Choledochocele with hyperplastic epithelium in a patient who developed severe acute pancreatitis and underwent subtotal stomach-preserving pancreatoduodenectomy: a case report.

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.

Successful radiological detection and surgical management of type 3 choledochocele: A case report.

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.

Diagnosis and minimally invasive treatment of type III choledochal cysts.

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.

Choledochal Cyst of the Cystic Duct - A Supplement to Todani's Classification.

Choledochal cyst of the cystic duct is a rare anomaly; broadly classified, the pathology may either present as a solitary anomaly or with concomitant involvement of other parts of the biliary tree. In the absence of standard guidelines for surgical planning, the definitive management is based on the experience shared in the literature in the form of case reports and case series. The available options should be discussed with the patient while explaining the possible short- and long-term complications. The authors herewith present two cases of choledochal cyst of the cystic duct along with a review of literature relevant to their etiopathogenesis and its implications for the definitive management.

Choledochocele observed on direct peroral cholangioscopy using an ultra-slim endoscope.

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.

An extremely rare combination of choledochocele and bile duct duplication escalating severe acute pancreatitis and cholangitis: A case report.

BACKGROUND: Combination of choledochocele and extra-hepatic duct duplication is an extremely rare congenital abnormality. CASE PRESENTATION: 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. CONCLUSION: 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.

Coledococele: caso diagnosticado por resonancia magnética / Choledococele: case diagnosed by MRI

Resumen: El coledococele es una dilatación quística del segmento distal del conducto biliar común que sobresale en el lumen duodenal. Estos comprenden menos del 2% de todos los casos reportados de quistes de colédoco (2). Los hallazgos imagenológicos del coledococele y de los quistes de duplicación duodenal son muy similares, ambos presentan además clínica variada e inespecífica, por lo que el diagnóstico definitivo suele ser durante la cirugía. Presentamos un caso de una paciente de 10 años que consulta por dolor abdominal con elevación de transaminasas que, tras estudio con resonancia magnética con colangioresonancia y EDA se diagnostica coledococele.

Abstract: Choledochocele is a cystic dilation of the distal segment of the common bile duct protruding into the duodenal lumen. Is also known as type III choledochal cyst according to Todani's classification. It is usually misdiagnosed as duodenal duplication cyst. Multislice spiral computed tomography and magnetic resonance cholangiopancreatography may be comparable to endoscopic retrograde cholangiography for diagnosis of choledochocele. We present a case of a 10-years-old girl presented with abdominal pain, elevation of transaminases and a magnetic resonance cholangiopancreatography (MRCP) scan that revealed a choledochocele.

Type B choledochocele vs duodenal duplication cyst: a diagnostic dilemma and its management: a case report.

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.

Choledochal malformations: global research, scientific advances and key controversies.

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.

Open surgical treatment of choledochocele: A case report and review of literature.

Choledochocele (also known as type III choledochal cyst according to Todani's classification) is a cystic dilation of the distal segment of the common bile duct protruding into the duodenal lumen. Cases are rare and the etiology remains unclear. It is usually misdiagnosed as peptic ulcer, as in the patient whose case is described here. Multislice spiral computed tomography and magnetic resonance cholangiopancreatography may be comparable to endoscopic retrograde cholangiography for diagnosis of choledochocele. Both endoscopic therapy and open surgical management are safe options, and size of the cyst plays a role in the decision-making for which approach to apply. A 50-year-old woman admitted to our hospital with upper abdominal pain caused by choledochocele with large size was successfully treated by open surgical management. We present the details of her case in this case report and discuss the recent literature on such cases and their therapeutic management.

[Choledochocele: a rare variety of congenital cystic dilation of the bile ducts]. / Le cholédococèle: une variété rare de dilatation kystique congénitale des voies biliaires.

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 classi?ed 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.

Choledochal Malformations: Surgical Implications of Radiologic Findings.

OBJECTIVE: The purpose of this article is to review the relevant pathophysiologic features, appearances, and surgical implications of choledochal malformations. CONCLUSION: Choledochal malformations, colloquially called choledochal cysts, initially described in 1723, have been recategorized multiple times, the most widely accepted being the Todani classification based on morphologic features and location. Although readily applied to imaging findings, this classification system does not correlate well with clinical and surgical management. In 2004, Visser and colleagues proposed an alternative that emphasized the etiologic factors, imaging appearance, and treatment approach.

Pediatric choledochal cysts: diagnosis and current management.

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.

The presentation and management of choledochocele (type III choledochal cyst): A 40-year systematic review of the literature.

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.

Ulcerated choledochocele: A case report.

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.

How to treat a neonate with duodenal atresia and intrapancreatic choledochocele causing persistent hyperbilirubinemia: A case report.

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.

Choledochocele: A Case Report and Discussion of Diagnosis Criteria.

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.

Robotic resection of choledochocele in an adult with intracorporeal hepaticojejunostomy and Roux-en-Y anastomosis: encouraging progress for robotic surgical treatment of biliary disease.

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.

Choledochocele with pancreas divisum: A rare co-occurrence diagnosed on magnetic resonance cholangiopancreatography.

We report a case of a 42-year-old male with symptomatic choledochocele and incidental pancreas divisum diagnosed with magnetic resonance cholangiopan- creatography (MRCP). Small choledochocele is rare congenital malformation associated with non-specific symptoms and a delay in diagnosis. The coexistence of choledochocele and pancreas divisum is extremely rare with only two case reports published in literature. In both cases MRCP failed to diagnose any biliary or pancreatic abnormality. This case suggests that the patients with recurrent abdominal pain and pancreas divisum should not be presumed to be suffering from pancreatitis. Careful evaluated for additional anomalies in the biliary tree should be sought for refractory symptoms. MRCP is a useful one-stop-shop for diagnosing pancreatic and biliary ductal anomalies.

Choledochocele: An unusual presentation in a premature neonate.

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.