Carcinoma Arising in the Pancreas 17 Years After Primary Excision of a Choledochal Cyst: Report of a Case

We report herein the case of a 42-year-old woman in whom a pancreatic carcinoma was found 17 years after excision of a choledochal cyst with Roux-en-Y hepaticojejunostomy. Although the incidence of biliary carcinoma associated with choledochal cyst is high, reports of cancer developing in the remaining intrapancreatic biliary tract or pancreas in patients who have previously undergone primary excision of a choledochal cyst are extremely rare. Long-term follow-up is recommended even for patients who have undergone excisional surgery for a choledochal cyst, in consideration of the possibility that cancer associated with the intrapancreatic biliary tract or pancreas may develop. Received: May 18, 2000 / Accepted: November 20, 2000     

Pseudopancreatitis in choledochal cyst in children: intraoperative study of amylase levels in the serum.

A case of acute necrotizing pancreatitis in association with choledochal cyst is presented. Pancreatitis associated with choledochal cyst is probably caused by a biliary reflux into the pancreatic duct via a pancreatobiliary malunion, as the intraductal pressure of the cyst exceeds that of the pancreatic duct. Ampullar stenosis due to gallstones or inflammatory changes may increase the intraductal pressure. Bile with activated pancreatic enzymes refluxes into the pancreatic duct, and possibly results in acute pancreatitis. However, patients with choledochal cyst presenting with recurrent bouts of abdominal pain, vomiting, and fever have often been diagnosed as having acute pancreatitis because of hyperamylasemia, despite no evidence of pancreatitis at the time of surgery. At the time of bouts, they also show a slight elevation of serum bilirubin, and an increase in the degree of the choledochal dilatation that are possibly caused by biliary obstruction, not ampullar obstruction, due to suppurative cholangitis. The term "fictitious pancreatitis" or "pseudopancreatitis" in choledochal cyst appears to be appropriate. This clinical study shows that amylase in the biliary tract has ready access to the blood stream, probably through a sinusoidal pathway by cholangiovenous reflux, and a lymphatic pathway, via the Disse's space and denuded cyst wall, provided the biliary ductal pressure is increased.     

Pancreatic disorders associated with anomalous pancreaticobiliary junction.

BACKGROUND: The aim of this study was to establish an optimal management strategy for pancreatic disorders associated with anomalous pancreaticobiliary junction (APBJ). METHODS: In 64 adult surgical cases of APBJ (common channel 15 mm or longer) (43 with and 21 without choledochal cyst), associated pancreatic disorders, pancreatographic results, and treatment outcomes were analyzed. RESULTS: Of 64 patients, 56 had pancreatobiliary symptoms. Twenty-four patients (38%) had pancreatic disorders: acute pancreatitis (n = 20), chronic calcifying pancreatitis (n = 2), and pancreatic carcinoma (n = 2). Twenty patients (31%) had abnormal pancreatograms. The incidence of acute pancreatitis was significantly higher in patients with an abnormal pancreatogram, particularly dilatation, protein plugs or stones of the common channel or main pancreatic duct, and coexisting pancreatic ductal anomaly. All patients with choledochal cyst underwent cyst excision and hepaticojejunostomy. Eleven patients without choledochal cyst or pancreatobiliary carcinoma underwent cholecystectomy alone. Protein plugs and pancreatic stones were extracted through the bile duct stump or by sphincterotomy. No patients experienced pancreatitis during a mean postoperative follow-up of 6.7 years. CONCLUSIONS: In managing APBJ, attention should be paid to the possibility of associated pancreatic disorders and an abnormal pancreatogram. APBJ with choledochal cyst requires cyst excision. Cholecystectomy alone may be adequate for APBJ without cyst.     

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.     

Choledochal cyst as a complex malformation of the intra and extrahepatic bile ducts

The authors present the case of a 64 years woman with a choledochal cyst along with a complex malformation of both intra and extrahepatic bile ducts. The patient was admitted with a diagnosis of acute and underestimated cholecystitis, which was in fact a real acute severe suppurated angiocolitis located at the cystic cavity level along with severe hepato-renal failure. As long as the choledochal cyst evolves as a stand-alone entity, it can frequently be associated with other malformations of the bile ducts--such as choledochal duct stenosis or abnormal connection of the common hepatic duct with pancreatic ducts. The main dilatation was engulfing the whole common bile duct with a fusiform aspect, and the cranial end is opening into a long hepatic duct from which were emerging several biliary segments branches, for both hepatic lobes. This aspect of a multistage convergence with four branches which is opening into a long hepatic bile duct and then in the choledochal cyst, represents an unusual malformation which does not respect the rules described by former published authors.     

Forme fruste choledochal cyst

Four patients had the characteristic features of choledochal cyst except for the cystic component. All patients had stenosis of the distal common bile duct, a "long common channel" secondary to a proximal junction of the common bile and pancreatic ducts, cholecystitis and the classic pathological microscopic features of choledochal cyst in the wall of the common bile duct. Three children had coexisting intrahepatic duct cysts and/or stenosis and one had intrahepatic choledocholithiasis. The clinical presentations were cholangitis (2), pancreatitis (1) and biliary obstruction (1). In all cases the common bile duct was resected and biliary reconstruction was carried out by choledochojejunostomy (Roux-en-Y). Morbidity was minor except in one patient with ductal disease extending far into the intrahepatic ducts. This child developed an anastomotic stricture requiring revision of the anastomosis and long-term "U" tube stenting. Forme fruste choledochal cyst appears to be another variation in the spectrum of pancreaticobiliary malformations of choledochal cyst. Treatment is identical, that is, excision of all malformed ductal tissue.     

Abnormalities of the pancreatic and biliary ducts in adult patients with choledochal cysts

A choledochal cyst in the adult patient is not just an aneurysmal dilatation of the common bile duct, but it is the most obvious manifestation of a more widespread pancreaticobiliary ductal dysplasia. Most patients have abnormalities of the pancreatic ducts and, frequently, multiple areas of dilatation involving the intrahepatic bile ducts. Of our nine patients, six had pancreatograms, five of which were distinctly abnormal. Two of the five had recurrent pancreatitis. Six patients were found to have marked stenosis of the outflow tract from the cyst. Because the mucosa lining the choledochal cyst was chronically ulcerated, anastomoses to the cyst formed strictures in seven (64%) of 11 patients, and the long-term risk of carcinoma arising in the cyst was increased. The preferred therapy was excision of the cyst with Roux-en-Y hepaticojejunostomy.     

Anomalous arrangement of the pancreatobiliary ductal system in patients with a choledochal cyst

An anomalous arrangement of the pancreatobiliary ductal system is usually observed in patients with a congenital choledochal cyst and is represented by a long common channel distal to the pancreatobiliary junction. According to the angle of the pancreatobiliary junction, anomalous junctions can be classified into four groups: right angle, acute angle, complex union, and normal union (presented here in the order of their incidence). The right-angle type tends to have cystic dilatation of the choledochus, whereas the acute-angle type generally has cylindrical dilatation. However, the grade and length of the stenosis on the distal part of the choledochus tend to determine the type of choledochal cyst. Cystic dilatation is usually observed in patients with high-grade and long stenosis, whereas cylindrical dilatation is seen in those with low-grade and short stenosis. Patients with an acute angle junction seem to only suffer from abdominal pain, and those in the complex union group tend to have jaundice and an abdominal tumor. A high amylase level in the bile within the cyst is observed frequently. In patients with obstructive jaundice, however, the amylase level is usually not elevated, because pancreatic juice cannot enter the choledochus.     

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.     

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.     

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??Etiology relationship and principles of treatment of pancreatic and biliary anomalous junction and choledochal cyst LI Long, ZHANG Jin-shan. Pediatrics Surgery of Capital Institute, Beijing 100020, China
Corresponding author: LI Long, E-mail??zjs851@163.com
Abstract Choledochal cyst (CC) is a pathologic condition characterized by varying degrees of congenital dilatation of the biliary system. Most of the reported cases come from Asia and its etiology remains unclear. Some people suggested that CC is a result of weakness of the wall and the distal obstruction of the common bile duct. But its underlying precise mechanisms remain unclear. The pathogenetic implications of pancreaticobiliary maljunction (PBM) is a popular hypothesis: In PBM, the common channel can be so long that the junction of the pancreatic and bile ducts is located outside of the duodenal wall; in such cases, sphincter action does not functionally affect the junction. As the hydropressure within the pancreatic duct is usually greater than in the bile duct, pancreatic juice frequently refluxes into the biliary duct (pancreatobiliary reflux) in PBM, resulting in the incidence of lesion in the wall of the common bile duct and dilatation of the common bile duct. Currently, most scholars tend to believe the ectopic hepatic diverticulum may cause the primitive common bile duct and common channel to become stretched, producing a long common bile duct and a long common channel. If growth of the epithelium in the common bile duct fails to keep up with the anomalous elongation during embryonic, the duct may become attenuated, resulting in stenosis and weakness of the wall, and eventually dilatation. In treatment, the cyst excision with Roux-en-Y hepatoenterostomy is the standard surgical method for choledochal cyst. To reduce the post-operative complications, doctors need to learn about the changes of the biliary system, deal with the abnormality in the intrahepatic bile duct, pancreatic duct and common channel. Laparoscopic surgery has become a successful method in treatment of choledochal cyst.     

Pathology of the anomalous choledocho-pancreatic junction--action of pancreatic juice on the biliary tract

The mechanism of action of pancreatic juice on the biliary tract was investigated in a 5-year-old girl with choledochal cyst and animal models of the anomalous choledocho-pancreatic junction produced by pancreatico-cholecystostomy in 26 dogs. The results are as follows: Pancreatic enzymes are activated when bile and pancreatic juice are mixed in the biliary tract. Successively, lysolecithin and free fatty acids are produced by hydrolysis of lecithin in bile with phospholipase A2. These substances including phospholipase A2 destroy the mucosal barrier. The initial site receiving mucosal damage appears to be the epithelial apical cell membrane. The transfer of phospholipase A2 but not amylase from the biliary tract to the blood stream was suggested by comparing enzyme activity of portal and hepatic venous blood. The presence of some mutagenic substances in the contents of the choledochal cyst was confirmed by Ames assay and Rec-assay. This suggests that activated pancreatic enzymes and successively produced harmful substances in the anomalous choledocho-pancreatic junction are responsible for not only inflammatory but positively carcinogenic action on the biliary tract. In conclusion, the anomalous choledocho-pancreatic junction requires operative separation of the bile duct from the pancreatic duct despite the presence or absence of a choledochal cyst.     

109例成人型胆管囊肿的诊治经验

目的 探讨成人型胆管囊肿的临床特点和手术方式,总结胆管囊肿的诊治经验.方法 回顾性分析1984-2007年北京协和医院收治的109例成人型胆管囊肿的病例,总结病例特点.109例中男女之比为1:3.8.平均年龄32.8岁.Ⅰ型78例,Ⅳa型26例,Ⅴ型5例,没有Ⅱ、Ⅲ型病例.结果 成人型胆管囊肿临床表现以右上腹不适或疼痛占大多数,共100例(91.7%),其次为寒战、发热等发作性胆管炎症状.30例(27.5%)有过胆道系统手术史,26例初次手术在2000年前施行.B超结合MRCP是确诊的主要手段.94例行囊肿切除、肝管空肠Roux-Y吻合重建术,4例因肝内病变局限于左肝加做左肝外侧段切除术.4例囊肿恶变.无手术死亡病例.手术近期并发症有胰瘘、膈下脓肿.后期并发症主要是发作性胆管炎、吻合口狭窄和肝内胆管结石.结论 成人型胆管囊肿的临床表现不典型,以右上腹痛或不适最为多见.B超是初查的主要手段,为明确诊断、了解胆管囊肿分型和累及范围可首选MRCP.胆管囊肿理想的手术方式是囊肿切除、肝管空肠Roux-Y吻合重建术.     

胆总管囊肿伴肝总管狭窄患儿的腹腔镜手术治疗

目的探讨腹腔镜手术治疗胆总管囊肿伴有肝总管狭窄患儿的治疗方法。方法自2007年1月至2009年12月，共收治胆总管囊肿患儿41例，其中7例伴有肝总管狭窄的患儿均在腹腔镜下完成手术。采用四孔Trocars技术，先在腹腔镜下游离胆囊并将其提出腹壁外，行38％泛影葡胺造影，判断胆总管囊肿及肝总管的情况；电凝钩或超声刀游离切除囊肿；于脐孔处提出空肠行端侧吻合术；腹腔镜探查近端肝总管情况，将狭窄肝总管前壁向近端游离、切开、成形，并探查肝内胆管；行镜下胆肠端侧吻合术。结果7例胆总管囊肿伴有肝总管狭窄的患儿均在腹腔镜下完成手术，术中造影及腔镜探查证实囊肿近端肝总管狭窄，直径1～2mm，长度2mm左右，切开狭窄肝总管前壁至宽敞部，3例伴有肝总管结石，1例伴有蛋白栓，均游离近端肝总管行肝肠吻合。术后黄染、腹疼等症状消失，随访6个月至2年均无胆系感染或胆道梗阻发生。结论胆总管囊肿伴有肝总管狭窄的患儿在腹腔镜下行狭窄肝总管切开成形、胆肠吻合术，比开放性手术更容易操作，预后好，能够预防胆道梗阻及胆管炎的发生。     

Laparoscopic repositioning of an aberrant right hepatic artery and hepaticojejunostomy for pediatric choledochal cyst: A case report

IntroductionThe right hepatic artery crossing the ventral side of the common hepatic duct is a relatively frequent abnormality. This aberrant right hepatic artery not only interferes with dissection of the common bile duct and hepaticojejunostomy for choledochal cyst but can also cause postoperative anastomotic stenosis.Case presentationA 14-year-old patient presented with upper abdominal pain and was diagnosed with a choledochal cyst (Type IVA in Todani Classification) and pancreaticobiliary maljunction. Abdominal enhanced computed tomography showed aberrant right hepatic artery located at the ventral side of the common hepatic duct. Laparoscopic choledochal cyst resection and hepaticojejunostomy were planned. Intraoperative findings also showed the aberrant right hepatic artery crossing the common hepatic duct ventrally as detected on preoperative computed tomography. Laparoscopic dorsal side repositioning of the aberrant right hepatic artery was performed because it appeared to compress the common hepatic duct and risked causing postoperative anastomotic stenosis. We performed laparoscopic hepaticojejunostomy by replacing the aberrant right hepatic artery dorsally to facilitate suturing and prevent postoperative anastomotic stenosis. The postoperative course was uneventful, with no findings suggestive of anastomotic stenosis.DiscussionThe abnormality of the right hepatic artery is reported to be a primary cause of anastomotic stenosis after hepaticojejunostomy. Once anastomotic stenosis or stricture develops, it is often difficult to treat. The prevention of the stenosis is important.ConclusionsIn choledochal cyst with aberrant right hepatic artery, dorsal repositioning is effective for preventing postoperative anastomotic stenosis and cholestasis.     

Pancreas divisum, pancreatic pseudocyst, and choledochal cyst in an 8-year-old child

An 8-year-old American Indian girl presented with acute pancreatitis. Ultrasonography and computerized tomography (CT) showed two large cystic masses near the head of the pancreas. During 4 weeks of complete bowel rest and total parenteral nutrition, she had multiple exacerbations of pancreatitis without resolution of the cysts. There were no symptoms of biliary ductal obstruction. Endoscopic cholangiopancreatography (ERCP) showed an 8-cm pancreatic pseudocyst, pancreas divisum, and a 10-cm choledochal cyst. The junction of the common bile duct and the pancreatic duct of Wirsung had a normal relationship. She underwent a Roux-en-Y pseudocyst-jejunostomy and an accessory sphincteroplasty. Three months later, a choledochal cyst excision was performed with biliary reconstruction. The patient is well and asymptomatic 1 year later. This case suggests that pancreatic juice reflux into the common bile duct may not be the only etiology of choledochal cyst formation.     

Development of intrahepatic biliary stones after excision of choledochal cysts

Background: The incidence of intrahepatic cholelithiasis and cholangitis has not yet been well studied postoperatively in patients with choledochal cysts. Methods: One hundred three patients with choledochal cysts had operative cholangiography, underwent standard excision of a choledochal cyst with Roux-en-Y hepatico-jejunal anastomosis, and were at a mean follow-up of 12[frac12] years. The incidence of intrahepatic bile duct stones was analyzed according to the 3 morphologic types of intrahepatic bile duct observed at initial operative cholangiography: type 1, no dilatation of the intrahepatic bile ducts; type 2, dilatation of the intrahepatic bile ducts but without any downstream stenosis; and type 3, dilatation of the intrahepatic bile ducts associated with downstream stenosis. Initially, there was no evidence of intrahepatic bile duct stones in any of the 103 patients. Results: Among 50 type 1 patients, intrahepatic cholelithiasis developed in only 1 patient (2%). Among 43 type 2 patients, 1 patient (2%) had intrahepatic cholelithiasis, and 2 (5%) had postoperative cholangitis. Among 10 type 3 patients, 4 (40%) had intrahepatic cholelithiasis (P [lt ] .01), and 3 (30%) had postoperative cholangitis. Time intervals between the initial surgery and the first identification of intrahepatic stones ranged from 3 to 22 years. Conclusions: One of the major causes of formation of intrahepatic cholelithiasis has been clarified; patients with intrahepatic biliary dilatation with downstream stenosis can get intrahepatic bile duct stones long after excision of a choledochal cyst.     

1098例胆管扩张症的病因构成及分类

目的 探讨胆管扩张症的病因构成及分类,提高诊断水平。方法回顾性分析2000年1月至2009年12月收治的1098例胆管扩张症的临床资料。结果 先天性胆管囊肿69例(6.3％),继发性胆管扩张1029例(93.7％)。22种病因中,排在前5位的病因分别为胆管结石(366例,33.3％)、胰头癌（137例,12.5％）、壶腹周围癌（122例,11.1％）、胆管癌（68例,6.2％）、慢性胰腺炎或胰头部囊肿（62例,5.6％）。结论胆管扩张症可分先天性和继发性两大类,先天性胆管囊肿约占6.0％;继发性胆管扩张占绝大多数（约占94.0％）。后者又可以分压力性、撑开性、压迫性、感染性、代偿性、粘连性六种扩张类型。最常见的病因是胆管结石、胰头癌和壶腹周围癌。     

Choledochal cyst. Etiological considerations and surgical management in 22 cases

In a 14-year period from 1962 to 1976, there were 22 cases of choledochal cyst treated by two different approaches: choledochocystenterostomy to the duodenum in three and to the jejunum in a Roux-en-Y operation in four cases, and excision of the cyst with hepaticojejunostomy in 14 and with end-to-end anastomosis of the common bile duct in one case. There was one death in each group. In five cases, direct cholangiography, either operative or endoscopic, demonstrated a reflux of the contrast material from the biliary system to the pancreatic duct. In four cases, the amylase level of the cystic content was elevated, ranging from 182 to 50,820 Somogyi units. A reflux of the pancreatic juice into the biliary system was thought to be a possible cause of choledochal cyst. Excision of the cyst with hepaticojejunostomy in a Roux-en-Y operation seems to be the treatment of choice from the aspects of cholangitis, malignant change, and termination of the pancreatic reflux to the biliary system.     

Surgery of coexisting biliary malformations in choledochal cyst

Five of 13 infants and children with a choledochal cyst had coexisting biliary malformations. The specific lesions were main hepatic duct cyst (2), common hepatic duct stenosis (1), left hepatic duct stenosis (1), and Caroli's disease (1). The malformations were corrected or partially corrected at the time of operative resection of the cyst. The lateral portion of either or both of the major hepatic ducts was incised to the liver hilus thereby dividing areas of stenosis or laying open cystic malformations. Biliary reconstruction was by Roux-en-Y jejunostomy to the fileted common hepatic bile duct. The opportunity to correct coexisting biliary malformations is another advantage of total excision of choledochal cysts.