Neuroendocrine tumor of the common hepatic duct, mimicking a choledochal cyst in a 6-year-old child

We report a rare case of a neuroendocrine tumor of the extrahepatic biliary tree in a child. A 6-year-old girl who presented with jaundice and pruritus was found to have elevated liver enzymes along with a cystic dilatation of the common hepatic duct. After further diagnostic testing, a working diagnosis of a type I choledochal cyst was established. Laparotomy revealed hydrops of the gall bladder, an elongated and dilated cystic duct and a cystic dilatation of the common hepatic duct. An unusually thickened common bile duct was also noted at the time. Follow-up histology revealed an invasive neuroendocrine tumor which lead to a second look operation in which extended resection and lymphadenectomy was performed. No histological or gross pathological evidence of lymph node metastasis was found. Postoperatively the patient continues doing well 2 years after the original diagnosis was established. Neuroendocrine tumors of the extrahepatic bile duct are extremely rare with only 4 pediatric cases of a total of 51 cases published in the literature. To our knowledge, this is the youngest patient reported so far. The rarity of this entity made it challenging to diagnose a case of a neuroendocrine tumor in an atypical location that radiologically mimicked a choledochal cyst.     

A New Operative Approach for Type I Choledochal Cysts

To present a novel modification of the surgical technique for open excision of type I choledochal cysts. The treatment of choice for choledochal cyst is surgical excision because such cysts are associated with an increased incidence of cancer. The commonly used operative technique provides poor access to the structures posterior to the cyst, principally the portal vein, and poor visibility of the junction of the cyst with the hepatic ducts and the pancreatic duct. The modification reduces these problems. The key operative step involves early transection of the choledochal cyst near its midpoint. This provides improved access to the back of the cyst. Slitting the sides of the cyst allows clear visibility from within the cyst of the union of the hepatic ducts with the cyst, and the entrance of the pancreatic duct, thus protecting them. Excision of type I choledochal cysts using the described method was completed in 11 adult patients between 2003 and 2012. One patient (9 %) was found to have a concurrent gallbladder cancer and underwent an R0 resection at the time of choledochal cyst excision. No patients experienced any intraoperative complications. One patient (9 %) developed a portal vein thrombosis postoperatively. The described technique is a safe and feasible method for the excision of extrahepatic choledochal cysts, and provides an advantage in allowing the surgeon to visualize the hepatic and pancreatic ducts from within the cyst and thus protect them during cyst excision.     

Laparoscopic treatment of congenital choledochal cyst

We describe the laparoscopic treatment of a patient presenting with congenital choledochal cyst. Our patient was a 19-year-old man with a complaint of recurrent abdominal pain due to pancreatitis. The choledochal cyst was type I and had a common channel of pancreatobiliary duct, as revealed by endoscopic retrograde cholangiopancreatography. Under laparoscopic guidance, the dilated bile duct and the gallbladder were excised, and a Roux-en-Y anastomosis was constructed with an endo-EEA. Finally, end-to-side anastomosis was carried out by the continuous suture method, aided by an Endostitch between the stump of the hepatic duct and the Roux-en-Y limb. After the operation, slight hyperamylasemia was observed for several days but further treatment was not necessary. Postoperative symptoms were minimal, and the patient was discharged on the 11th day after the procedure. Although it is difficult and time-consuming, laparoscopic operation is highly beneficial for the patient. The use of such instruments as the endostapler and Endostitch may help to simplify this complex intracorporeal procedure involving division and anastomosis of the digestive tract. Received: 7 March 1997/Accepted: 11 April 1997     

Choledochal Cyst Associated With Polycystic Kidney Disease: Report of a Case

We report a very rare case of type I choledochal cyst associated with a polycystic kidney disease. A 48- year-old female had been dependent on hemodialysis for chronic renal failure due to polycystic kidney disease and was incidentally diagnosed to have a dilated common bile duct by an ultrasonography. An endoscopic retrograde cholangiopancreatography showed a spindle-shaped, dilated common bile duct (type I choledochal cyst) without visualization of the pancreatic duct. She underwent a resection of the choledochal cyst. Intraoperative cholangiography showed no reflux of contrast medium into the pancreatic duct. Amylase level of the aspirated bile from the bile duct was not elevated. In the case of choledochal cyst combined with renal fibropolycystic disease, pancreaticobiliary maljunction may not contribute to the etiology of choledochal cyst. In such cases, management of choledochal cyst is still controversial and requires further discussion.     

Malignant tumors in choledochal cysts.

Between 1960 and 1975, 17 patients with congenital cystic dilatation of the common bile duct (choledochal cyst) were treated and three were associated with malignant tumors in the cysts and one was with carcinoma of the gallbladder. Preoperative diagnosis of adenocarcinoma in the choledochal cyst was established in one patient by cytologic examination of the bile which was obtained during the procedure of endoscopic pancreaticocholangraphy (EPCG) and percutaneous transhepatic cholangiography (PTC). Definitive treatment of the choledochal cysts associated with malignant tumors in the biliary tract was accomplished by excision of the cysts with tumor and choledochojejunostomy in two patients, by cystoduodenostomy following external drainage in one and by cholecystectomy with resection of invaded transverse colon in one with cancer of the gallbladder. Successful excision of choledochal cysts in 11 patients including three cases associated with malignant tumors in the biliary tract during the last 15 year period is the basis of this report.     

Choledochal cyst, pancreatobiliary malunion, and cancer

A review of the clinical features of biliary cancer with choledochal cyst and pancreatobiliary malunion is presented, together with a recent case report. Biliary cancer develops in about 25% of patients with choledochal cyst and pancreatobiliary malunion, and usually occurs in younger patients (usually those in their 40s) than does biliary carcinoma in the general population. The risk of malignancy in the retained cyst with internal drainage is higher than that in the primary cyst. Early excision of the retained cyst should be performed as quickly as possible, even if the patient is symptomless. Some bile acid fractions and refluxed pancreatic enzymes in bile are possibly responsible for carcinogenesis. Carcinoma generally develops in the extrahepatic bile duct and gallbladder, and rarely in the intrahepatic bile duct. In cystic dilatation, cancer usually occurs in the common bile duct, while in diffuse or non-dilated type, it develops in the gallbladder. Multicentric carcinomas develop in the bile duct either synchronously or metachronously. The prognosis of biliary cancer is usually dismal. However, aggressive procedures are now gaining better results than conventional approaches. The procedure of choice for choledochal cyst or malunion is to prevent the development of cancer by performing an early excision. Removal of the entire extrahepatic bile duct is necessary, even in patients with malunion and no biliary dilatation. Carcinoma rarely arises in the intrahepatic bile duct after excisional surgery, probably due to the long-standing stricture of the bile duct. Capacious anastomosis and/or ductoplasty is essential. Carcinoma may also develop in the remnant bile duct. Excision of the distal duct extending into the pancreas is also necessary.     

Congenital bile duct dilation —Possibly an hereditary condition—

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father—a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

Intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst: report of a case

We report a case of intrahepatic cholangiocarcinoma arising 33 years after excision of a choledochal cyst. A 61-year-old woman was admitted to our hospital complaining of fever. Thirty-three years ago she had undergone extrahepatic choledochal cystectomy and choledochojejunostomy for a choledochal cyst. Computed tomography showed a tumor in the anterior segment of the liver, extending to the posterior and medial segments and the right portal vein. Intrahepatic biliary stones were seen in the bile ducts. We performed extended right lobectomy. Microscopically, the tumor was cholangiocarcinoma. Most of the tumor area was composed of invasive adenocarcinoma but a carcinoma-in-situ component was also observed in some regions including the hilar bile duct, where an intrahepatic biliary stone was seen. This suggests that the cancer development could be related to intrahepatic cholestasis. Patients with choledochal cyst may have to be carefully followed up for more than 30 years even after diversion surgery.     

腹腔内胃和食管异位发育形成囊肿

A 13-year-old female patient with a mass in the right upper quadrant of the abdomen for 2 months were admitted to the Southwest Hospital on February 1,2009.She was first misdiagnosed as with congenital choledochal cyst,while further examination revealed that there was no relationship between the cyst and bile duct,so exploratory laparotomy was carried out.The cyst was located at the mesentery,and was confirmed as abdominal cyst developed from the oesophagus and ectopic gastric mucosa.     

Choledochal cyst following operation for idiopathic perforation of the biliary tract in childhood

Three patients with idiopathic perforation of the biliary tract who had been treated by a drainage procedure, had a choledochal cyst during 2–8 years of follow up. In all patients, anomaly of pancreaticobiliary duct was revealed by cholangiography and/or retrograde cholangiopancreaticography. It was strongly suggested that biliary perforation in childhood and congenital choledochal cyst are common in pathogenesis and that an anomalous arrangement may play an important causative role. Presented at the 15th Annual Meeting of the Pacific Association of Pediatric Surgeons, Vancouver, British Columbia, Canada, May 11–14, 1982.     

Congenital bile duct dilatation--possibly an hereditary condition

A female patient underwent resection of a large choledochal cyst and hepaticojejunostomy when she was 11 years old at Tokyo Medical and Dental University Hospital. Her mother, 48 years old, also had congenital bile duct dilatation (CBD) with cancer. The mother had an anomalous pancreatico-biliary ductal junction. To investigate the possibility of a genetic link, family members of another three consecutive CBD cases were examined for bile duct dilatation and anomalous pancreatico-biliary junction. Endoscopic retrograde choledocho-pancreatography (ERCP) revealed an anomalous pancreatico-biliary ductal junction, without bile duct dilatation in the mother of a 17-year-old CBD patient. Two familial occurrences of CBD had been reported in the Japanese literature. Their combinations were; a father--a daughter and two sisters, respectively. Considering that the sex ratio of CBD is one male to 2.6 females, our results suggest that CBD or anomalous pancreatico-biliary ductal junction may be 1) an X-linked dominant trait or 2) autosomal dominant trait with relatively low penetrance in males. Since anomalous pancreatico-biliary ductal junction does not necessarily develop into bile duct dilatation, more familial investigations on pancreatico-biliary ductal junction and bile duct dilatation are essential to clarify the genetic contribution to CBD.     

Congenital stenosis of the hepatic duct at the porta hepatis in children with choledochal cyst

Background

The intrahepatic gallstone is known as one of the complications of choledochal cyst. Stenosis of the hepatic duct may contribute to the formation of the stone. This report describes concurrent congenital stenosis of the hepatic duct identified during the primary operation of choledochal cyst.

Methods

Fourteen children were operated on for choledochal cyst. To identify the concurrent stenosis, cholangiography and inspection of the hepatic duct were performed during the operation. In those patients with identified membranous stenosis, the membrane was resected. In those with nonmembranous stenosis, the wide hilar hepaticojejunostomy was performed after hepatic duct plasty.

Results

Eleven sites of hepatic duct stenosis were identified, 8 membranous stenoses and 3 relative stenoses without membrane. The membranous stenoses were classified into 4 subtypes as follows: a small central opening, a marginal opening, 2 openings, and a bridgelike structure. At the primary surgery on choledochal cyst, those stenoses were released, and the formation of the gallstone has not been observed in 4 to 11 years in all cases.     

Management of a ruptured bile duct cyst

A case of a ruptured bile duct cyst in a 25-year-old male patient is presented. The initial management of the clinical presentation of acute abdomen consisted of an exploratory laparotomy and a T-tube cystostomy of a choledochal cyst. Two months later, he was admitted to our surgical department. Preoperative evaluation showed a type IV-A choledochal cyst. The patient underwent excision of the choledochal cyst, cholecystectomy, and the construction of a Roux-en-Y end-to-side hepaticojejunostomy.     

Concomitant hilar cholangiocarcinoma with choledochal cyst and cholelithiasis in an asymptomatic patient: A case report

Introduction and importanceCholedochal cyst is an important risk factor for cholangiocarcinoma. Concomitant hilar cholangiocarcinoma with choledochal cyst with cholelithiasis is a rare finding.Case presentationA 52-year-old male presented with non-specific complaints of generalized weakness for 3 months. Transabdominal ultrasound showed dilated common bile duct with hyperechoic mass at hilar region; further evaluation with magnetic resonance cholangiopancreatography and contrast-enhanced computer tomography of abdomen and pelvis revealed concomitant intraductal hilar cholangiocarcinoma (Bismuth Corlette type 1) with Choledochal cyst (type IVa) with cholelithiasis. After optimization patient underwent left hepatectomy with common bile duct excision with cholecystectomy with Roux-en-Y hepaticojejunostomy. Histopathological examination confirmed it to be well-differentiated adenocarcinoma, intestinal type at the hilar confluence.Clinical discussionAn asymptomatic male patient with the concomitant finding of perihilar cholangiocarcinoma (Bismuth Corlette type I) with choledochal cyst type IVa with cholelithiasis is a rare finding. The patient was managed with surgical excision of the common bile duct with Roux-en-Y hepaticojejunostomy and cholecystectomy. Diagnostic evaluation should be proper not to miss or overlook such a synchronous lesion.ConclusionIncidental finding of concomitant perihilar cholangiocarcinoma with the choledochal cyst is rare. Proper evaluation of the patient with CECT abdomen and pelvis and MRCP is necessary for the diagnosis. Proper surgical resection with adequate lymph node removal is important for surgical clearance.     

Intrahepatic cholangiocarcinoma arising 28 years after excision of a type IV-A congenital choledochal cyst: report of a case

This report presents a rare case of intrahepatic cholangiocarcinoma (IHCC) arising 28 years after excision of a type IV-A congenital choledochal cyst. The patient underwent excision of a congenital choledochal cyst (Todani’s type IV-A) at 12 years of age, with Roux-en-Y hepaticojejunostomy reconstruction. She received a pancreaticoduodenectomy (PD) using the modified Child method for an infection of a residual congenital choledochal cyst in the pancreatic head at the age of 18. She was referred to this department with a liver tumor 22 years later. Left hemihepatectomy with left-side caudate lobectomy was performed and the tumor was pathologically diagnosed to be IHCC. The cause of the current carcinogenesis was presumed to be reflux of pancreatic juice into the residual intrahepatic bile duct during surgery. This case suggests that a careful long-term follow-up is important for patients with congenital choledochal cysts, even if a separation-operation was performed at a young age, and especially after PD.     

Anomalous union of the pancreaticobiliary duct without choledochal cyst: is cholecystectomy alone sufficient?

Purpose

Anomalous union of the pancreaticobiliary duct (AUPBD) is frequently associated with choledochal cyst and biliary tract cancers. Management of AUPBD with choledochal cyst consists of extrahepatic bile duct excision and cholecystectomy. In cases of AUPBD without choledochal cyst, cholecystectomy alone is usually recommended. This study aimed to evaluate the occurrence of biliary tract cancer in AUPBD patients in order to assess the validity of the currently recommended operative management.

Methods

Of a total of 10,255 endoscopic retrograde cholangiopancreatography cases performed at two Korea University hospitals from 2001 to 2010, 55 (0.54 %) cases of AUPBD were identified. Patients with AUPBD were divided according to its subtype (P-C union and C-P union) and the presence of choledochal cyst for analysis. The occurrence of benign and malignant disease was evaluated and compared between the groups.

Results

Gallbladder stones were more frequently found in AUPBD patients without choledochal cyst (p?=?0.032). Biliary tract cancer occurred more frequently in P-C union (p?=?0.050), especially the common bile duct cancer (p?=?0.023). When analyzed according to the presence of choledochal cyst, biliary tract cancer occurred more frequently in AUPBD patients without choledochal cyst (p?=?0.005), with bile duct cancer being significantly more common (p?=?0.015). However, there was no difference in the presence of gallbladder cancer between the two groups (p?=?0.318).

Conclusions

Since cancers of the biliary tract occur more frequently in the AUPBD group without choledochal cyst, cholecystectomy alone may not be protective of the future occurrence of bile duct cancers, and thus, vigilant surveillance is necessary in this population group.     

Embryonal rhabdomyosarcoma of the common bile duct mimicking choledochal cyst

Embryonal rhabdomyosarcoma of the common bile duct is a very rare malignancy of childhood. The radiological appearance of the lesion is similar to that of congenital choledochal cyst if there is no local invasion to the adjacent tissues. The authors present a case of embryonal rhabdomyosarcoma of the common bile duct which was considered to be a congenital choledochal cyst preoperatively, and they discuss this very rare childhood tumor, with a brief survey of the literature. In conclusion, it is important to know that this rare tumor can simulate congenital choledochal cyst; it should be considered in the differential diagnosis of obstructive jaundice in children.     

小儿先天性胆总管囊肿自发性穿孔的临床分析

目的：探讨先天性胆总管囊肿自发性穿孔的诊断方法和手术方式。方法：回顾性分析16例先天性胆总管囊肿自发性穿孔患儿的临床资料。结果：本组16例患儿年龄均小于4岁，平均年龄23个月。临床表现为胆胀，腹痛，呕吐及发热，同时有全腹压痛，10例术前经腹腔穿刺明确胆道自发性穿孔的诊断，其中5例经B超或CT检查证实有胆总管囊肿，采用囊肿外引流加腹腔引流治疗。16例患儿术后均恢复良好，3个月左右再地囊肿切除加胆道重建。结论：先天性胆总管囊肿穿孔与胆道梗阻有关，对小婴儿有腹膜炎表现，全腹压痛并以右上腹为著者，应常规行腹腔穿刺检查，急诊治疗宜选择囊肿外引流术，而后再行囊肿切除和胆道重建。     

Traumatic rupture of choledochal cyst in a child

Traumatic rupture of choledochal cyst is an extremely rare disorder. The current patient is a 4-year-old boy who fell in a bathroom and suffered a blow to the abdomen. Percutaneous transhepatic cholangiography revealed pancreaticobiliary maljunction. Inflammation of the peritoneal cavity was moderate. At first look, the choledochal cyst was excised and hepaticojejunostomy was performed. At this time, a rupture approximately 2 mm in diameter was recognized at the rear surface of the inferior part of the common bile duct.     

Biliary cystadenoma of the extrahepatic bile ducts: Report of a case and review of the literature

Benign neoplasms of the extrahepatic bile ducts are rare and there have only been thirteen reported cases of cystadenoma. One case of cystadenoma of the common hepatic duct and solitary hepatic cyst is presented here and a total of fourteen cases are reviewed. The patient was a 45 year old woman, who complained of abdominal pain with rigidity, but without jaundice. Ultrasonography of the liver showed a 6 cm cyst of the right lobe. Percutaneous transhepatic cholangiography revealed a multilobular cystic mass. At emergency laparotomy, a polypoid multilobular tumor with a stalk was found hanging from the common hepatic duct and was excised. Almost all the patients reported were middle aged woman with jaundice. Preoperative diagnosis was made possible by percutaneous transhepatic cholangiography, endoscopic retrograde cholangiography or ultrasonography. The treatment of choice was complete excision. In three cases, a solitary hepatic cyst accompanied the cystadenoma of the common hepatic duct.