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.

The present study was aimed to update the classification of hepatobiliary malformations and study the clinicopathological profile of pediatric choledochal cyst (CDC) and pediatric giant choledochal cyst (GCC) patients undergone surgery.

We have retrospectively analysed the data of 57 consecutive cases of CDCs in paediatric patients from a time period spanning from 2016 to 2020.

Revised classification of hepatobiliary malformations was proposed and these were divided into 2 broad headings, choledochal (congenital and acquired) and extra-choledochal spectrum. 57 pediatric patients were diagnosed as having CDC with average age 4.615 years and female to male ratio of 3.7:1. We have also reported one of the largest GCC measuring 23 × 10 × 9 cm size. The classical triad was known to be more common and seen in 60% GCCs as opposed to 14.5% in CDCs. Values of serum bilirubin, SGOT, SGPT, PT/INR were elevated in CDC series and normal in GCC patients. 55 patients (96.5%) underwent cyst excision and Roux-en-Y hepaticojejunostomy. Mortality was seen in 7.01% patients (n = 4) following surgery.

Simplified and broader classification system for CDCs has been proposed. Clinical studies found that GCC differs considerably from classical CDCs.

Highlight Ikarashi and colleagues report a case of choledococele treated by endoscopic sphincterotomy and observed on direct peroral cholangioscopy. Histological examination reveals duodenum-like mucosa without atypical changes in the choledochocele. Direct peroral cholangioscopy using an ultra-slim endoscope may be a useful technique for diagnosis and surveillance of choledochocele.

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.

Choledochoceles may cause biliary obstruction and harbor malignancy. We conducted a 40-year systematic review of the literature for this rare anomaly.

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.

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.

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.

Biliary cystic disease has been known of for centuries. It has traditionally been classified as 5 major types of disease, each with different clinical profiles and attributes. In this article, the basis for the existing classification schemes is reviewed and a simplified classification scheme and treatment regimen are suggested.

Choledochoceles are cystic dilatations of the intraduodenal portion of the common bile duct. Although often classified as Type III biliary cysts, choledochoceles have distinctive demographic and anatomic features and a lower risk of malignancy than other types of choledochal cysts. Type A choledochoceles are cystic dilatations of a segment of the intra-ampullary bile duct and are located proximal to the ampullary orifice. Type B choledochoceles are diverticula of the intra-ampullary common channel and are located distal to the ampullary orifice; they can be distinguished from duodenal duplication cysts both anatomically and histologically. Both types of choledochocele may present with pancreatitis, biliary obstruction, or nonspecific gastrointestinal symptoms. Cross-sectional imaging, endoscopic ultrasound, and endoscopic retrograde cholangiopancreatography are useful for diagnosis. Choledochoceles may be drained or resected endoscopically. Surveillance for dysplasia should be considered for lesions that are not resected.

The classification of choledochoceles as a type of choledochal cyst stems from the 1959 article by Alonso-Lej and colleagues describing 94 choledochal cysts, only 4 of which were choledochoceles. Even then, Alonso-Lej questioned the propriety of including the choledochocele, stating it was unclear "as to whether or not it originates from the same etiologic factors [as other choledochal cysts]". In 1971, Trout and Longmire also questioned the validity of classifying choledochoceles as choledochal cysts, noting the anatomic position article and variant mucosa of the choledochocele. Wearn and Wiot, in an article titled "Choledochocele: not a form of choledochal cyst", cite the differences in clinical presentation, demographics, and histology as reasons why choledochoceles represent separate entities from choledochal cysts. Over the ensuing decades, numerous investigators have questioned the legitimacy of classifying choledochoceles as choledochal cysts. In our recent series (the only one to our knowledge directly comparing patients with choledochocele and other [type I, II, IV, and V] choledochal cysts), patients with choledochoceles differed from patients with choledochal cysts in their age, gender, presenting symptoms, history of previous cholecystectomy, pancreatobiliary ductal anatomy, management, and most importantly, propensity to developing biliary malignancy. Based on the available cases of choledochoceles found in the literature, combined with the recent series from our institution, we conclude that choledochoceles seem to be distinct entities from choledochal cysts.

The aim of this analysis was to report a multidisciplinary series comparing choledochoceles to Todani Types I, II, IV, and V choledochal cysts.

Choledochoceles have been classified as Todani Type III choledochal cysts. However, most surgical series of choledochal cysts have reported few choledochoceles because they are managed primarily by endoscopists.

Surgical, endoscopic, and radiologic records were reviewed at the Riley Children's Hospital and the Indiana University Hospitals to identify patients with choledochal cysts. Patient demographics, presenting symptoms, radiologic studies, associated abnormalities, surgical and endoscopic procedures as well as outcomes were reviewed.

A total of 146 patients with "choledochal cysts" including 45 children (31%) and 28 with choledochoceles (18%) were identified, which represents the largest Western series. Patients with choledochoceles were older (50.7 vs. 29.0 years, P < 0.05) and more likely to be male (43% vs. 19%, P < 0.05), to present with pancreatitis (48% vs. 24%, P < 0.05) rather than jaundice (11% vs. 30%, P < 0.05) or cholangitis (0% vs. 21%, P < 0.05), to have pancreas divisum (38% vs. 10%, P < 0.01), and to be managed with endoscopic therapy (79% vs. 17%, P < 0.01). Two patients with choledochoceles (7%) had pancreatic neoplasms.

Patients with choledochoceles differ from patients with choledochal cysts with respect to age, gender, presentation, pancreatic ductal anatomy, and their management. The association between choledochoceles and pancreas divisum is a new observation. Therefore, we conclude that classifications of choledochal cysts should not include choledochoceles.

Cystic diseases of the biliary tract encompass a complex group of congenital disorders. Some of the disorders, such as Caroli disease and the hepatobiliary cysts of autosomal-dominant polycystic kidney disease, share common embryologic origins, whereas others, such as choledochal cysts, biliary diverticula, and choledochoceles, have unclear origins. This article reviews the embryologic, clinical, pathologic, and imaging features of biliary cystic disease.

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.

A 46-yr-old man presenting with biliary obstruction from an intrapancreatic tumor underwent pancreaticoduodenectomy. The pathology report showed the tumor to be an enteric duplication cyst. Diagnostic imaging features and operative management are discussed.

Choledochoceles (type III biliary cysts) are cystic dilations of the terminal common bile duct or common pancreatobiliary channel. Although no size criteria have been defined, it is generally assumed these must be large. However, we describe patients who do not meet the perceived size criteria for choledochoceles, but who nonetheless have a dilated common pancreatobiliary channel.

We reviewed the presenting symptoms, endoscopic and radiographic findings, and response to endoscopic therapy of patients meeting our criteria for the dilated common channel syndrome.

Of 2847 patients undergoing ERCP, 100 (3.5%) had the dilated common channel syndrome. Common presenting symptoms and signs included abdominal pain in 97%, abnormal liver function test(s) in 66%, and a history of acute or recurrent pancreatitis in 46%. A bulge was visible above the papilla in 88%, with a dilated common bile duct in 54% and a dilated pancreatic duct in 28%. After endoscopic unroofing of the common channel, 77% had complete and long-lasting resolution of symptoms, 18% had partial or transient improvement, and 5% had no change.

Although classic choledochoceles are rare, a lesser degree of dilation of the common channel is more frequent than generally appreciated. We postulate that this finding represents an "incomplete," acquired form of choledochocele, possibly caused by underlying papillary stenosis. Whatever the etiology and appropriate term, the presence of a dilated common channel predicts a high rate of clinical response to endoscopic therapy.

Congenital bile duct cysts are now a well-documented anomaly of the biliary tree, and have become more common in Japan. Familial occurrence of congenital bile duct cysts, however, is extremely rare, with only six reported cases in the literature. We report a familial pattern of congenital bile duct cysts in a mother and her daughter. A 33-year-old female was admitted for evaluation of right upper quadrant abdominal pain and fever 6 days after an uneventful delivery of her second child. A computed tomography (CT) and ultrasound scan (US) revealed an obstructed biliary tract. Percutaneous transhepatic biliary drainage was then performed, and a cholangiogram revealed a Scholtz type B choledochocele without an anomalous connection of the pancreaticobiliary ducts. Endoscopic US demonstrated that the choledochocele was associated with a stone in the cyst. A pylorus-preserving pancreatoduodenal resection was performed, and a histological study revealed that the choledochocele was lined by biliary mucosa without evidence of malignancy. The newborn infant had an abdominal tumour. An US and CT revealed a congenital bile duct cyst. An operation was performed and the intraoperative cholangiogram showed an Alonso-Lej type I congenital bile duct cyst with an anomalous connection of the pancreaticobiliary ducts. Whether congenital bile duct cysts are hereditary remains to be elucidated.

This report demonstrates the use of endoscopic ultrasound in the diagnosis of choledochocele. The patient was an elderly female who presented with abdominal pain, weight loss, and biliary obstruction. At endoscopic ultrasound a 5 cm cystic lesion was found near the ampulla, which clearly communicated with the common bile duct.

A case of a 57-year-old farmer with a rare type of choledochal cyst (choledochocele; Alonso-Lej's type III) is described. The patient was admitted because of obstructive jaundice and acute biliary infection. Abdominal computed tomography scan showed a cystic lesion in the head of the pancreas, and endoscopic retrograde cholangiopancreatography disclosed cystic dilatation of the terminal portion of the common bile duct. It was suspected that the choledochocele could swell and compress the common bile duct, causing obstructive jaundice and acute cholangitis; therefore, it was surgically resected. We also reviewed 61 cases of choledochocele reported in Japan; the findings were similar to those reported in the English literature.

The management of 23 patients treated for choledochal cysts at the Oregon Health Sciences University between 1969 and 1990 is reviewed. The median age was 27 years, with a range from 1 month to 90 years. Seventy-eight percent of patients presented with abdominal pain, and 35% were jaundiced. Three patients presented with cholangitis, two with cyst rupture, and one with recurrent pancreatitis. Nine patients had had previous biliary surgery. The diagnosis was made in all patients with ultrasound and/or cholangiography. Fifteen patients (65%) had type I cysts, 2 had a type II cyst, 5 (22%) had type III cysts, and 1 had a type IV cyst. Stones were present in four (17%) cysts, and all excised cysts were benign. Seventeen patients with type I and II choledochal cysts had complete cyst excision and choledochoenterostomy. Four of five patients with type III cysts had endoscopic cyst incision and drainage, while the fifth patient had transduodenal cyst excision and sphincteroplasty. The patient with a type IV cyst had extrahepatic cyst excision and choledochojejunostomy. There were no operative deaths. Two postoperative complications occurred: cholangitis and a prolonged ileus. All patients had resolution of their pain and jaundice. Two patients had late cholangitis. Cyst excision and choledochojejunostomy are the treatment of choice for types I and II choledochal cysts. Extrahepatic cyst excision and choledochojejunostomy may be adequate treatment for type IV cysts. Endoscopic incision and drainage is appropriate for selected patients with type III cysts.

A 47-year-old woman was admitted for evaluation of pain in the right upper quadrant of the abdomen. Seventeen years previously, she had undergone cholecystectomy for cholelithiasis. Endoscopic retrograde cholangiopancreatography (ERCP) showed a cystic dilatation of the terminal portion of the common bile duct (CBD) protruding into the duodenal lumen and delaying the drainage of contrast medium. In this patient the CBD and the pancreatic duct appeared to be unimportant in the formation of the cyst. Repeated changes in the radius of the cyst suggested dysfunction of the ampullary component of the sphincter of Oddi, with maintenance of normal function of the common duct component. The pathogenesis of the choledochocele in this patient is discussed in relation to dysfunction of the sphincter of Oddi. In addition, 2 criteria for the diagnosis of choledochocele by ERCP are proposed: [1] cystic dilatation of the terminal portion of the CBD protruding into the duodenal lumen, and [2] absence of the narrow segment of the CBD.

We report two patients with periampullary cysts associated with recurrent attacks of acute pancreatitis. In both patients the diagnosis was made preoperatively by upper gastrointestinal endoscopy and ERCP, which was also useful in determining the relationship of the cysts to the biliary and pancreatic ductal systems. Simple marsupialisation of the cysts resulted in long term relief of symptoms. Congenital cystic anomalies in the second part of the duodenum should be diligently sought in patients with pancreatitis of unexplained cause, as surgical therapy is safe and effective.

Choledochocele or type III choledochal cyst is a rare lesion of the biliary tree. It may be of acquired or congenital etiology and can assume one of two anatomic variants. Either the common bile duct and the pancreatic duct enter the choledochocele together, or it is formed by the common bile duct alone with the pancreatic duct entering as a separate opening. The most frequent presenting symptoms are abdominal pain, pancreatitis, and jaundice. Traditional therapy has been either marsupialization of the cyst into the duodenum or complete surgical excision. This paper cites the eighth patient reported in the English-language literature whose choledochocele was treated endoscopically. It is the authors' opinion that either an endoscopic papillotomy or an endoscopic fistulotomy with extension of the incision over the cyst is the simpler and preferred method of treatment.

A case history has been presented that reports a rare type III choledochal cyst (choledochocele) and describes the association of a choledochocele and recurrent acute pancreatitis. The most reliable diagnostic technique, as used in our patient and supported by our literature review, is endoscopic retrograde cholangiopancreatography. This case further supports surgical correction employing transduodenal sphincteroplasty. This procedure is highly recommended for management of the intramural choledochocele (type IIIc), especially when associated with recurrent pancreatitis. Finally, patients who present with recurrent pancreatitis without the usual historical or diagnostic findings of biliary tract or alcoholic disease should have a choledochocele included in the differential diagnosis.

Choledochocele is a rare abnormality involving the intramural segment of the common bile duct. It may present clinically as recurrent acute pancreatitis, biliary colic, or cholestatic jaundice. A choledochocele may be easily overlooked by the conventional diagnostic methods, such as upper gastrointestinal series, intravenous cholangiogram, abdominal ultrasound, and computed tomography. Endoscopic retrograde cholangiopancreatography is helpful in demonstrating a choledochocele. Additionally, in selected cases, a choledochocele may be effectively managed by endoscopic sphincterotomy. We present the clinical, endoscopic, and radiographic findings in a series of 8 patients with choledochocele. The radiologic technique most useful in demonstrating a choledochocele at the time of endoscopic retrograde cholangiopancreatography is detailed. The pathogenesis, differential diagnosis, and relevant current literature pertaining to choledochocele are discussed.

A case of choledochocele seen in a 68-year-old male was reported. Analysis of fourteen cases reported under a variety of designations featuring cystic dilatation of the distal common bile duct protruding into the duodenal lumen and the case presented here disclosed that these cases could be classified into two types of pathology; both of the bile duct and the pancreatic duct opened into the cyst of the ampulla in 6 cases, whereas the bile duct and the pancreatic duct joined at the papilla of Vater and distal end of the bile duct formed a cystic dilatation protruding into the duodenal lumen in the remaining 9 cases.

A patient with a choledochocele associated with development of acute fulminant pancreatitis is presented. Treatment had been severely compromised by this rare anomaly before it was identified by percutaneous cholangiography. Because it is potentially curable with surgery, identification of the lesion by percutaneous or intravenous cholangiography has important clinical significance.

The eighth report case of choledochocele is described. This lesion is a cyst communicating with the terminal portion of the common bile duct. It is characteristically lined with duodenal mucosa and is probably a form of duplication of the duodenum. The surgical treatment is marsupialization of the cyst to the interior of the duodenum.