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Choledochal Cyst (Choledochal Cysts)

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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.

Also Known As:

Choledochal Cysts; Choledochal Cyst, Diverticulum; Choledochal Cyst, Type II; Choledochal Cyst, Type III; Choledochal Cyst, Type IV; Choledochal Cyst, Type V; Choledochal Diverticulum; Choledochocele; Cyst, Common Bile Duct; Cysts, Common Bile Duct; Intrahepatic Choledochal Cyst; Multiple Choledochal Cysts; Choledochal Cyst, Congenital; Choledochal Cyst, Intrahepatic; Choledochal Cyst, Multiple; Choledochal Cysts, Congenital; Choledochal Cysts, Diverticulum; Choledochal Cysts, Intrahepatic; Choledochal Cysts, Multiple; Choledochal Diverticulums; Choledochoceles; Congenital Choledochal Cysts; Cyst, Choledochal; Cyst, Congenital Choledochal; Cyst, Intrahepatic Choledochal; Cyst, Multiple Choledochal; Cysts, Choledochal; Cysts, Congenital Choledochal; Cysts, Intrahepatic Choledochal; Cysts, Multiple Choledochal; Diverticulum Choledochal Cyst; Diverticulum Choledochal Cysts; Diverticulum, Choledochal; Diverticulums, Choledochal; Intrahepatic Choledochal Cysts; Multiple Choledochal Cyst; Choledochal Cyst, Type I; Common Bile Duct Cyst; Congenital Choledochal Cyst

Networked: 392 relevant articles (6 outcomes, 18 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Cysts
2.	Pathologic Constriction (Stenosis)
3.	Wounds and Injuries (Trauma)
4.	Pancreatitis
5.	Biliary Atresia

Experts

1.	Kamisawa, Terumi: 5 articles (01/2011 - 01/2005)
2.	Ono, Shigeru: 4 articles (11/2013 - 06/2008)
3.	Li, Long: 4 articles (07/2013 - 06/2004)
4.	Yamataka, Atsuyuki: 4 articles (02/2010 - 12/2003)
5.	Chen, Chao-Long: 4 articles (01/2010 - 09/2007)
6.	Chuang, Jiin-Haur: 4 articles (01/2010 - 09/2007)
7.	Tsuruta, Kouji: 4 articles (11/2009 - 01/2005)
8.	Egawa, Naoto: 4 articles (11/2009 - 01/2005)
9.	Miyano, Go: 3 articles (05/2015 - 01/2005)
10.	Cowles, Robert A: 3 articles (01/2015 - 07/2009)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Choledochal Cyst:

1.	Amylases FDA Link 05/01/1983 - "Increased renal clearance of amylase in fusiform choledochal cyst--a report of 2 cases." 03/01/1990 - "Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum." 07/01/2015 - "The aim was to compare biliary amylase, common channel, and gall bladder/liver histopathology between spherical and fusiform choledochal cysts. " 07/01/2015 - "Comparative analysis of spherical and fusiform choledochal cyst based on three-dimensional magnetic resonance cholangiopancreatography, biliary amylase, and histopathological examination." 09/01/2012 - "From May 1995 to August 2010, 80 patients under 18 years old who underwent choledochal cyst excision and hepaticojejunostomy and measurements of biliary amylase levels were allocated to a low-amylase-level group (amylase level < 200 U/L, n = 26) and a high-amylase-level group (amylase level > 200 U/L, n = 54). "
2.	chenodeoxycholate sulfate conjugate (CDCS)IBA 07/01/2013 - "The aim of this study was to compare the efficacy of single-incision laparoscopic hepaticojejunostomy (SILH) with that of conventional laparoscopic hepaticojejunostomy (CLH) for children with choledochal cysts (CDCs). " 07/01/2013 - "This study presents our 11-year surgical experience with choledochal cysts (CDC), focusing on the complexity in the diagnosis and management of large-size CDCs. " 10/01/2006 - "The purpose of this analysis is to study the clinical features, liver histology and the outcome after surgery in infants with choledochal cysts (CDCs), and to draw conclusions on the management strategy accordingly. " 10/01/2014 - "Choledochal cysts (CDCs) are believed to represent a risk factor for the development of neoplasia. " 03/01/2012 - "Choledochal cysts (CDCs) are increasingly being diagnosed antenatally. "
3.	phytobacteriomycin (PBM)IBA 12/01/2011 - "The present study shows that flow diversion surgery for congenital choledochal cysts with PBM significantly reduces the risk of subsequent development of malignancy in the biliary tract, and it is vital to choose the appropriate operative procedure to prevent occurrence of these postoperative complications." 12/01/2011 - "Therefore, we sought to review the relationship between operative procedure for types I and IV-A (Todani's classification) congenital choledochal cyst with PBM, and long-term treatment outcome. " 05/01/2011 - "Thus, total resection of the extrahepatic bile duct and hepaticojejunostomy are recommended in children diagnosed with PBM with choledochal cyst. " 01/16/2011 - "PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst) and PBM without biliary dilatation (maximal diameter of the bile duct ≤ 10 mm). " 11/01/2009 - "PBM can be divided into PBM with biliary dilatation (congenital choledochal cyst [CCC]) and PBM without biliary dilatation (maximal diameter of the bile duct <or= 10 mm). "
4.	Messenger RNA (mRNA)IBA 11/01/2008 - "Liver tissues were obtained from 10 patients with early and late stage of BA, 10 patients with choledochal cyst, and 4 normal controls to study upstream stimulatory factor 2 (USF2) messenger RNA (mRNA) and protein expressions. " 07/01/2004 - "Frozen liver tissue obtained from patients with biliary atresia, neonatal giant cell hepatitis, total parenteral nutrition (TPN)-related cholestasis, choledochal cysts, and normal control subjects was used for fluorescent immunohistochemistry studies of cellular infiltrates, cytokine mRNA expression, and in situ hybridization for localization of cytokine-producing cells. " 11/01/2000 - "Immunohistochemical stains of Fas and Fas ligand (FasL) and in situ hybridization of Fas ligand messenger RNA (mRNA) were performed on paraffin-embedded liver specimens of 36 biliary atresia, 6 choledochal cysts, and 14 nontumorous parts of pediatric liver tumors. "
5.	Primary sclerosing cholangitisIBA 06/01/2012 - "Primary sclerosing cholangitis, nitrosamine exposure, choledochal cysts, Clonorchis sinensis and Opisthorchis viverrini represent important risk factors for CCA, although a specific risk factor cannot be identified for many patients. " 10/01/2011 - "Choledochal cyst originating from primary sclerosing cholangitis in a child." 06/01/2007 - "They shared a digital file containing histological pictures of 30 foci of non-invasive neoplastic lesions selected from the biliary system of patients suffering from primary sclerosing cholangitis, choledochal cyst or hepatolithiasis. " 05/01/2004 - "Those patients with underlying risk factors such as primary sclerosing cholangitis (PSC) and choledochal cysts generally present 2 decades earlier. " 02/01/1999 - "We conclude that primary sclerosing cholangitis should be considered when interpreting ERCP films from patients who are supposed to have choledochal cysts type I."
6.	Annular pancreasIBA 01/01/2011 - "Recent studies have shown the advantages of investigating the development of frogs, mice, and chickens for understanding early embryonic development of the pancreas and congenital anomalies, such as choledochal cysts, anomalous pancreaticobiliary junction, annular pancreas, and pancreas divisum. " 07/01/2015 - "Intraoperatively, we found annular pancreas with malrotation and type I choledochal cyst. " 07/01/2015 - "Annular pancreas, type I choledochal cyst and malrotation in a low-birth weight newborn: A case report." 01/01/2007 - "In patients with pancreas divisum (n = 17), pancreaticobiliary maljunction (n = 12), choledochocele (n = 2), and annular pancreas (n = 1) who underwent ERCP and MRCP, the diagnostic accuracy and findings on MRCP were compared with those on ERCP. " 02/01/2004 - "Antenatal diagnosis of a choledochal cyst and annular pancreas."
7.	Carbon MonoxideIBA 12/31/2006 - "The purpose of this study was to investigate and discuss imaging methods and management strategies for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies. " 07/28/2013 - "Choledochocele with pancreas divisum: A rare co-occurrence diagnosed on magnetic resonance cholangiopancreatography." 12/31/2006 - "Management strategy for congenital choledochal cyst with co-existing intrahepatic dilation and aberrant bile duct as well as other complicated biliary anomalies." 06/01/1998 - "Co-existing biliary anomalies and anatomical variants in choledochal cyst." 01/01/2014 - "Spontaneous choledochal cyst perforation in pregnancy with co-existent chronic pancreatitis."
8.	EnzymesIBA 02/01/2001 - "The pathophysiology of choledochal cysts remains unclear, although an association with anomalous pancreato-biliary junction and the reflux of pancreatic enzymes into the biliary tree is known. " 06/01/1995 - "Choledochal cyst is commonly associated with pancreatobiliary maljunction, and high concentrations of pancreatic enzymes in bile are usually observed. " 12/01/1994 - "This disputes the traditional hypothesis of reflux of pancreatic enzymes and supports the recent theory of primary obstruction as the etiologic cause of choledochal cyst. " 07/01/1989 - "Pancreatic enzymes in the bile of congenital choledochal cyst with anomalous pancreaticobiliary ductal union were analyzed. " 07/01/1989 - "[Analysis of pancreatic enzymes in the bile of congenital choledochal cyst with anomalous pancreaticobiliary ductal union]."
9.	Congenital diaphragmatic herniaIBA 06/01/1994 - "Twenty-one patients (oesophageal atresia [5], congenital diaphragmatic hernia [4], exomphalos [4], patent vitellointestinal duct [1], anorectal anomaly [2], choledochal cyst [1], renal cyst [1], ovarian cyst [1], myelomeningocoele [1], and pyloric stenosis [1]) operated on at a median age of 3 days (range, 1 to 24 days) and 12 age-matched controls were included in the study. " 04/01/2007 - "Completed procedures included Nissen fundoplication (n = 25), cholecystectomy (n = 18), Heller myotomy (n = 2), splenectomy (n = 2), Morgagni hernia repair (n = 2), and single cases of complex pyloroplasty in the chest, bowel resection, left Bochdalek congenital diaphragmatic hernia repair, esophageal atresia and tracheoesophageal fistula repair, and choledochal cyst excision. " 07/01/2008 - "The other advanced procedures include laparoscopic-assisted anorectoplasty, surgery for Hirschprung's disease, thoracosocpic decortication, congenital diaphragmatic hernia repair, nephrectomy, fundoplication, etc. Our complications are postoperative fever, bleeding, bile leak following choledochal cyst excision and pneumothorax following bronchogenic cyst excision. " 05/01/2010 - "The procedures included the following: 39 fundoplications; 34 cholecystectomies; 25 gastric bandings; 13 splenectomies; 4 anorectal pull-through operations for imperforate anus; 4 nephrectomies; 4 appendectomies; 4 sympathectomies; 3 choledochal cyst excisions with hepaticojejunostomies; 3 inguinal hernia repairs; two each of the following: liver cyst excision, repair of congenital diaphragmatic hernia, Heller's myotomy, and ovarian cyst excision; and one each of the following: duodeno-duodenostomy, adrenalectomy, and hysterectomy. "
10.	Lipase (Acid Lipase)FDA Link 12/01/2009 - "The majority of biliary amylase and lipase comes from the pancreas in all biliary diseases; pancreaticobiliary reflux is the predominant source in the common bile duct cyst group and duodenal-biliary reflux is responsible for the ductal pigment stone group." 04/01/2014 - "The aim of this study was to examine the dynamic post-operative changes in drain amylase and lipase values after CEHE for choledochal cysts, and then compare these values with the clinical outcomes in order to identify risk factors for post-operative pancreatitis after CEHE. " 04/01/2014 - "High drain amylase and lipase values predict post-operative pancreatitis for choledochal cyst." 05/01/2011 - "We reviewed 29 patients < or = 15 years old with acute pancreatitis, as evidenced by abdominal pain, increased serum amylase and lipase concentrations (>200 IU/L for each), and radiological examination, who underwent surgery for congenital choledochal cysts between 2000 and 2010. "

Therapies and Procedures

1.	Endoscopic Sphincterotomy 02/01/2007 - "Endoscopic sphincterotomy is feasible and effectively treats both lesions even in larger choledochoceles." 01/01/1998 - "Small choledochoceles can be safely managed by endoscopic sphincterotomy." 01/01/1997 - "Additionally, in selected cases, a choledochocele may be effectively managed by endoscopic sphincterotomy. " 01/01/1996 - "In 6 patients with symptomatic or questionably symptomatic choledochoceles, endoscopic treatment in the form of endoscopic papillotomy was carried out. " 03/01/1994 - "Two patients, each with a small choledochocele, were treated successfully by endoscopic sphincterotomy and stone extraction. "
2.	Liver Transplantation 12/27/2012 - "Liver transplantation from a donor with asymptomatic type IV-a choledochal cyst: the long-term postoperative course." 07/01/2008 - "Plasma levels of HGF were determined using an enzyme-linked immunosorbent assay from 19 patients with BA, 10 at the time of Kasai procedure (KP) and 9 at liver transplantation (LT), and 10 patients with nonicteric choledochal cyst served as control. " 09/01/2007 - "Hepatic immunoreactive 8-OHdG expression was investigated during the early stage of BA when the patients received Kasai portoenterostomy (KP), during the late stage when the patients received liver transplantation (LT), in patients with choledochal cyst as disease control, and in patients with histologically normal liver as normal control. " 08/15/2001 - "Living donor with type IV-A choledochal cyst in liver transplantation." 01/01/2010 - "The plasma levels of endotoxin and soluble CD14 were measured with a pyrochrome Limulus amebocyte lysate assay and enzyme-linked immunosorbent assay in patients with early-stage BA when they received the Kasai procedure (KP), in patients who were jaundice-free post-KP and followed-up at the outpatient department, in patients with late-stage BA when they received liver transplantation, and in patients with choledochal cysts. "
3.	Hepatectomy 01/01/2005 - "The prognosis for patients with malignancy complicating choledochal cyst remains poor despite aggressive radical surgery such as Whipple's operation or additional hepatectomy. " 11/01/2000 - "In this report we evaluate the therapeutic benefit of hepatectomy in a patient with a type IV-A choledochal cyst involving the intrahepatic bile ducts." 04/01/2001 - "Subsequently, an obstructed choledochal cyst was diagnosed clinically, and a right hepatectomy followed by a Roux-en-Y loop implantation on the raw surface of the left lobe were performed. " 11/01/2014 - "The records of patients who received extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts from January 2002 to December 2011 were retrospectively reviewed, and surgical outcomes analysed. " 11/01/2014 - "To report the results of combined extrahepatic cyst excision, partial hepatectomy, and Roux-en-Y hepaticojejunostomy for type IV-A choledochal cysts. "
4.	Laparoscopes (Laparoscope) 10/01/1999 - "Laparoscope-assisted minimally invasive treatment for choledochal cyst." 01/01/2015 - "In this case, we present our experiences for the management of choledochal cyst through radical removal combined with Roux-en-Y anastomosis under laparoscope. " 09/07/2004 - "Total cyst excision with Roux-en-Y hepatoenterostomy by ultrasonic scalpel under the laparoscope was effective and safe for choledochal cyst. " 09/07/2004 - "[Total cyst excision with ultrasonic scalpel under the laparoscope for choledochal cyst]."
5.	Total Parenteral Nutrition 07/01/2014 - "It was a cross-sectional study where all the patients suffering from choledochal cyst (CDC), extrahepatic portal venous obstruction (EHPVO), and infantile obstructive cholangiopathy undergoing diagnostic preoperative cholangiogram; and patients with history of total parenteral nutrition (TPN) undergoing surgery for some other condition were included in the study. " 01/01/2004 - "The biliary tree was completely opacified in 5 infants with the following diagnosis: neonatal hepatitis (2), duplication of the gallbladder (1), choledochocele (1), total parenteral nutrition (TPN) cholestasis (1). " 08/01/1994 - "Controls consisted of five patients with various conditions including total parenteral nutrition-induced cholestasis, choledochal cyst, viral hepatitis, metastatic carcinoma, and thrombotic thrombocytopenic purpura. " 10/01/2013 - "Other diagnoses included neonatal hepatitis, total parenteral nutrition cholestasis, Alagille syndrome, cystic fibrosis, choledochal cyst, hypothyroidism, alpha-1 antitrypsin deficiency and persistent cholestasis of unknown etiology. " 11/01/2005 - "The etiologies of cholestasis were idiopathic neonatal hepatitis (INH) 23%, extrahepatic biliary atresia (EHBA) 22.2%, total parenteral nutrition (TPN)-related cholestasis 18.3%, infection 9.9%, endocrine causes 6%, choledochal cyst 5.6%, Down syndrome 4.4%, hemolytic anemia 1.6%, and miscellaneous causes 9.1%, respectively. "