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Choledochocele is defined as a congenital dilatation of the distal intramural part of the common bile duct protruding into the wall of the descending duodenum, typically without pancreaticobiliary maljunction. However, some cases present with a similar pathophysiology to pancreaticobiliary maljunction, including reciprocal reflux of pancreatic juices and bile, leading to protein plugs, pancreatitis, and biliary tract carcinogenesis. Choledochocele is relatively rare and its anatomy, physiology, pathology, and clinical features are thus not well known. We describe a patient with choledochocele who suffered from repeated severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy, in whom the pathological findings of choledochocele showed hyperplasia. [ABSTRACT FROM AUTHOR]

According to Todani's classification, a choledochocele is a cystic dilatation of the distal section of the common bile duct that protrudes into the duodenal lumen. It is also known as a type III choledochal cyst. There are not many cases, and the cause is still unknown. Typically, it is misdiagnosed as a peptic ulcer, as was the case with the patient in this case study. For choledochocele diagnosis, multislice spiral computed tomography and magnetic resonance cholangiopancreatography may be equivalent to endoscopic retrograde cholangiography. Both endoscopic therapy and open surgical care are risk-free possibilities, and the cyst's size influences which strategy should be used. A 35-year-old woman admitted to the hospital with upper abdominal pain caused by a large choledochocele was successfully treated by open surgical management. In this case study, we outline the specifics of her situation and talk about the most recent research on similar cases and how to treat them therapeutically. There is ongoing debate regarding the best course of action. However, to achieve a successful outcome, open surgical care for choledochocele of large size (ie, > 3 cm in diameter) is advised.

Background: Congenital biliary tract dilatation is a common malformation in children and is classified into several types. Choledochocele is a rare type of choledochal cyst that is still debated today. Its etiology and treatment are distinct from those of the other types. We present a case of choledochocele complicated with choledocholithiasis and acute pancreatitis. Case presentation: Here we report the case of an eight-year-old boy who presented a cholangitis. Radiological assessments showed dilatation of extrahepatic and intrahepatic bile ducts with dilatation of the terminal part of the terminal bile duct protruding into the duodenal lumen and microlithiasis into extrahepatic ducts. The patient presented acute pancreatitis that recovered well, then he was operated on. The diagnosis of choledochocele was confirmed with cholangiography. A partial resection of the cyst and marsupialization were done with an open procedure. Conclusion: Choledochocele is a rare type of choledochal cyst that has the lowest risk of developing cholangiocarcinoma. The optimum treatment is partial resection or endoscopic unroofing. [ABSTRACT FROM AUTHOR]

Abstract Background Type III choledochal cysts (CCs) are the rarest and least well-characterized of the five variants of biliary cysts. Only a few relevant studies have been conducted and a gold standard treatment for type III CCs is still lacking because of their scarcity. An unusual case of type III CC with choledochocele at the end of the distal common bile duct (CBD) with no bulging of the duodenum is presented here. Case presentation A 61-year-old woman presented to our department with repeated upper abdominal pain for one year. Laboratory examination revealed abnormal liver function and a slight increase in the white blood cell (WBC) count and proportion of neutrophils. In an MRCP of the patient, the distal CBD was found to have a cystic structure indicative of a type III CC. Endoscopic retrograde cholangiopancreatograpy (ERCP) revealed cystic findings compatible with Todani type III CCs. However, duodenoscopy did not show a bulge in the duodenum so laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy were performed. In her 6-month follow-up, the patient reported that recovery from the operation was uneventful. Conclusions ERCP has become the gold standard for diagnosing type III CCs. Type III CCs can be treated minimally invasively with laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy.

A choledochocele is a rare congenital abnormality of the extrahepatic bile duct characterised by localised dilatation of the distal common bile duct within the wall of the duodenum. We present two children with a long history of undiagnosed abdominal pain and subtle signs of cholestasis. Specific investigations included magnetic resonance cholangiopancreatography (MRCP) that led to a possible diagnosis of choledochocele, and then confirmatory cholangiograms (ERCP and open). Both children required intervention to unroof the dilatation allowing improved pancreato-biliary drainage and complete resolution of symptoms.Elephants have no gallbladder, instead they have a localised dilatation of the distal common bile duct within the wall of the duodenum – analogous to the situation described here.

Background: Type III choledochal cysts (CCs) are the rarest and least well-characterized of the five variants of biliary cysts. Only a few relevant studies have been conducted and a gold standard treatment for type III CCs is still lacking because of their scarcity. An unusual case of type III CC with choledochocele at the end of the distal common bile duct (CBD) with no bulging of the duodenum is presented here.Case Presentation: A 61-year-old woman presented to our department with repeated upper abdominal pain for one year. Laboratory examination revealed abnormal liver function and a slight increase in the white blood cell (WBC) count and proportion of neutrophils. In an MRCP of the patient, the distal CBD was found to have a cystic structure indicative of a type III CC. Endoscopic retrograde cholangiopancreatograpy (ERCP) revealed cystic findings compatible with Todani type III CCs. However, duodenoscopy did not show a bulge in the duodenum so laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy were performed. In her 6-month follow-up, the patient reported that recovery from the operation was uneventful.Conclusions: ERCP has become the gold standard for diagnosing type III CCs. Type III CCs can be treated minimally invasively with laparoscopic cholecystectomy and Roux-en-Y hepaticojejunostomy. [ABSTRACT FROM AUTHOR]

Choledochocele is a cystic dilation of the distal segment of the common bile duct protruding into the duodenal lumen. Which is an extremely rare congenital lesion. Here we report the case of a 2-year-old girl with a choledochocele complicated with choledocho lithiasis and acute pancreatitis who was treated by transduodenal cyst resection with sphincteroplasty. The patient had an uneventful clinical course for one year after the procedure. Very few reports have been published on this condition in infants.

Choledochal cysts are an uncommon anomaly of unknown etiology of the bilious system. This anomaly, characterized by cystic dilatations on intrahepatic or extrahepatic bile ducts, can be seen at any age from birth. Most rare congenital bile duct cysts choledochocele (type III) is usually diagnosed in adults. Since the congenital choledochal cyst has not a unique clinical finding, the basic criteria for diagnosis are based on imaging findings. This article presents a case of choledochoceleaccompanying.recurrent pancreatitis in a 19-year-old male patient.

Abstract Introduction Duplication cyst of the alimentary tract is a rare congenital anomaly. Duodenal duplication cyst accounts for less than 5% overall. These entities rarely present in adults. They are often mistaken as choledochoceles. Management is most often complete excision, but it is individualized to the particular case. Case presentation A 22-year-old woman was admitted to our hospital with a history of intermittent colicky right hypochondrial pain not relieved by any medications for the past 3 months. Initially, she was given proton pump inhibitors, but her pain was not relieved. Further evaluation was done, and preoperative imaging showed a cyst in the second part of the duodenum. Magnetic resonance imaging revealed it as a choledochocele, but duodenal duplication cyst was kept in the differential diagnosis. Further ultrasound identified it to be a duplication cyst. After failed endotreatment, the patient was successfully managed with partial excision and marsupialization. Conclusion Duodenal duplication cyst is uncommon and rarely diagnosed in adults. Duplications in the duodenum should always be a part of the differential diagnosis, especially in cystic lesions. Ultrasonogram of the cyst might lead to the proper diagnosis. Surgery is the treatment of choice if endotherapy is not successful.

• The article represents an extremely rare case of the combination of choledochocele and extra-hepatic bile duct duplication. • The combination of choledochocele and bile duct duplication might escalate the pancreatitis and cholangitis progression. • The pathogenesis in relation to pancreaticobiliary maljunction is discussed. Combination of choledochocele and extra-hepatic duct duplication is an extremely rare congenital abnormality. The patient was an 81-year-old Japanese man. He visited the emergency room for severe abdominal colic pain. He was diagnosed with severe pancreatitis with cholelithiasis and treated conservatively by percutaneous trans-hepatic gallbladder drainage (PTGBD) for 4 months. Thereafter, he was transferred to our institute and cholangiography was performed via the PTGBD tube, revealing cholecysto- and choledocho-lithiasis. The cystic-duct joined the right hepatic duct with extra-hepatic bile duct duplication and the terminal bile duct flowed into the cystic papilla of Vater. The main pancreatic duct also joined into the cystic papilla. These observations confirmed choledochocele with extra-hepatic bile duct duplication. Surgical exploration was performed, and hepatico-jejunostomy with hepatic-ductplasty and cholecystectomy with choledocholithotomy were carried out. He was discharged and his course was uneventful. A very rare combined case of choledochocele with bile duct duplication, which would escalate the pancreatitis and cholangitis, was successfully treated. Their pathogeneses in relation to pancreaticobiliary maljunction is discussed. [ABSTRACT FROM AUTHOR]

Choledochal malformations (CMs) represent a spectrum of relatively rare and complex congenital anomalies, characterized by abnormal dilatation of the biliary tract in the absence of any acute obstruction. Today, almost 20% of CMs can be detected in-utero using maternal ultrasonography. Formal scientometric analysis was used to identify where modern CM research is taking place and perhaps where our attention should be directed in the future. Thus, this article offers a comprehensive review of recent scientific advances relating to CMs including the current understanding of etiology and classification, whilst also discussing key controversies such as risk of malignant transformation and the role of newer modalities of surgical treatment. Although laparoscopic excision of CMs and biliary reconstruction is nowadays feasible and safe, care should be taken before dispensing with standard open techniques, which have minimal complication rates and proven long-term benefit. [ABSTRACT FROM AUTHOR]

Biliary tract cysts are a group of rare congenital diseases that have been classified by Todani in 8 types. Hepaticojejunostomy has been the preferred intervention for Type I and IV biliary cysts. It has been postulated that, due to the low incidence of cancerization of Types II and III biliary cysts, a less invasive approach could be suggested, namely cyst resection in Type II, and endoscopic sphincterotomy with opening of choledochocele in small (

Choledochal cysts are rare congenital anomalies of the biliary tree which may progress to obstruction or malignancy. Of the five Todani variants, choledochocele, or type III choledochal cyst is the rarest. In this case report, we describe a previously healthy 10-year old female who presented with a choledochocele and was treated by near-total excision with transposition of the common channel, resulting in an extended sphincteroplasty.

Choledochocele (type 3 choleduct cyst) is rarely seen in children. Here we report the case of a 5-year-old female who complained of abdominal pain for 2 years. A choledochocele with a diameter of 31 mm localized on the second part of the duodenum, in the periampullary region, was detected. Upper gastrointestinal endoscopy revealed a mass starting from the second part of the duodenum and constricting the duodenal lumen. A 2-cm-long incision was made at the apex of the cyst using a needle tip sphincterectome and three 5- to 6-mm stones were extracted. There were no postoperative complications.

Abstract A 6-year-old girl presented with intermittent abdominal pain, without jaundice and a palpable mass in the epigastrium. Preoperative imaging and upper endoscopy suggested duodenal duplication. During surgery, the patient was diagnosed with a rare type of choledochal cyst—choledochocele (type 3b). The authors emphasize that, in children, choledochocele should be included in the differential diagnosis of cystic lesions located in the duodenal area and the head of the pancreas area, regardless of jaundice or abnormal liver function. Since mucosal histology showing duodenal mucosa did not match the final diagnosis, we suggest that three criteria should be met for the diagnosis of a choledochocele to be diagnosed: (1) a cyst protruding into the duodenal lumen; (2) filling with contrast during cholangiography and (3) a filling defect on X-ray barium meal.

Copyright of Pediatrie pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract Choledochocele is a congenital abnormality of the biliary system, which characterized by a cystic dilatation of intramural segment of the distal common bile duct. Choledochocele manifests as cystic lesions in the duodenal lumen and resembles duodenal duplication cysts in imaging. We reported a patient with choledochocele. Magnetic resonance cholangiopancreatography showed a thin-walled sac in the duodenal cavity and a fine tubular structure connecting with the end of the common bile duct. Magnetic resonance cholangiopancreatography is a noninvasive and convenient technique in the diagnosis of choledochocele. [ABSTRACT FROM AUTHOR]

Cystic dilation of the bile ducts (CDBD) is a rare disease in which pain is the major symptom. It mainly affects young women. Carcinogenesis is the main risk of this disease. Complete surgical resection is the treatment of choice. TODANI's classification defines five groups of congenital cystic dilatation of the bile ducts. Choledochocele is classified as type III and is defined as an isolated cystic dilation of the ampulla of Vater. Few studies were published in the literature due to its rarity and to its multitude of clinical presentations. The lack of guidelines resulted in approaches to treatment based on endoscopic treatment, making rapid progress but considered as a conservative option, or on hardly obtainable complete surgical resection. This study reports the case of a 32-year old female patient presenting with cyclic epigastric pain. Morpho-clinical examinations showed holedochocele. Complete resection of the cyst via duodenotomy with reimplantation of the common bile duct and of the Wirsung duct associated with cholecystectomy were performed. [ABSTRACT FROM AUTHOR]

Choledochal cysts are rare congenital disorders first described by Vater and Ezler in 1723. Their exact etiology remains incompletely understood; however, an anomalous pancreaticobiliary union (APBDU) and subsequent reflux of biliary contents into the biliary tree are thought to play a role. Accordingly, APBDU-associated choledochal cyst patients are significantly more likely to have evidence of hepatitis, cholangitis or pancreatitis and pathologically confirmed inflammation. In 1977, Todani and colleagues modified the original Alonso-Lej classification to include five types of CC. Type I and IV are the most common and most likely to be associated with malignancy. The majority of choledochal cysts are diagnosed in childhood. Clinical presentation varies and most often consists of nonspecific abdominal pain. Diagnosis is typically accomplished using multimodality imaging techniques including computed tomography, magnetic resonance imaging, ultrasound and MRCP. The use of diagnostic PTC and ERCP in CC has been largely replaced by MRCP. Appropriate management consists of prompt, complete cyst excision followed by restoration of biliary enteric continuity when necessary. Minimally invasive CC resection in the pediatric population has demonstrated acceptable outcomes. Prognosis is generally excellent; however, malignancy risk remains higher than the general population even after complete surgical excision. [ABSTRACT FROM AUTHOR]

Background Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly. Methods PubMed and Cochrane databases were accessed 1975–2015 using terms “choledochocele” or “choledochal cyst”. Studies reviewed that met the following criteria: English language, published 1975–2015 with human subjects. Results 325 patients with a choledochocele were identified, including 71 case reports and 254 cases within institutional reviews. 13 pediatric case reports of choledochocele exist, with abdominal pain being the most common symptom (n = 11). The most frequent diagnostic and treatment modalities were ultrasound (n = 10), and endoscopic sphincterotomy (n = 5). No malignancies were reported. 58 adult case reports exist, with the most common presenting symptom being abdominal pain (n = 54). Ultrasound was the frequently employed diagnostic modality (n = 32). Open procedures were performed more often (n = 30). Malignant lesions were identified in 5. In 42 institutional reviews, the frequency of choledochocele was 0.7%. Of those for whom treatment was reported, 69% underwent endoscopic sphincterotomy. Conclusion Choledochocele is a rare malformation. Similarities exist between pediatric and adult patients, but malignancy has only been reported in adults. An algorithm based on patient age, cyst size, lining and amenability to endoscopic resection may be considered as a treatment strategy for this uncommon condition. [ABSTRACT FROM AUTHOR]

Recurrent acute pancreatitis can be caused due to mechanical, metabolic, anatomical abnormalities and other miscellaneous causes. Among the anatomical causes, choledochoceles are rare entities that result in recurrent acute pancreatitis. Choledochoceles are different in morphological and clinical features as compared to other choledochal cysts. We present a case of 21-year-old female with recurrent episodes of acute pancreatitis due to choledochocele as etiology. She was managed with endoscopic sphincterotomy and was asymptomatic for last 24 months.

Choledochal cysts are rare congenital anomalies of the biliary tree which may progress to obstruction or malignancy. Of the five Todani variants, choledochocele, or type III choledochal cyst is the rarest. In this case report, we describe a previously healthy 10-year old female who presented with a choledochocele and was treated by near-total excision with transposition of the common channel, resulting in an extended sphincteroplasty. [ABSTRACT FROM AUTHOR]

Choledochocele in a premature neonate who presented with obstructive jaundice and was managed surgically is reported. At 1-year follow-up, the child is asymptomatic.

A choledochocele is a rare congenital abnormality of the extrahepatic bile duct characterised by localised dilatation of the distal common bile duct within the wall of the duodenum. We present two children with a long history of undiagnosed abdominal pain and subtle signs of cholestasis. Specific investigations included magnetic resonance cholangiopancreatography (MRCP) that led to a possible diagnosis of choledochocele, and then confirmatory cholangiograms (ERCP and open). Both children required intervention to unroof the dilatation allowing improved pancreato-biliary drainage and complete resolution of symptoms. Elephants have no gallbladder, instead they have a localised dilatation of the distal common bile duct within the wall of the duodenum – analogous to the situation described here. • Choledochoceles may have non-specific symptoms making them difficult to suspect. • Cholangiogram is the gold standard to confirm the diagnosis. • Drainage of the choledochocele into the duodenum resolves symptoms and can be done endoscopically or surgically. • Biopsy should always be taken as a malignancy risk exists, at least in adults. [ABSTRACT FROM AUTHOR]

Choledochocele (type 3 choleduct cyst) is rarely seen in children. Here we report the case of a 5-year-old female who complained of abdominal pain for 2 years. A choledochocele with a diameter of 31 mm localized on the second part of the duodenum, in the periampullary region, was detected. Upper gastrointestinal endoscopy revealed a mass starting from the second part of the duodenum and constricting the duodenal lumen. A 2-cm-long incision was made at the apex of the cyst using a needle tip sphincterectome and three 5- to 6-mm stones were extracted. There were no postoperative complications. [ABSTRACT FROM AUTHOR]

Background: Robotic surgery offers three-dimensional visualization and precision of movement that could be of great value to hepatobiliary surgeons. Previous reports of robotic choledochocele resections in adults have detailed extracorporeal jejunojejunostomies. We describe a total robotic excision of a choledochal cyst with hepaticojejunostomy and intracorporeal Roux-en-Y anastomosis. Methods: A 58-year-old woman underwent a robotic excision of a small choledochocele with hepaticojejunostomy and intracorporeal Roux-en-Y. Result: Port placement was determined via collaborative surgical discussion and previously reported robotic right hepatectomies. Total operative time was 386 min and total robot working time was 330 min. The hepaticojejunostomy was performed using 5-0 PDS suture with parachute-style back wall and running front wall sutures. The jejunojejunostomy was a stapled anastomosis. Estimated blood loss was less than 100 mL. The patient was ambulating and tolerating oral intake on post-operative day 1, and was discharged home on post-operative day 2. Conclusions: Robotic resection of choledochal cyst with intracorporeal Roux-en-Y anastomosis is feasible, with advantages over open surgery such as superior visualization, precision, and post-operative patient recovery. [ABSTRACT FROM AUTHOR]

Choledochocele is a cystic dilation of the distal segment of the common bile duct protruding into the duodenal lumen. Which is an extremely rare congenital lesion. Here we report the case of a 2-year-old girl with a choledochocele complicated with choledocho lithiasis and acute pancreatitis who was treated by transduodenal cyst resection with sphincteroplasty. The patient had an uneventful clinical course for one year after the procedure. Very few reports have been published on this condition in infants. • Two years old girl presented with abdominal, pain, and fever. • A choledochocele of 2cm complicated by choletlithiasis and acute pancreatitis. • Choledochocele classified as type A2 according to Sarris et al. • An open surgery with transduodenal cyst resection and sphincteroplasty. • A pathology examination of the specimen found an intestinal mucosa. [ABSTRACT FROM AUTHOR]

Enteric duplication cysts are rare congenital anomalies of unclear etiology. While they can occur anywhere in the gastrointestinal tract, they typically occur in the ileum or ileocecal region and very rarely in the duodenum. Here, we report a case of a periampullary duodenal duplication cyst in a 13-year-old male who presented with clinical and laboratory evidence of small bowel obstruction, hepatitis, and pancreatitis. Based on radiologic imaging, the patient was thought to have a type III choledochal cyst (choledochocele) within the duodenal lumen. Intraoperative findings and postoperative pathological evaluation, however, revealed that the lesion was a duodenal duplication cyst masquerading as a choledochal cyst. Interestingly, the duplication cyst was communicating with the common bile duct, simultaneously causing biliary and small bowel obstruction. [ABSTRACT FROM AUTHOR]

Copyright of Journal Africain D'Hépato-Gastroentérologie is the property of Lavoisier and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Abstract: Purpose: Annular pancreas (AP) is usually associated with duodenal obstruction in neonates. Pancreatitis with AP occurs frequently in adults but is rare in children. This article describes pancreatitis in children with AP and pancreatobiliary anomalies and its surgical treatment. Patients and Methods: Six children who underwent duodenal bypass for AP subsequently developed recurrent pancreatitis. Three had trisomy 21. Duodenoduodenostomy had been performed in 5 patients and gastrojejunostomy in 1 patient for neonatal duodenal obstruction. We reviewed overall management, imaging, and surgical treatment in these children. Results: All children subsequently complained of recurrent abdominal pain. Pancreatitis developed in 6 children, and magnetic resonance cholangiopancreatography and endoscopic retrograde cholangiopancreatography (ERCP) revealed associated pancreatobiliary anomalies such as pancreas divisum, pancreatobiliary malunion, choledochocele, and intraluminal duodenal diverticulum. In 5 cases, surgery for recurrent or chronic pancreatitis was performed. The range of follow-up was 11 to 54 months, and all children who underwent surgery had excellent results. Conclusions: Children with AP occasionally require reoperation for recurrent pancreatitis because of associate pancreatobiliary anomalies. Magnetic resonance cholangiopancreatography and ERCP provide excellent images of pancreatobiliary anomalies. Intraoperative cholangiopancreatography is also essential for accurate depiction of the ductal structure and selection of the appropriate surgical procedure. [Copyright &y& Elsevier]

Background The cystic dilatation of the biliary tract is an uncommon anomaly. Choledochocele, a cystic dilatation of the distal common bile duct, rarely presents clinically as massive gastrointestinal bleeding. Aim This is to report a very rare disease condition and highlight minimal access options in surgical care. Case summary A 13 year-old boy was referred with a day history of sudden onset of passage of bright red blood per rectum with a fainting episode. There was no anal protrusion, jaundice, recurrent epigastric pain nor bleeding from any other orifice. An initial endoscopic assessment of the upper digestive tract showed profuse bleeding from a sub-mucosal mass in the region of ampulla of Vater. Emergency laparotomy revealed small intestine filled with blood from duodenum to ileum. A duodenotomy showed a cystic mass with an ulcerated mucosa at the dome containing bilious fluid in the second part of the duodenum. The cyst was de-roofed and marsupialized. Post-operative recovery was complicated by features of adhesive small bowel obstruction on the 9th post op day and treated by laparoscopic adhesiolysis. He was discharged home in good clinical state. Conclusion Choledochocele is a differential diagnosis in the endoscopic finding of a submucosal mass in the second part of the duodenum. An initial oesophagogastroduodenoscopy endoscopy is necessary in the evaluation of massive lower gastrointestinal bleeding. [ABSTRACT FROM AUTHOR]

Introduction The congenital combination of duodenal atresia and choledochal cyst has only been reported in a few children. None of these patients had an intrapancreatic choledochocele causing persistent hyperbilirubinemia in the newborn period. Presentation of case A female newborn presented with duodenal atresia and received a duodeno–duodenostomy on day two of life. The postoperative course was uneventful except for progressive hyperbilirubinemia and elevation of liver enzymes. No evidence for surgical obstruction, malformations of the hepatobiliary system, or infectious diseases were found. At three months of age and persistent hyperbilirubinemia an intrapancreatic choledochocele type III according to Todani was confirmed by ultrasound and MRI. Upon laparotomy no lesion was visible or palpable within the pancreas. Even after duodenotomy distally of the duodeno–duodenostomy only a normal papilla Vateri could be identified. Transduodenal ultrasound allowed for localization and saline distension to outline the borders of the choledochocele. A transduodenal marsupialization provided immediate biliary drainage and postoperatively bilirubin levels returned to normal limits. Discussion We present a case of duodenal atresia and choledochocele requiring surgical treatment in the neonatal period. Transduodenal marsupialization prompted adequate biliary drainage without inflicting the potential complications of biliary and pancreatic diversion at this early age. A life-long endoscopic observation seems mandatory to examine the potential risk of metaplasia of the cystic remnant. Conclusion Early transduodenal marsupialization of an intrapancreatic choledochocele in a case of duodenal atresia is safe and feasible to prevent secondary liver cirrhosis. [ABSTRACT FROM AUTHOR]

Choledochocele, type III choledochal cyst, is a rare abnormality. We report a rare case of choledochocele involving a 15-year-old girl who presented with intermittent episodes of upper abdominal pain. She underwent computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP), which revealed no abnormalities of biliary and pancreatic ducts. Endoscopic retrograde cholangiopancreatography (ERCP) initially suggested a diagnosis of pancreas divisum (PD), and so we failed to diagnose choledochocele at that time. Her condition improved and she was discharged, but she presented again with recurrent episodes of upper abdominal pain. Then, spiral computed tomography with intravenous infusion cholangiography (IVC-SCT) facilitated a diagnosis of choledochocele. The coexistence of choledochocele and pancreas divisum is extremely rare, and it is difficult to diagnose. However, this case suggests that the absence of pancreatitis does not rule out choledochal cysts, and modern imaging techniques, such as IVC-SCT, must be applied in difficult cases. [ABSTRACT FROM AUTHOR]

A 10-month-old, previously healthy boy presented with one week of mild jaundice, light-colored stools and irritability. Abdominal sonography showed a large type I choledochal cyst and a separate, distinct cystic mass at the head of the pancreas. Magnetic resonance cholangiopancreatography was performed to evaluate the relationship of the two lesions. A type I choledochal cyst was confirmed, and a coexisting type III choledochocele was identified as the second cystic structure in conjunction with pancreaticobiliary malunion. The infant had complete resection of the type I choledochal cyst with Roux-en-Y hepaticojejunostomy, and anterior duodenotomy with marsupialization of the choledochocele. After five years of follow-up, the child is thriving and has had no recurrence of his symptoms. An exhaustive review of the literature identifies only one previous case of synchronous types I and III choledochal cysts, and this association is not clearly defined among the traditional classifications of type IV multiple choledochal cysts. Because operative management of a type III cyst requires the addition of a transduodenal approach, we encourage accurate reporting of mixed type choledochal cysts for the benefit of surgical planning, epidemiologic tracking and outcomes. [ABSTRACT FROM AUTHOR]

A 74-year-old woman, who had suffered from acute cholangitis, was referred to our department for further evaluation of the biliary tree. A diagnosis of choledochocele was made by endoscopic ultrasonography and endoscopic retrograde cholangiopancreatography. A small flat elevation with cholesterolosis was observed in the lower bile duct, and circumferential wall thickening was apparent in some parts of the upper and middle bile duct by intraductal ultrasonography and peroral cholangioscopy. With the diagnosis of choledochocele associated with superficial spreading cancer and cholesterolosis of the bile duct, pylorus-preserving pancreaticoduodenectomy was carried out. Microscopically, the tumor had spread extensively from the lower to the upper bile duct. Its invasion was limited to the mucosa for the most part, with microinvasion of the subserosa in the upper bile duct. In the lower bile duct, foamy cells were located beneath the neoplastic epithelium of the elevation. The number of cases of choledochocele associated with biliary cancer is becoming higher than previously reported. This anomaly may play some role in the development of biliary malignancy. Histological examination seems to be mandatory before making a diagnosis of cholesterolosis of the bile duct, since this condition may occasionally accompany cancer. [ABSTRACT FROM AUTHOR]

Congenital pancreaticobiliary malformations are sometimes associated with acute or chronic pancreatitis and biliary carcinoma. Currently, magnetic resonance cholangiopancreatography (MRCP) is one of the first choices for investigating and diagnosing pancreaticobiliary diseases noninvasively. We compared the accuracy of conventional MRCP and endoscopic retrograde cholangiopancreatography (ERCP) in making the diagnosis of congenital pancreaticobiliary malformations. 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. Of the 32 patients with congenital pancreaticobiliary malformations diagnosed on ERCP, 23 (72%) presented the same diagnosis on MRCP. Complete pancreas divisum was diagnosed in 73% on MRCP based on the finding of a dominant dorsal pancreatic duct crossing the lower bile duct and emptying into the duodenum without communicating with the ventral pancreatic duct. Pancreaticobiliary maljunction was diagnosed in 75% on MRCP based on the finding of an anomalous union between the common bile duct and the pancreatic duct and the existence of a long common channel. Conventional MRCP is a useful, noninvasive tool for diagnosing congenital pancreaticobiliary malformations; and the diagnostic accuracy can be increased with three-dimensional MRCP or dynamic MRCP with secretin stimulation. [ABSTRACT FROM AUTHOR]

Background. We aimed to clarify the clinical characteristics of choledochocele and to evaluate the possibility of choledochocele as a risk factor for biliary malignancies. Methods. The clinical feature, the configuration of the pancreatobiliary ductal system, coexistent pancreatobiliary lesions, and amylase level in bile in 21 patients with choledochocele were reviewed. The correlation between the configuration, comorbid diseases, and amylase level in the bile was investigated. Results. There was a female predominance, and 57% of the patients showed abdominal pain. Quite a few patients showed elevation of the levels of hepatobiliary enzymes. The configuration of the pancreatobiliary ductal system and choledochocele was classified into two categories: type I, where the choledochocele and pancreatic duct were visualized independently or simultaneously (90.5%); and type II, where the pancreatic duct was visualized after filling of the choledochocele (9.5%). Among coexistent biliopancreatic diseases, biliary stone diseases were the most frequent. Biliary malignancy was seen in 3 patients (14.3%). The amylase level in the bile was high in 50% (4/8) of the patients examined. The rate of abnormal elevation of amylase level in the bile in the two types of pancreatobiliary ductal system and choledochocele was 3/7 and 1/1, respectively. Conclusions. The prevalence of organic abnormal arrangement of the pancreatobiliary ductal system in which the choledochocele serves as a common channel is low. However, there are patients with suspected functional abnormal arrangement of the pancreatobiliary ductal system, who may possibly be a high-risk group for biliary malignancy. [ABSTRACT FROM AUTHOR]

Background: There are five types of choledochal cysts, which are anomalies that involve intrahepatic or extrahepatic bile ducts, or both. These lesions are found most frequently in patients who are Asian, female, infants but are recognized with increasing frequency in adults.Methods: We have managed 16 patients with this anomaly. One patient was Asian, and 1 was a child. There were 3 males and 13 females. The mean age was 29 years. There were 9 type I, 1 type II, 1 type III, 4 type IV, and 1 type V cysts. Resection of cysts and hepatico Roux-en-Y jejunostomy were performed in 9 patients for type I cysts. Pancreaticoduodenectomy was performed for a type I and a type IV cyst. The extrahepatic portion of a type IV cyst along with a segment of liver was resected in 1 patient. Operation was terminated on 1 patient with a type IV cyst because of extensive involvement of the intrahepatic ducts. She will undergo liver transplantation. The type II cyst was resected. No surgery was performed on a type III and type V cyst. Four of these patients were previously treated unsuccessfully by internal drainage procedures.Results: There was no mortality. Morbidity was limited to a patient who previously underwent incomplete resection of a cyst and a cyst Roux-Y jejununostomy. No cholangiocarcinoma has been encountered in our patients after a mean follow-up of 5.5 years from the time of initial discovery of the choledochal cyst.Conclusions: Management of choledochal cysts is successful after their complete removal. Partial cyst resection and internal drainage is less satisfactory because of occasional pancreatitis, cholangitis, and cholangiocarcinoma. Resection of the intrahepatic and intrapancreatic portions of the cysts reduces the risk of cancer even though this risk is low after incomplete cyst excision. Biliary continuity after cyst resection is best established by Roux-Y hepaticojejunostomy. [Copyright &y& Elsevier]