A rare malformation of the pancreaticobiliary junction long common channel choledochal cyst and pancreas divisum in a patient with pancreatic cancer.

Congenital variants of the pancreaticobiliary junction are rare anomalies that are usually diagnosed in childhood because of recurrent abdominal pain and jaundice. These lesions are associated with several pancreaticobiliary diseases including pancreatitis and malignancy. We observed a rare anomaly of the pancreaticobiliary tract with a combination of several ductal malformations, i.e., choledochal cyst, long common channel and incomplete pancreas divisum in a patient with pancreatic cancer.     

Endoscopic therapy in anomalous pancreatobiliary duct junction.

BACKGROUND: Anomalous pancreaticobiliary duct junction is associated with bile duct strictures, pancreatitis, choledochal cysts, and biliary carcinoma. Limited data are available for outcomes of endoscopic therapy. METHODS: Review of 7537 patients undergoing endoscopic retrograde cholangiopancreatography from 1988 to 1997 yielded 18 patients with anomalous pancreaticobiliary duct junction. Therapeutic responses were tallied by chart review and phone calls. RESULTS: There were 13 women and 5 men, with a mean age of 36 years. Twelve patients had no ductographic evidence of pancreatitis and 6 had chronic pancreatitis. Seven had choledochal cysts. Fifteen patients (83%) underwent endoscopic biliary sphincterotomy, two of whom underwent repeat endoscopic biliary sphincterotomy for recurrence of symptoms. The other therapies included stent placement for benign biliary strictures in 5 patients, lithotripsy of pancreatic stones in 1 patient, and choledochal cyst removal in 4 patients. Three cases with malignant biliary strictures are excluded from endoscopic outcome studies. The 12 patients with pancreatitis had a mean of 2.0 episodes per year before any treatment. After endoscopic therapy 7 patients had no further episodes of pancreatitis, whereas 5 patients had further episodes, with a mean of one additional attack per year, over 3 years mean follow-up. CONCLUSIONS: Patients with anomalous pancreaticobiliary duct junction have complex pathology associated with strictures, choledochal cysts, pancreatitis, and malignancies. Endoscopic therapy appeared to benefit 13 of 15 patients without malignant disease with elimination of or decreased frequency of pancreatitis. Endoscopic therapy appears to be a logical first step in the management of most symptomatic patients with anomalous pancreaticobiliary duct junction.     

A case of diverticular form type of congenital choledochal dilatation with anomalous arrangement of pancreaticobiliary duct

We report a case of diverticular form type of congenital choledochal dilatation with anomalous arrangement of pancreaticobiliary duct and bile duct stone. The patient was a 63-year-old woman with the chief complaint of epigastralgia. Abdominal CT showed low density area suggesting cystic lesion at the region of pancreatic head. MRCP and ERCP revealed dilatation of the common bile duct in the multiple diverticular form with anomalous arrangement of pancreaticobiliary duct. A 2.0cm sized stone was also recognized in the dilated common bile duct. The patient underwent resection of gall bladder and dilated common bile duct, followed by hepatico-jejunostomy. Histological findings did not revealed malignant changes in the mucosa of both gall bladder and dilated common bile duct.     

Mucinous cystadenoma of the pancreas 17 years after excision of gallbladder because of a choledochal cyst

A 56-year-old woman who had undergone excision of the gallbladder because of a choledochal cyst had a tumorous lesion of the pancreas identified by upper abdominal ultrasonography, but an operation was not carried out, because there was no apparent increase in the cystic mass and no elevation of serum tumor markers. In October 2001, she was admitted to our hospital to check for malignancy because of elevated levels of the tumor marker Dupan-2. Abdominal enhanced computed tomography and upper abdominal ultrasonography revealed a large multilocular cystic mass in the body to tail of the pancreas. Endoscopic retrograde cholangiopancreatography showed elongation of the common duct that communicates with the common bile duct and the main pancreatic duct, indicating an anomalous arrangement of the biliary and pancreatic duct system. No apparent communications between the cystic mass and the main pancreatic duct were observed. In January 2002, the patient underwent a spleen-preserving distal pancreatectomy, and histopathological and immunohistochemical examinations led to the diagnosis of pancreatic mucinous cystadenoma with ovarian-like stroma. The mucinous cystadenoma was detected 17 years after the operation for the choledochal cyst. To the best of our knowledge, no documented case reports of mucinous cystadenoma of the pancreas associated with a choledocal cyst have been reported to date. We present here the first case report of pancreatic mucinous cystadenoma occurring in the body to tail of the pancreas, associated with a choledocal cyst.     

Pancreatic ductal morphological pattern and dilatation in postoperative abdominal pain in patients with congenital choledochal cyst: an analysis of postoperative pancreatograms

BACKGROUND: Postoperative abdominal pain associated with pancreatitis has been reported as a pancreas-associated complication after total biliary excision. The aim of the present study was to determine pancreatic ductal patterns in the head of the pancreas and evaluate pancreatic ductal dilatation in patients presenting with postoperative abdominal pain after radical biliary excision for congenital choledochal cyst. METHODS: Postoperative endoscopic retrograde pancreatography (ERP) was performed in a total of 38 patients (27 female and 11 male) with a history of postoperative abdominal pain or an increase in serum and urinary amylase on laboratory analysis. Pancreatic ductal configuration and ductal dilatation were determined. RESULTS: Pancreatic configurations included the usual type (n = 20), ansa pancreatica type (n = 11), loop type (n = 5), and divisum type (n = 2). Dilatation of the pancreatic duct was observed in 34 patients. The common channel was dilated in 29 patients. Mild postoperative pancreatitis occurred in 4 patients in the long term. Protein plugs were observed in the dilated pancreatic duct. CONCLUSIONS: Pancreatic ductal morphological pattern and ductal dilatation, possibly caused by long-standing stagnation of the pancreatic juice, may be associated with postoperative abdominal pain and pancreatitis in congenital choledochal cyst.     

A case of intrahepatic cholangiocarcinoma associated with Type IV choledochal cyst

Anomalous union of the pancreaticobiliary duct (AUPBD) is a congenital anomaly that is defined as a junction of the bile duct and pancreatic duct outside the duodenal wall. This anomaly results in a loss of normal sphincteric mechanisms at the pancreaticobiliary junction. As a result, regurgitation of pancreatic juice into the biliary system develops and causes choledochal cysts, choledocholithiasis, cholangitis, pancreatitis and malignancy of the biliary tract. Gallbladder cancer or common bile duct cancer associated with AUPBD and choledochal cysts have been frequently reported. But, intrahepatic cholangiocarcinoma associated with this condition has been only rarely reported. Here, we report a case of intrahepatic cholangiocarcinoma associated with AUPBD and choledochal cyst.     

Bile duct schwannoma developed in the remnant choledochal cyst—a case associated with total agenesis of the dorsal pancreas

A 59-year-old Japanese woman was referred to our hospital due to upper abdominal pain. At the age of 44, she was diagnosed with a congenital choledochal cyst, Todani's type Ic. She then underwent bypass operation with end-to-side choledochojejunostomy with Roux-en-Y technique as well as cholecystectomy. Magnetic resonance cholangiopancreatography revealed an 'oval' shaped cystic lesion with a maximal diameter of 25 mm, which had been 'spindle' shaped with a maximal diameter of 18 mm, 15 years ago. It also showed an anomalous pancreaticobiliary ductal union. In addition, a complete absence of the dorsal primordia of pancreas was revealed by magnetic resonance image and computed tomography scan. The patient underwent the surgical exploration for the resection of the 'oval' shaped cystic lesion. Haematoxylin and eosin staining of the thin section of the resected cyst showed a compact spindle cell pattern which was compatible with schwannoma, Antoni type A, which was confirmed by immunocytochemical technique. We present a very interesting case showing choledochal cyst, anomalous pancreaticobiliary ductal union, total agenesis of the dorsal pancreas and late-development of bile duct schwannoma in the remnant choledochal cyst.     

Pancreatic stone predominantly composed of fatty acid calcium.

A 41-year-old man with chronic pancreatitis and pancreatic stones predominantly composed of fatty acid calcium is reported. He had complained of occasional abdominal pain for 10 years and visited the hospital because of a severe attack of abdominal pain. Laboratory data supported a diagnosis of pancreatitis. Computed tomography (CT) showed a high-density area in the head of the pancreas, and the CT number of this high-density area was lower than usual for pancreatic stones. Ultrasonography and endoscopic retrograde pancreatography showed a cystic lesion with small pancreatic stones in the head of the pancreas and irregular dilatation of the main pancreatic duct. Pancreaticojejunostomy and resection of pancreatic cyst were carried out for repeated episodes of abdominal pain under the diagnosis of chronic pancreatitis. The pancreatic stones obtained at surgery were proved to be mainly composed of fatty acid calcium after analysis of chemical composition of the stones. Fatty acid calcium was sometimes found in the biliary stones but never in the pancreatic stones.     

Squamous Cell Carcinoma Arising Within a Choledochal Cyst

Choledochal cysts are rare congenital or acquired cystic dilatations of the intra- or extrahepatic bile ducts. Their exact pathophysiology remains unclear. Extensive complications exist with untreated cysts. including biliary stasis and inflammation, with resultant stricture and stone formation, cholangitis, pancreatitis, and malignant degeneration. Most commonly reported malignancies include cholangiocarcinoma, adenocarcinoma, and gallbladder cancer. We report the case of a 41-year-old female with a history of chronic calcific pancreatitis, choledocholithiasis, and a large type I choledochal cyst, who presented with flu-like symptoms followed by painless jaundice and weight loss. Endoscopic retrograde cholangiopancreatography and direct cholangioscopy revealed a mass at the biliary bifurcation, a 4-cm choledochal cyst with multiple calculi, absence of anomalous pancreaticobiliary ductal union, and multiple calcifications in the pancreatic head. Pathology demonstrated a synchronous choledochal cyst and squamous cell carcinoma, an infrequently reported phenomenon. We report the associated rare finding of chronic calcific pancreatitis, without anomaly of the pancreatic biliary junction. This suggests that the formation of calculi within the choledochal cyst may contribute to chronic calcific pancreatitis, possibly by virtue of papillary stenosis.     

A Case Report of Anomalous Pancreaticobiliary Duct Connection with Choledocholithiasis and Acute Pancreatitis

Abstract: A 25-year-old man was admitted to hospital with epigastric pain. He had had a history of episodic abdominal pain since early childhood. An anomalous pancreaticobiliary duct connection was seen by endoscopic retrograde cholangiopancreatography. In many cases, this type of abnormality is caused by an anomaly in the ventral pancreas. In this case, however, the common bile duct, with calculi, was joined to the pancreatic duct which did not arise from the ventral pancreas but from the dorsal pancreas. The pancreatic duct arising from the ventral pancreas was absent in this case. The patient underwent a prophylactic cholecystectomy, a transduodenal sphincteroplasty, a choledocholithotomy, a partial resection of the common bile duct, and a hepaticojejunostomy, performed by a Roux-en-Y anastomosis. His postoperative recovery was satisfactory. An anomalous pancreatobiliary duct connection allows pancreatic juices and bile to mix. This is considered to be an etiological factor in pancreatitis and choledocholithiasis.     

Duodenum-preserving head resection in chronic pancreatitis

A duodenum-preserving head resection was performed in 295 patients with chronic pancreatitis and an inflammatory mass in the head of the pancreas. Ninety-four percent of patients suffered severe pain syndrome, 48% had a common bile duct stenosis, 17% a vascular obstruction in the portal vein and splenic vein branches, and 6% had a severe stenosis of the duodenum. Surgical resection of the inflammatory mass in the head of the pancreas was indicated after a medical treatment of 4.1 years (median). Subtotal resection of the head of the pancreas, including the inflammatory mass, resulted in decompression of the narrowed common bile duct segment, decompression of the pancreatic main duct, and the relief of duodenum stenosis, as well as a relief of portal hypertension. The mean hospitalization time was 13 days, frequency of re-operation 5.8%, and hospital mortality 1.02%. Seventy-nine percent of patients experienced long-lasting pain relief and 11% reported a significant and long-lasting reduction of pain; late morbidity proved to be low. In comparison to the Whipple procedure the duodenum-preserving head resection has the advantage of preserving the stomach, duodenum and biliary tract.     

Pancreatic carcinoma associated with anomalous pancreaticobiliary junction

Anomalous pancreaticobiliary junction often leads to biliary tract carcinoma but only rarely to pancreatic carcinoma. We report three cases of pancreatic carcinoma associated with anomalous pancreaticobiliary junction. All three were female with a mean age of 68 years. Carcinomas were located in the pancreatic head (n = 2) or body (n = 1). None had choledochal cyst and one had experienced recurrent acute pancreatitis. All carcinomas were at an advanced stage with a poor prognosis. No unique imaging or histologic findings of the carcinomas could be identified. Attention should be paid to the possibility of pancreatic carcinoma in patients with anomalous pancreaticobiliary junction, particularly in aged patients. Early diagnosis and treatment of anomalous pancreaticobiliary junction may prevent development of pancreatic carcinoma.     

Spontaneous perforation of right hepatic duct in a child with choledochal cyst

A 5-year-old girl with choledochal cyst (Alonso Lej, type I) and spontaneous perforation of the right hepatic duct during an attack of acute pancreatitis was treated by two-stage surgery. Biliary panperitonitis was diagnosed by abdominal paracentesis, bilirubin and amylase levels in the collected fluid being 41.3 mg/dl and 1812 Somogyi U, respectively. The initial surgery involved simple peritoneal drainage and decompression of the bile duct, followed by removal of a choledochal cyst and the common hepatic bile duct, including the biliary stricture of the left hepatic duct, and biliary reconstruction 5 months later. Cholangiogram after the initial surgery demonstrated pancreaticobiliary maljunction and stricture in the left hepatic duct and the common hepatic duct. Regurgitation of pancreatic juice into the bile duct was shown by the extremely high amylase level (90100 Somogyi U) in the bile drained through a T-tube. The patient is currently well, with normal liver and pancreatic function tests. The experience in this patient is significant in that it should be known that spontaneous perforation of the bile duct may occur at the right hepatic duct in choledochal cyst, causing biliary peritonitis.     

无先天性胆管囊性扩张型的胰胆管汇流异常与胆胰疾病的关系

目的：研究无先天性胆管囊性扩张型的胰胆管汇流异常（APBJ）及其与与胆胰疾病的关系。方法：分析1995年1月－2002年1月间逆行胰胆管造影（ERCP）、术中胆道造影（IOC）、磁共振胰管造影（MRCP）等影像学检查的2150例胆胰疾病中的52例无先天性胆管囊性扩张型APBJ及其与胆胰疾病的关系。结果：本组APBJ的发生率为2．4％，APBJ的检查发现率为ERCP2．9％，IOC2．4％、MRCP2．0％，在52例无先天性胆管囊性扩张型APBJ中有P－C型（胰管注入胆总管）32例、C－P型（胆总管汇入胰管）20例。胆囊上皮增生的发生率为51．9％，胆囊结石的发生率为73．1％，胆囊息肉样病变的发生率为26．9％，急性胰腺炎的发生率为34．6％，结论：APBJ与胆囊疾病关系密切，无先天性胆管囊性扩张型APBJ的胆囊上皮增生和胆囊息肉样病变发生率高，是胆囊癌发生的一个高危因素，对此类型APBJ病人主张预防性胆囊切除，APBJ是胰腺炎发病的一个重要因素，C－P型APBJ的胰腺炎发生率高，在反复胰腺炎发作而无明显原因时，应考虑有APBJ的存在。     

Inferior head resection of the pancreas for noninvasive intraductal papillary mucinous adenocarcinoma

We report inferior head resection of the pancreas for noninvasive intraductal papillary mucinous cancer. A 77-year-old man was admitted to our hospital complaining of epigastric pain. Contrast-enhanced computed tomographic scan and magnetic resonance imaging showed a multilocular cystic tumor 3.5 cm in diameter in the head of the pancreas. We performed inferior head resection of the pancreas with partial resection of the duct of Santorini. The duodenum, the common bile duct, and most of the duct of Santorini were preserved. He is presently alive and well without any evidence of recurrent disease 49 months after the operation. Partial pancreatic resection has the advantage of preserving pancreatic parenchyma. Inferior head resection of the pancreas would play an important role in surgical management of low-grade malignant neoplasm.     

Pancreatitis associated with pancreaticobiliary maljunction

BACKGROUND/AIMS: Pancreaticobiliary maljunction is a rare anomaly, but causes various pathological conditions in the biliary tract and the pancreas. This study aims at clarifying the features of pancreatitis associated with pancreaticobiliary maljunction. METHODOLOGY: A total of 100 patients with pancreaticobiliary maljunction were reviewed. Clinical findings and cholangiopancreatographic results in patients with acute or chronic pancreatitis associated with pancreaticobiliary maljunction were analyzed. RESULTS: Of 100 patients, 14 had pancreatic disorders: acute pancreatitis (n = 3), chronic pancreatitis (n = 5), hyperamylasemia (n = 4), and pancreatic carcinoma (n = 2). The acute pancreatitis was mild (n = 3) and relapsing (n = 2). In patients with chronic pancreatitis, pancreatic stones (n = 2) and radiolucent protein plugs (n = 2) were detected only in the dilated common channel or in the main pancreatic duct near the common channel. Two patients received cyst-duodenostomy in the infant developed chronic pancreatitis 11 and 27 years later. CONCLUSIONS: Acute or chronic pancreatitis was sometimes associated with pancreaticobiliary maljunction. These pancreatitis cases showed different clinical and pancreatographic findings from others. These differences might be due to the peculiar mechanism that they were induced by bile reflux into the pancreatic duct via the anomalous connection.     

Pancreatitis associated with congenital abnormalities of the pancreaticobiliary system

BACKGROUND/AIMS: The relationship between pancreas divisum and pancreatitis remains controversial. The aim of this study was to examine the frequency and features of pancreatitis associated with congenital abnormalities of the pancreaticobiliary system, and the role that these malformations have in the occurrence of pancreatitis. METHODOLOGY: We retrospectively examined endoscopic retrograde cholangiopancreatograms of 6850 patients including 186 patients who were diagnosed clinically as having acute pancreatitis and 211 who were diagnosed as having chronic pancreatitis. RESULTS: A total of 175 patients were diagnosed as having congenital abnormalities of pancreaticobiliary system. Acute and chronic pancreatitis was associated respectively with 3.0% and 5.0% of patients with pancreaticobiliary maljunction (n=100), 17.1% and 28.6% of those with pancreas divisum (n=35), 6.6% and 20.0% of those with partial pancreas divisum (n=30), and 16.7% and 33.3% of those with choledochocele (n=6). These anomalies were present in 6.5% and 10.9% of patients with acute and chronic pancreatitis, respectively. Isolated chronic dorsal pancreatitis was detected in 8 patients with pancreas divisum. In chronic pancreatitis associated with pancreaticobiliary maljunction, pancreatic stones or protein plugs were situated only in the dilated common channel or main pancreatic duct of the head. CONCLUSIONS: Congenital abnormalities of the pancreaticobiliary system constitute one etiology of pancreatitis.     

Clinicopathological studies of anomalous arrangement of the pancreaticobiliary ductal system with pancreas divisum

We examined the morphological aspects of the pancreaticobiliary ductal system in 13 patients with both anomalous arrangement of the pancreaticobiliary ductal system (AAPB) and associated pancreas divisum (PD), and compared their clinicopathological findings with those of patients with either AAPB or PD alone. PD is classified into three types, i.e., separate pancreas, nonfusion of the ventral and dorsal pancreatic ducts, and partial fusion of the ventral and dorsal pancreatic ducts. Of the 13 patients with AAPB and associated PD, 5 were male and 8 female; their mean age was 57 years. Nine of the 13 had clinical symptoms. Five of the 13 had gallbladder cancer, 3 had cholecystolithiasis, and 2 choledochal cyst. One patient showed nonfusion of both pancreatic ducts and the others showed partial fusion of the ducts. The length of the common duct from the orifice of the papilla of Vater to the junction of the common bile duct with the ventral pancreatic duct and the location of the union varied. The presence of both AAPB and PD made the arrangement of the pancreaticobiliary ducts very complicated. Clinical aspects were similar to those of AAPB and the patients were likely to be treated for a condition other than PD. However, since relapsing acute pancreatitis and intermittent epigastric pain are typical symptoms in patients with AAPB as well as in those with PD, we should carefully follow up those patients with both AAPB and PD.     

Long-term survival with carcinoma arising from a congenital choledochal cyst

We report a case of 11-year survival with carcinoma arising from a congenital choledochal cyst. A 34-year-old Japanese woman was admitted due to epigastric discomfort. Ultrasonography revealed a choledochal cyst involving a polypoid tumor. Endoscopic retrograde cholangiopancreatography revealed pancreaticobiliary maljunction in which the main pancreatic duct joined the dilated common bile duct 2 cm above the papilla of Vater. A choledochal cyst containing a polypoid tumor was resected with the regional lymph nodes. Grossly, a 2×2-cm papillary tumor was noted in the posterior wall of the choledochal cyst. Microscopically, the tumor was well-differentiated tubular adenocarcinoma invading the mucosa and fibromuscular layer. Neither anticancer chemotherapy nor radiotherapy was performed. The patient recovered uneventfully after the operation and has been well for 11 years without evidence of recurrent cancer. The good results in this patient may have been due to the early stage of the cancer.     

Association of anomalous pancreaticobiliary ductal junction with gallbladder carcinoma in Chinese patients: an ERCP study

BACKGROUND: Anomalous pancreaticobiliary ductal junction, a rare congenital anomaly, is associated with various biliary and pancreatic diseases. The aim of this study was to determine the frequency of anomalous pancreaticobiliary ductal junction in Chinese patients with gallbladder cancer. METHODS: One thousand eight hundred seventy-six patients underwent ERCP between April 2000 and September 2001 with biliary and pancreatic duct opacification in 1082. Among the latter patients, those with proven gallbladder carcinoma were identified. Anomalous pancreaticobiliary ductal junction was defined as a common channel greater than 15 mm in length or a contractile segment totally distal to the union of the biliary and pancreatic ducts. When the common bile duct appeared to join the main pancreatic duct, the anomalous pancreaticobiliary ductal junction was denoted as B-P subtype; if the main pancreatic duct appeared to join the common bile duct, it was denoted P-B subtype. RESULTS: Fifty-four patients had gallbladder carcinoma, 7 of whom (3 men, 4 women) had anomalous pancreaticobiliary ductal junction (P-B subtype 6, B-P subtype 1). The mean (SD) length of the common channel was 21.0 mm (11.2 mm) with a range of 12 to 45 mm. One patient had early cystic dilation of bile duct. Three other patients had anomalous pancreaticobiliary ductal junction; 1 had an associated choledochal cyst and 2 a normal biliary tree. The overall frequency of anomalous pancreaticobiliary ductal junction was 0.9% (10/1082 cases). The frequency of anomalous pancreaticobiliary ductal junction was significantly higher in patients with gallbladder carcinoma (p < 0.001; OR, 50.7; 95% CI [12.7, 202.3]). CONCLUSIONS: Anomalous pancreaticobiliary ductal junction is strongly associated with gallbladder cancer among Chinese patients.