Intraductal papillary-mucinous adenoma developed in the ventral pancreas in a patient with pancreas divisum

A 34-year-old man was admitted to our hospital with the chief complaints of back pain and epigastralgia. The physical examinations on admission disclosed no abdominal tumor. The serum concentration of total bilirubin was 1.4?mg/dl. The serum elastase-1 level was elevated to 526?ng/dl. Computed tomography showed a cystic lesion, 1?cm in diameter, in the head of the pancreas, without dilatation of the main pancreatic duct. Endoscopic retrograde cholangiopancreatography via the papilla of Vater and the accessory papilla revealed an enlarged ventral pancreatic duct and pancreas divisum. The preoperative diagnosis was mucin-producing pancreatic tumor in the ventral pancreas of a patient with pancreas divisum. A pylorus-preserving pancreatoduodenectomy was performed. The gross findings of the cut surface of the resected specimen disclosed mural nodules in the dilated duct of the ventral pancreas. A histological examination of the mural nodules in the ventral pancreas revealed mucin and intraductal papillary adenoma. Benign tumors associated with pancreas divisum are rare; to the best of our knowledge, only three cases have been reported. Although in these three patients the tumor developed in the dorsal pancreas, the tumor developed in the ventral pancreas in our patient.     

Pancreatic tumor associated with pancreas divisum

BACKGROUND: Although there has been considerable controversy regarding pancreas divisum and pancreatitis, little discussion of this has taken place. The purpose of the present paper was to investigate the relationship between these two conditions. METHODS: A retrospective investigation was undertaken of pancreatic tumors associated with pancreas divisum, in 650 cases of pancreatic carcinoma, 80 cases of intraductal papillary mucinous tumor of the pancreas and 32 cases of pancreas divisum. RESULTS: Of these 32 cases, four (12.5%) were associated with pancreatic tumor: pancreatic carcinoma (n = 3) and intraductal papillary mucinous tumor (n = 1). All tumors developed from the dorsal pancreas of pancreas divisum. Periductal and interlobular fibrosis detected in the non-carcinomatous pancreas of the margin of distal pancreatectomy implied that chronic dorsal pancreatitis associated with pancreas divisum preceded carcinoma. CONCLUSIONS: Pancreatic tumors were detected in 12.5% of cases of pancreas divisum. In pancreas divisum, longstanding pancreatic duct obstruction caused by relative stenosis of the minor duodenal papilla might be a factor promoting oncogenesis.     

Clinical significance of the minor duodenal papilla and accessory pancreatic duct

The accessory pancreatic duct (APD) is the main drainage duct of the dorsal pancreatic bud in the embryo, entering the duodenum at the minor duodenal papilla (MIP). As development progresses, the duct of the dorsal bud undergoes varying degrees of atrophy at the duodenal end. In cases of patent APD, smooth-muscle fiber bundles derived from the duodenal proper muscular tunics surround the APD. The APD shows long and short patterns on pancreatography, and ductal fusion in the two types appears to differ embryologically. Patency of the APD in control cases, as determined by dye-injection endoscopic retrograde pancreatography, was 43%. Patency of the APD may depend on duct caliber, course, and terminal shape of the APD. A patent APD may prevent acute pancreatitis by reducing the pressure in the main pancreatic duct. Pancreas divisum is a common anatomical anomaly in which the ventral and dorsal pancreatic ducts do not unite embryologically. As the majority of exocrine flow is routed through the MIP in individuals with pancreas divisum, interrelationships between poor function of the MIP and increased flow of pancreatic juice caused by alcohol or diet may increase dorsal pancreatic duct pressure and lead to the development of pancreatitis. Wire-guided minor sphincterotomy, followed by dorsal duct stenting, is recommended for acute recurrent pancreatitis associated with pancreas divisum.     

Intraductal papillary-mucinous tumor of the pancreas head with complete absence of the ventral pancreatic duct of Wirsung

A case of intraductal papillary mucinous tumor of the pancreas with complete absence of the ventral pancreatic duct of Wirsung is presented. A 74-year-old Japanese man was admitted to our hospital because of elevated serum amylase concentration. Abdominal computed tomography (CT) scanning revealed diffuse dilatation of the main pancreatic duct and a diffuse and uncircumscribed area with heterogeneous density in the pancreas head. Endoscopic retrograde cholangiopancreatography revealed that the main pancreatic duct was connected with an accessory papilla and was diffusely dilated, without any irregularity of the duct wall being observed in the entire length of the duct. The common bile duct was detected only by cannulation through Vater’s papilla, and no pancreatic duct or its communicating branch was found. Some branches, directed to the dorsal portion of the pancreas head, were found arising from the accessory pancreatic duct. Intraductal ultrasound examination performed through the accessory papilla and the common bile duct revealed a small tumor with a heterogeneous echo level in the pancreas head. From these findings, intraductal papillary-mucinous tumor (IPMT) occurring in the pancreas head was diagnosed, and pylorus-preserving pancreaticoduodenectomy was performed. The resected specimen revealed IPMT in the pancreas head. A roentgenographic study of the resected specimen revealed a defect caused by the tumor located in the pancreatic duct connected with the accessory papilla and showed that there was complete absence of the pancreatic duct connected with Vater’s papilla. Surgical resection enabled us to completely analyze the duct system of pancreas divisum. Although it is not known whether there is a relationship between the pathogenesis of IPMT and embryological anomaly of the pancreatic duct system, this case may provide an insight into the pathogenesis of IPMT.     

A giant retention cyst of the pancreas (cystic dilatation of dorsal pancreatic duct) associated with pancreas divisum

We describe a rare case of pancreas divisum associated with a giant retention cyst (cystic dilatation of the dorsal pancreatic duct), presumably formed following obstruction of the minor papilla. The patient was treated by pancreatico(cysto)jejunostomy. A 50-year-old man was admitted with complaints of increasing upper abdominal distension and body weight loss. There was no previous history of pancreatitis, gallstones, drinking, or abdominal injury. An elastic-hard tumor-like resistance was palpable in the upper abdomen. Computed tomography and ultrasound (US) examinations revealed a giant cystic lesion expanding from the pancreas head to the tail. Endoscopic retrograde cholangiopancreatography findings showed a looping pancreatic duct which drained only the head and uncinate process of the pancreas to the main papilla. A US-guided puncture to the cystic lesion revealed that the lesion continued to the main pancreatic duct in the tail of pancreas. The lesion was connected to a small cystic lesion, which was located inside the minor papilla, and ended there. The amylase level in liquid aspirated from the cyst was 37 869 IU/l, and the result of cytological examination of the liquid showed class II. A pancreatico(cysto)jejunostomy was performed, with the diagnosis being pancreas divisum associated with a retention cyst following obstruction of the minor papilla. The histological findings of a specimen from the cyst wall revealed that the wall was a pancreatic duct covered with mildly inflammatory duct epithelium; there was no evidence of neoplasm. The patient is currently well, and a CT examination 2 years after the operation showed disappearance of the cyst and normal appearance of the whole pancreas. Received: April 24, 2001 / Accepted: September 14, 2001     

Annular pancreas: a clinical, endoscopic, and immunohistochemical study.

A patient with annular pancreas presenting with severe upper abdominal pain is discussed. Endoscopic retrograde cholangiopancreatography (ERCP) was diagnostic, with successful injection of major and minor papillae showing pancreas divisum, an annular duct emptying at the major papilla and changes of severe chronic pancreatitis in all duct systems. Pylorus preserving pancreatoduodenectomy gave complete pain relief. The annulus was shown immunohistochemically to be entirely of ventral gland origin. Chronic pancreatitis was histologically less severe in the dorsal gland. Antegrade dye injection with x-ray showed dorsal to ventral connection in the resected specimen.     

Mucin-producing adenoma associated with pancreas divisum and hepatic hilar carcinoma: An autopsy case

We present the autopsy case of an 82-year-old Japanese woman with a mucin-producing adenoma accompanied by pancreas divisum and a hepatic hilar carcinoma. She had suffered from a cholangiocellular carcinoma at the hepatic hilus for 2 months, which was treated with radiation and chemotherapy. She did not complain of any abdominal pain. Obstructive jaundice deteriorated despite percutaneous transhepatic bile duct drainage, and she died of hepatic insufficiency. At autopsy, a hepatic tumor was confirmed to have caused severe obstructive jaundice. Histological examinations showed moderately to poorly differentiated cholangiocellular adenocarcinoma with squamous metaplasia, probably due to radiation. A yellowish mucinous tumor was found in the head of the pancreas near the minor papilla. It consisted of multiple rice-sized cystic lesions with thin septa. Although it had no capsule, its margin was clear. Neither a wide opening of the major or minor papilla nor mucous drainage was observed. Gross examinations revealed unfused pancreatic ducts. The slightly dilated dorsal duct and a branch of the mildly dilatated ventral duct showed tumor involvement. Histological examinations showed mild atypia of the epithelia, and this pancreatic tumor was diagnosed as branch duct-type mucin-producing adenoma with postradiation dysplasia. The combination of a mucin-producing tumor and pancreas divisum is rare, and this is only the third reported case.     

Adenoma of the minor papilla associated with pancreas divisum

Tumors of the minor papilla of the duodenum are quite rare. We report the first documented case of an adenoma of the minor papilla complicating pancreas divisum. A 52-year-old woman was admitted to our hospital for treatment of an asymptomatic duodenal tumor detected by computed tomography scan. Endoscopy showed an 18-mm, whitish-colored, sessile mass located in the descending duodenum proximal to a normal appearing major papilla. Endoscopic retrograde pancreatography revealed divisum of the pancreas with dilatation of pancreatic duct ranged in the dorsal pancreas. Transduodenal minor papillectomy was performed because there is malignant potential of the tumor and the possibility of acute pancreatitis. The Santorini orifice was then re-approximated to the duodenal wall for protection against acute pancreatitis caused by scarring and stenosis of the duct orifice as a possible late complication. The patient's postoperative course was uneventful and she has been asymptomatic without evidence of tumor recurrence or stenosis of the Santorini orifice on endoscopic examination for the last 4 years.     

ENDOSCOPIC APPROACH TO THE MINOR DUODENAL PAPILLA: SPECIAL EMPHASIS ON ENDOSCOPIC MANAGEMENT ON PANCREAS DIVISUM

Diagnostic and therapeutic approach to the minor duodenal papilla including standardized technique was reviewed. In cases in which a pancreatogram is not achieved or those in which only a small portion of the ductal anatomy is visualized via the major duodenal papilla, cannulation of the minor papilla provides a second route of access to the ductal system. Successful minor papilla cannulation requires meticulous attention to technique. As the orifice of the minor papilla is usually of pinpoint size, needle‐tipped catheters are useful. As minor papilla cannulation in pancreas divisum carries the risk of severe pancreatitis, the procedure should be performed with more caution. In some patients with pancreas divisum, an increased resistance to flow across the small orifice results in dorsal pancreatic duct hypertension and clinical symptoms including acute recurrent pancreatitis, chronic pancreatitis, and pancreatic‐type pain. Pancreas divisum patients with acute recurrent pancreatitis are the best candidates for endoscopic management for dorsal‐duct decompression including endoscopic minor papilla sphincterotomy and stenting.     

Endoscopic papillectomy of minor papillar adenoma associated with pancreas divisum

Tumors of the minor papilla of the duodenum are quite rare. We successfully and safely treated an 18-mm adenoma of the minor papilla associated with pancreas divisum using endoscopic papillectomy. A 64-year-old man was admitted to our hospital for treatment of an asymptomatic mass in the minor papilla detected by upper gastrointestinal endoscopy. Endscopic analysis showed an 18-mm, whitish, sessile mass, located in the duodenum proximal to a normal-appearing major papilla. Endoscopic retrograde pancreatography did not reveal the pancreatic duct. Magnetic resonance cholangiopancreatography showed a lack of the ventral pancreatic duct. We suspected this case was associated with pancreatic divisum; therefore, we performed endoscopic papillectomy of the minor papilla tumor. Subsequently, endoscopic pancreatic stent placement in the minor papilla was done to prevent drainage disturbance. The patient has been asymptomatic without recurrence of tumor or stenosis of the Santorini orifice upon endoscopic examination for the past 2 years.     

An improved technique for accessory papilla cannulation in pancreas divisum

Cannulation of the accessory papilla with dorsal pancreas ductography was accomplished in 13 of 15 consecutive cases of pancreas divisum. When routine cannulation of the main pancreatic papilla fails to produce a pancreatogram or yields only a ventral pancreas, the use of intravenous secretin and a 23-gauge needle catheter is recommended to facilitate accessory duct cannulation.     

Use of methylene blue to identify the minor papilla or its orifice in patients with pancreas divisum

BACKGROUND: In some patients with pancreas divisum, minor papilla cannulation is difficult because of uncertain identification of the papilla or its orifice, even after pancreatic secretory stimulation with secretin or cholecystokinin agonist. METHODS: Two techniques with methylene blue were used to identify the minor papilla and its orifice more clearly in a series of patients: spraying methylene blue over duodenal mucosa in the region suspected to contain the minor papilla with/without secretin or cholecystokinin agonist administration, and injection of contrast medium containing methylene blue into the ventral pancreatic duct by means of the major papilla in cases of incomplete pancreas divisum. Results were reviewed retrospectively. RESULTS: From January 2001 to May 2002, minor papilla cannulation with conventional methods initially failed in 38 of 305 patients with pancreas divisum because of an inconspicuous minor papilla orifice. Methylene blue was used to identity the minor papilla orifice in 14 of 38 patients (spraying, 13; injection, 1). Minor papilla cannulation was successful in 12 of 14 (86%) patients (spraying 11, injection 1). Mild pancreatitis developed in 1 patient. CONCLUSIONS: Methylene blue spraying or injection appears to be a helpful technique for identification of the inconspicuous minor papilla orifice in patients with pancreas divisum.     

ERCP and CT diagnosis of pancreas divisum and its relation to etiology of chronic pancreatitis

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Santorinicele in a patient with unresectable pancreatic cancer

A santorinicele is defined as a focal cystic dilatation of the terminal portion of the dorsal pancreatic duct at the minor papilla; most cases have been reported in patients with pancreas divisum. It has been suggested that a santorinicele results from a combination of a minor papilla obstruction which prevents the flow of pancreatic juice and a weakness of the duodenal wall where the dorsal pancreatic duct terminates. However, these conditions can occur in patients with invasive ductal cancer in the pancreatic head. We encountered a rare case of a santorinicele with unresectable adenocarcinoma of the pancreatic head in an 81-year-old woman.     

Ultrasound secretin test in patients with pancreas divisum--an aid in the diagnosis of papillary or dorsal duct stenosis?

BACKGROUND: Pancreas divisum is a common anatomical variant of pancreatic ductal anatomy. Obstruction of the accessory papilla could cause pain and pancreatitis. It has been suggested that accessory papillary sphincter obstruction can be assessed by sonographic measurement of pancreatic duct diameter after secretin stimulation. METHODS: We now compared our results of sonographic pancreatic duct diameter measurements before and during 10 min after intravenous injection of 1 CU secretin per kg body weight in 32 patients with confirmed pancreas divisum and 20 healthy volunteers. RESULTS: The healthy controls showed a short-lasting duct caliber enlargement by about 93% of the basal diameter within 5 min after secretin injection. 25 pancreas divisum patients without pancreatic disease had a secretin-induced duct dilatation by about only 58%. In four patients with pancreas divisum and chronic pancreatitis no or just a slight duct dilatation was observed after stimulation. Two patients with dorsal duct stenosis as well as one patient with accessory papilla stenosis, however, showed a marked and prolonged secretin-induced duct enlargement by about 155% of the basal duct diameter. CONCLUSION: In this investigation pancreatic duct response to secretin stimulation in pancreas divisum patients without pancreatic disease was less marked than in normal individuals. Thus, a particularly distinct and long-lasting duct dilatation could support the suspicion of accessory papilla or pancreatic duct stenosis.     

Elevated pressure in the dorsal part of pancreas divisum: the cause of chronic pancreatitis?

In 6 patients with upper abdominal pain of unknown origin presenting with pancreas divisum, the pressure in the pancreatic duct was measured via the minor papilla into which in these patients the main part of the pancreatic duct system drains. For comparison intraductal manometry via the major papilla (papilla of Vater) was performed in 8 patients with normal pancreatic duct system. The pressure in the pancreatic duct of the control group was 10.5 +/- 0.9 mm Hg, whereas in the patients with pancreas divisum it was 23.7 +/- 1.3 mm Hg. The results demonstrate that in patients with pancreas divisum, intraductal pressure may be largely increased even in the fasting state.     

Minor papilla cannulation and dorsal ductography in pancreas divisum

Until recently, pancreas divisum represented a major technical barrier to a complete evaluation of pancreatic ductal anatomy. Technical refinements have now made it possible to achieve minor papilla cannulation and dorsal ductography in more than 90% of attempts. In 120 consecutive dorsal ductograms, structural pathology was demonstrated in 36 subjects (30%): chronic pancreatitis in 23, pancreatic stones in 10, pseudocyst(s) in 4, ductal "cut-off" in 7, pancreatic cancer in 3, and partial agenesis in 1 (some patients had more than one finding). For patients in whom alcohol abuse was excluded, ductal pathology was present in 25%. Abnormal ventral ductograms were present in only 8% of cases, demonstrating that dorsal ductography has an appreciable additional diagnostic yield. When the clinical situation indicates the need for pancreatography, minor papilla cannulation should be performed if major papilla cannulation fails or reveals only the ventral pancreatogram of pancreas divisum.     

Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis.

Pancreas divisum occurs when the embryological ventral and dorsal parts of the pancreas fail to fuse, so that pancreatic drainage is mainly through the accessory papilla. In 169 patients with primary biliary tract disease who underwent pancreatography incidental to endoscopic cholangiography, the incidence of pancreas divisum was 3.6%. Among 78 patients with unexplained recurrent pancreatitis, the incidence was 25.6%. The hypothesis that the duct anomaly can cause obstructive pain and pancreatitis is presented, and supported by a detailed analysis of 47 patients with the condition.     

A Histopathological Study on Ventral and Dorsal Pancreas Tissues in Patients with Pancreas Divisum

Abstract: Five cases of pancreas divisum, clinically diagnosed by endoscopic retrograde choledochopancreatography (ERCP), were studied histopathologically. They consisted of four biopsied cases and one patient who underwent pancreatoduodenectomy under the diagnosis of a suspected pancreatic carcinoma. Fibrosis was found in three out of the five cases, two cases with fibrosis in the dorsal pancreas and one in the ventral pancreas. In the two dorsal fibrosis pancreas cases, fibrosis was distributed in the intra- and interlobular areas and was accompanied by atrophy or disappearance of the acinar cells. These findings were compatible with chronic obstructive pancreatitis, distal to a stricture of the pancreatic duct. In the one ventral fibrosis pancreas case, i. e., the pancreatoduodenectomized case, fibrosis was distributed mainly in the interlobular area associated with hemosiderin deposition. Hemosiderin deposition was also observed in inflammatory foci at the posterior aspect of the ventral pancreas. Hence, such interlobular fibrosis developed in relation to the inflammatory foci, and was not caused by pancreas divisum. Therefore, in pancreas divisum, abnormal findings were observed in the dorsal pancreas tissue, and they were compatible with chronic obstructive pancreatitis. (Dig Endosc 1994; 6 : 74–79)     

Endoscopic minor papilla interventions in patients without pancreas divisum

BACKGROUND: Endoscopic treatment through the minor papilla is well known in patients with pancreas divisum. However, there are few data concerning endoscopic minor papilla interventions in patients without pancreas divisum when access to the main pancreatic duct via the major papilla is technically difficult. METHODS: Records for 213 patients without pancreas divisum who, from April 2001 to June 2003, underwent ERCP for various pancreatic diseases were retrospectively reviewed. Patients were included if they had endoscopic interventions via the minor papilla because access through the major papilla was not possible. OBSERVATIONS: Minor papilla papillotomy or fistulotomy with endoscopic interventions was successful in 10 (91%) of 11 patients. Of these 10 patients, 9 had chronic pancreatitis and one had pancreatic ductal leak from previous pancreatic surgery. The reasons for the inability to access the main pancreatic duct to the tail of the gland via the major papilla included a distorted course of the main pancreatic duct (n=5), impacted stone (n=5), and stricture (n=8). In 8 patients, there were two causes. No complication related to the minor papilla interventions was observed in any patient. CONCLUSIONS: Endoscopic minor papilla interventions are technically feasible in patients with pancreatic diseases but not pancreas divisum when access to the main pancreatic duct via the major papilla is not possible.