct (AJPBD) and pancreatitis. METHODS: 25 children with choledochal cyst treated in our hospital were included in this study. Their ages ranged from 2 months to 14 years. Intraoperative cholangiography was performed in 24 children. Pancreatic samples obtained from the head and body of the pancreas at operation were observed under light and electron microscope. Serum amylase was analyzed one week before and after operation respectively. Bile amylase in the cyst and gallbladder was measured at operation. RESULTS: The incidence of AJPBD was 70%. Inflammatory cell infiltration and fibrous hyperplasia were seen in the pancreatic specimens of 5 children, of whom 4 had AJPBD. No ultrastructural changes were found in 2 children without AJPBD, while different changes were seen in 6 with AJPBD, including those showing no pathological changes under light microscope. All the changes became more severe as the age of the patient increased. The bile and serum amylase levels were higher in children with AJPBD than those without AJPBD (P < 0.05). CONCLUSION: Congenital choledochal cyst has a close relationship with AJPBD and pancreatitis. Pancreatic pathological changes have a long and chronic course from ultrastructural changes to macroscopic changes for the developing of pancreatitis.  (+info)

Diagnosis and treatment of congenital choledochal cyst: 20 years' experience in China. (5/107)

AIM: To summarize the experience of diagnosis and treatment of congenital choledochal cyst in the past 20 years (1980-2000). METHODS: The clinical data of 108 patients admitted from 1980 to 2000 were analyzed retrospectively. RESULTS: Abdominal pain,jaundice and abdominal mass were presented in most child cases. Clinical symptoms in adult cases were non-specific, resulting in delayed diagnosis frequently. Fifty-seven patients (52.7%) had coexistent pancreatiobiliary disease. Carcinoma of the biliary duct occurred in 18 patients (16.6%). Ultrasonic examination was undertaken in 94 cases, ERCP performed in 46 cases and CT in 71 cases. All of the cases were correctly diagnosed before operation. Abnormal pancreatobiliary duct junction was found in 39 patients. Before 1985 the diagnosis and classification of congenital choledochal cyst were established by ultrasonography preoperatively and confirmed during operation, the main procedures were internal drainage by cyst enterostomy. After 1985, the diagnosis was established by ERCP and CT, and cystectomy with Roux-en-Y hepaticojejunostomy was the conventional procedures.In 1994, we reported a new and simplified operative procedure in order to reduce the risk of choledochal cyst malignancy. Postoperative complication was mainly retrograde infection of biliary tract, which could be controlled by the administration of antibiotics, there was no perioperative mortality. CONCLUSION: The concept in diagnosis and treatment of congenital choledochal cyst has obviously been changed greatly.CT and ERCP were of great help in the classification of the disease.Currently, cystectomy with Roux-en-Y hepaticojejunostomy is strongly recommended as the choice for patients with type I and type IV cysts. Piggyback orthotopic liver transplantation is indicated in type V cysts (Caroli's disease) with frequently recurrent cholangitis.  (+info)

Proliferative activity of bile from congenital choledochal cyst patients. (6/107)

AIM: To explore the potential carcinogenicity of bile from congenital choledochal cyst (CCC) patients and the mechanism of the carcinogenesis in congenital choledochal cyst patients. METHODS: 20 bile samples from congenital choledochal cyst patients and 10 normal control bile samples were used for this study. The proliferative effect of bile was measured by using Methabenzthiazuron (MTT) assay; Cell cycle and apoptosis were analyzed by using flow cytometry (FCM), and the PGE(2) levels in the supernatant of cultured cholangiocarcinoma cells were quantitated by enzyme-linked immunoabsorbent assay (ELISA). RESULTS: CCC bile could significantly promote the proliferation of human cholangiocarcinoma QBC939 cells compared with normal bile (P=0.001) and negative control group (P=0.002), and the proliferative effect of CCC bile could be abolished by addition of cyclooxygenase-2 specific inhibitor celecoxib (20 microM). The QBC939 cells proliferative index was increased significantly after treated with 1 % bile from CCC patient (P=0.008) for 24 h, the percentage of S phase (29.48+/-3.27)% was increased remarkably (P<0.001) compared with normal bile (11.72+/-2.70) %, and the percentage of G0/G1 phase (54.19+/-9.46) % was decreased remarkably (P=0.042) compared with normal bile (69.16+/-10.88) %, however, bile from CCC patient had no significant influence on apoptosis of QBC939 cells (P=0.719). CONCLUSION: Bile from congenital choledochal cyst patients can promote the proliferation of human cholangiocarcinoma QBC939 cells via COX-2 and PGE(2) pathway.  (+info)

Congenital choledochal dilatation with emphasis on pathophysiology of the biliary tract. (7/107)

Of 37 patients with congenital choledochal dilatation, aged 8 days to 12 years, who had undergone excision with Roux-en-Y hepaticojejunostomy, 26 patients could be analyzed for morphologic abnormalities and pathophysiology of the biliary tract. Of the 26 patients with congenital choledochal dilatation, 25 (96.2%) had an abnormal choledochopancreaticoductal junction. Of the 12 patients with cystic-type choledochal dilatation, 10 had the C-P type of abnormal choledochopancreaticoductal junction, and of the 13 patients with fusiform-type choledochal dilatation, nine had the P-C type. The amylase levels in the choledochal cyst and the gallbladder were elevated regardless of the form of choledochal dilatation. An adenocarcinoma in a cystic choledochal dilatation was found in one child. Therefore, longstanding inflammation of the biliary tract caused by the reflux of pancreatic juice might be one of the factors in carcinogenesis in the biliary tract. This free reflux of pancreatic juice was demonstrated not only by amylase levels in the biliary tract but also by intraoperative biliary manometry. This reflux might be explained by the lack of sphincter function at the junction of the common bile and pancreatic ducts.  (+info)

Procedures for congenital choledochal cysts and curative effect analysis in adults. (8/107)

OBJECTIVE: To evaluate the procedures and timing of operation as well as long-term postoperative effect of congenital choledochal cysts (CCC) in adults. METHODS: The procedures and timing of operation, effective rate, re-operation rate and incidence of carcinoma after operation for 70 adult patients with CCC from January 1980 to June 1999 were analyzed retrospectively. RESULTS: The re-operation rate of external drainage was 86% (6/7). The effective rate of internal drainage was significantly lower than that of cyst resection (3/10 vs 45/49, chi2=20.94, P<0.001). The re-operation rate and incidence of carcinoma of internal drainage were higher than those of cyst resection (5/10 vs 3/49, chi2=13.64, P<0.001 and 3/10 vs 3/49, chi2=5.18, P<0.025). The reoperation rate of emergency surgery was higher than that of selective operation (8/10 vs 6/56, chi2=24.37, P<0.001). CONCLUSIONS: External drainage should be the first-aid measure and the therapy of choice on emergency basis. Internal drainage should never be attempted. Cyst resection with Roux-en-Y hepaticojejunostomy is recommended as the treatment of choice in selective operation.  (+info)

  • A recent article published in Journal of Surgery suggested that choledochal cysts could also be treated with single-incision laparoscopic hepaticojejunostomy with comparable results and less scarring. (
  • Biliary reconstruction can be performed with a Roux-en-Y hepaticojejunostomy as high as possible, near the hilum of the liver. (
  • US scanning is an excellent choice for initial imaging, but it does have limitations, including the fact that its effectiveness is dependent on operator experience, that cysts on US images may be misinterpreted as the gallbladder or other structures, and that US scanning suffers decreased sensitivity in the presence of overlying bowel gas, pancreatitis, cholangitis, or other inflammatory processes. (
  • Frequently, adults with choledochal cysts complain of vague pain in the upper middle region or right upper quadrant and can develop jaundice or cholangitis. (
  • These procedures left the cyst behind, and the free reflux of pancreatic enzymes into the cyst via the anomalous pancreaticobiliary junction resulted in a high incidence of calculi, recurrent cholangitis, anastomotic strictures, and carcinoma arising from the cyst. (
  • [ 53 , 54 ] Recurrent cholangitis and chronic inflammation in the remaining cyst eventually produces metaplasia that leads to malignant transformation. (
  • In older patients with repeated cholangitis and marked pericystic inflammation, this disease may be best managed with resection of the anterolateral part of the cyst followed by an endocystic resection of the lining, leaving the back wall adjacent to the portal vein in place, as reported by Lilly in 1977. (
  • Pathology: Cyst with marked acute and chronic inflammation, fibrosis and focal ossification. (
  • 2012. Gonzales KD, Lee H. Chapter 106: Choledochal Cyst [Prenatal Diagnosis]. (
  • Complete cyst excision without compromising the pancreatic duct and common pancreaticobiliary channel should be performed as soon as the diagnosis is made to reduce the risk of malignancy later in life. (
  • Ramollissement cysts arise in compact tissue when the tissue softens (with hemorrhage, inflammation, necrosis) in a limited section (for example, a brain cyst, after infarction or hemorrhage). (
  • A number of imaging modalities can be used to detect a choledochal cyst, such as ultrasonography, CT, MR cholangiopancreatography (MRCP), endoscopic retrograde cholangiopancreatography, and percutaneous transhepatic cholangiography. (