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.     

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.     

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.     

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.     

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.     

Annular pancreas associated with carcinoma in the dorsal part of pancreas divisum

Summary A carcinoma in the dorsal part of the pancreas divisum with an annular pancreas in the anterior part is reported. A 79-yr-old female was admitted in our hospital complaining of epigastralgia. Computed tomography (CT) and ultrasound (US) showed an irregular mass in the pancreatic body. A pancreatogram obtained through the major duodenal papilla demonstrated only the ventral pancreatic duct that encircled the duodenum. Contrast medium injected from the minor duodenal papilla showed Santorini’s duct obstruction at the neck portion of the pancreas without communication with the ventral pancreatic duct. The patient died with liver metastases. Autopsy confirmed annular pancreas and a 6-cm tumor in the pancreatic body extending to the pancreatic head and pancreas divisum. Pancreatic carcinoma; histologically a moderately differentiated adenocarcinoma; originated from the dorsal part of pancreas divisum. To our knowledge this is the first report of pancreatic carcinoma associated with annular pancreas coexistent with pancreas divisum.     

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.     

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.     

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.     

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.     

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     

Clinical experience in 82 patients with pancreas divisum: preliminary results of manometry and endoscopic therapy

Although it is clear that the majority of patients with pancreas divisum have no clinical disease, there is a subset of patients who have either unexplained abdominal pain or recurrent pancreatitis. Endoscopic therapy of the minor papilla may alter the clinical course of those patients with pancreas divisum and recurrent pancreatitis. Manometric study of the minor papilla is feasible and reveals a sphincter mechanism similar to the major papilla. Clinical response to endoscopic therapy may aid in selecting patients who might benefit from surgical sphincteroplasty. Refinement of manometric study of the minor papilla offers a potential method of detecting functional obstruction of dorsal duct drainage.     

Endoscopic resection of adenoma of the minor papilla

Tumors of the minor papilla of the duodenum are rare. We successfully and safely treated a 16 mm sessile adenoma of the minor papilla using endoscopic resection. Endoscopic retrograde cholangiopancreatography (ERCP) yielded a normal cholangiopancreatogram with blind termination of the duct of Santorini in the minor papilla. We recommend endoscopic resection and histological examination of the entire lesion for adenomas of the minor papilla in patients without pancreas divisum.     

Intraductal papillary adenocarcinoma with mucin hypersecretion and coexistent invasive ductal carcinoma of the pancreas with apparent topographic separation

We report a 66-year-old man who had a cystic intraductal papillary adenocarcinoma containing a papillary adenoma, in the head of the pancreas and a coexistent invasive, well differentiated solid tubular adenocarcinoma in the tail of the pancreas. He was hospitalized with acute epigastralgia. Computed tomography demonstrated a multilocular cystic mass in the head of the pancreas and a solid tumor in the tail. Endoscopic retrograde pancreatography showed mucin secretion from an enlarged papilla of Vater, marked dilatation of the main pancreatic duct in the head and body, cystic dilatation of the uncinate branch, and irregular narrowing of the main pancreatic duct in the tail. Total pancreatectomy was performed. Between the cystic tumor and the solid tumor there was a distance of 4.8 cm of normal pancreatic parenchyma and duct, recognized both grossly and microscopically. The patient died 35 months after the operation. At autopsy, peritonitis carcinomatosa was found in the abdominal cavity. Microscopically, disseminated nodules were also well differentiated tubular adenocarcinoma. The apparent anatomic separation of these two tumors within the pancreas is extremely unusual.     

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.     

Santorinicele as a Cause of Chronic Pancreatic Pain

The role pancreas divisum plays in recurrent pancreatitis and chronic pancreatic pain remains controversial. When pancreatic disease does occur secondary to pancreas divisum, the pathogenesis is thought to the stenosis of the accessory duct with a resulting increase inductal pressure.
A case is reported in which stenosis of the accessory papilla orifice is thought to be responsible for cystic dilatation of the terminal portion of the duct of Santorini in a patient with pancreas divisum. This resulted in chronic pancreatic pain that resolved after sphincterotomy of the accessory papilla.     

Hemosuccus pancreaticus due to intraductal papillary-mucinous carcinoma of the pancreas

A 72-year-old female was referred to our hospital for evaluation of a hyperechoic mass in the pancreatic head with ultrasound sonography. She had no symptom expect slight anemia (Hb 11.3 g/dl). On endoscopy, blood was expelled from the orifice of the major duodenal papilla, but excretion of mucus was not detected. Endoscopic retrograde pancreatography revealed an irregular defect in the main pancreatic duct at the head of the pancreas. Computed tomography revealed a 2-cm mass with a low density lesion in the pancreas head. On suspicion of malignant tumor of the pancreas, pylorus-preserving pancreaticoduodenectomy was performed. Histological diagnosis was intraductal papillary-mucinous carcinoma without mucin hypersecretion. It grew within the inferior branch of the main pancreatic duct, and the top of the tumor stood out into the main pancreatic duct. As the causes of hemosuccus pancreaticus, pancreatic benign diseases, for example, chronic pancreatitis, pseudocyst, arterial aneurysm and pseudoaneurysm, are known, but pancreatic tumors are rare. In particular, this may be the first report of hemosuccus pancreaticus induced by intraductal papillary-mucinous carcinoma of the pancreas without mucin hypersecretion.     

Endoscopic pancreatic duct stent placement for inflammatory pancreatic diseases

The role of endoscopic therapy in the management of pancreatic diseases is continuously evolving; at present most pathological conditions of the pancreas are successfully treated by endoscopic retrograde cholangio- pancreatography （ERCP） or endoscopic ultrasound （EUS）, or both. Endoscopic placement of stents has played and still plays a major role in the treatment of chronic pancreatitis, pseudocysts, pancreas divisum, main pancreatic duct injuries, pancreatic fistulae, complications of acute pancreatitis, recurrent idiopathic pancreatitis, and in the prevention of post-ERCP pancreatitis. These stents are currently routinely placed to reduce intraductal hypertension, bypass obstructing stones, restore lumen patency in cases with dominant, symptomatic strictures, seal main pancreatic duct disruption, drain pseudocysts or fluid collections, treat symptomatic major or minor papilla sphincter stenosis, and prevent procedure-induced acute pancreatitis. The present review aims at updating and discussing techniques, indications, and results of endoscopic pancreatic duct stent placement in acute and chronic inflammatory diseases of the pancreas.     

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.