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.     

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.     

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.     

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.     

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.     

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.     

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.     

A two-year old boy with recurrent bouts of acute abdominal pain

In a small number of patients with pancreas divisum (with stenotic minor papilla) a relative obstruction to pancreatic exocrine secretory flow results in pancreatitis. We report a 2-year-old boy presenting with recurrent bouts of abdominal pain. The diagnosis of acute pancreatitis was made based on blood biochemistry results. Ultrasound, computed tomography and magnetic resonance imaging showed several abdominal pseudocysts, peritoneal exsudate and confirmed pancreatitis but initially failed to reveal the aetiology. Ascites and cysts contained pancreatic enzymes. After weeks of combined conservative and surgical treatment, a magnetic resonance cholangiopancreaticography with secretin, showed a pancreas divisum with a cyst between the ducts of Santorini and Wirsung. Based on these findings, two endoscopic papillotomies (minor and major papilla) were performed. Three years follow-up was uneventful. In a child with recurrent pancreatitis or pancreatitis with chronic recurrent abdominal pain it is crucial to search aggressively for congenital abnormalities, including pancreas divisum. Secretin-enhanced magnetic resonance cholangiopancreaticography or diffusion-weighted magnetic resonance imaging is a valuable diagnostic tool for visualizing pancreatic duct anatomy.     

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.     

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.     

Pancreas head carcinoma with total fat replacement of the dorsal exocrine pancreas

We report a case of pancreas head carcinoma associated with fat replacement of the body and tail. A 68-year-old man presented with obstructive jaundice and was admitted to our hospital. Ultrasonography and computed tomography showed pancreas head tumor with a neighboring cystic lesion and fatty replacement of parenchyma of the pancreas body and tail. By endoscopic retrograde pancreatography, abruption of the main pancreatic duct and the presence of an accessory duct were detected. After percutaneous transhepatic biliary drainage, pancreatoduodenectomy was successfully performed. At laparotomy, the pancreas head was easily dissected from the replaced fatty tissue of the body and tail without continuity of the ductal system or parenchyma. Microscopic examination revealed the existence of an infiltrating ductal adenocarcinoma and a neighboring. cyst in the pancreas head. The dorsal exocrine pancreas was completely replaced by the fat tissues, in which viable Langerhans islets were scattered. The patients postoperative course was uneventful, and exogenous insulin administration was unnecessary for the maintenance of normal blood sugar level. Acquired fat replacement of the body and tail of the pancreas is an uncommon disorder, mimicking congenital agenesis of the dorsal pancreas. Though the mechanism is controversial, obstruction of the main pancreatic duct by a cystic lesion or carcinoma in the pancreas head is a possible cause of fatty degeneration of the pancreatic parenchyma.     

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.     

A case of intraductal papillary mucinous neoplasm of the pancreas arising from pancreas divisum without ventral pancreatic duct of Wirsung]

Most reported cases of intraductal papillary mucinous neoplasms (IPMNs) originate from Wirsung's duct or their branches. IPMNs arising from Santorini's duct and its branches have rarely been reported. Eight cases of IPMN arising from Santorini's duct have been published worldwide. However, these cases are associated with incomplete type of pancreas divisum. Recently, one report of IPMN with complete absence of Wirsung's duct has been reported. This patient was a 57-year-old woman who was admitted to the hospital due to progressive jaundice. On endoscopic retrograde cholangiopancreatography, there was a severely bulging ampulla of Vater and patulous minor papilla draining mucinous material and a cystic lesion communicating with the dilated Santorini's duct without any communication with Wirsung's duct. A pancreaticoduodenectomy was performed and the pathologic examination of resected specimen showed no evidence of Wirsung's duct, but an IPMN arising from Santorini's duct with peripancreatic lymph node metastasis. Herein, we report a case of invasive IPMN arising from pancreatic head without ventral pancreatic duct with a review of the relevant literatures.     

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.     

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 ultrasound and pancreas divisum

Pancreas divisum is the most common congenital anatomic variation of the pancreatic ductal anatomy and in most of the individuals it is asymptomatic. However, in minority of individuals it is presumed to cause recurrent acute pancreatitis and chronic pancreatitis. Endoscopic retrograde cholangiopancreatography is the gold standard for its diagnosis, but is invasive and associated with significant adverse effects. Endoscopic ultrasound (EUS) allows the detailed evaluation of the pancreaticobiliary ductal system without injecting contrast in these ducts. Moreover, it provides detailed images of the parenchyma also. Therefore EUS, both radial and linear, has potential for being a minimally invasive diagnostic modality for pancreas divisum. A number of EUS criteria have been suggested for the diagnosis of pancreas divisum. These criteria have varying sensitivity and specificity and hence there is a need for objective and uniform criteria that have the best diagnostic accuracy. Secretin EUS has a potential for diagnosing minor papilla stenosis and thus help in planning appropriate therapy. EUS guided pancreatic duct interventions can help in draining dorsal duct in symptomatic patients with failed minor papilla cannulation. But these techniques are technically demanding and associated with potential severe complications.     

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.     

Pancreatic carcinoma accompanied by pseudocyst: Report of two cases

Two cases of pancreatic cancer accompanied by pseudocyst are reported. Case 1 was a 60-year-old man who was admitted to our hospital complaining of left lower abdominal discomfort. A cystic lesion, about 3 cm in diameter, was found in the pancreatic tail by ultrasonography (US) and computed tomography (CT). No signs of chronic pancreatitis were found. At operation, an elastic, hard, white tumor, about 1 cm in diameter, was felt adjacent to the cystic lesion on the duodenal side. Histologically, this tumor was a duct cell carcinoma with an adjacent pseudocyst upstream of the pancreas. Case 2 was a 57-year-old man who complained of back pain and loss of body weight. US and CT examination revealed a cystic lesion, 11×7 cm in size, in the tail of the pancreas. Histological examination of the resected speciment revealed both a duct cell carcinoma, 3 cm in size, in the body of the pancreas and a pseudocyst, 9 cm in size. Pseudocysts accompanying carcinoma are thought to develop from obstruction of the pancreatic duct by the carcinoma, followed by intraductal high pressure and disruption of ductules upstream of the pancreas. Thus, we should pay careful attention to pseudocyst of the pancreas, especially when signs of diffuse chronic inflammation cannot be found, to help identify duct cell carcinoma in the early stage. Further detailed examinations of the cyst fluid or pancreatic juice, such as cytology, tumor marker determinations, or establishment of K-ras codon 12 mutation, are needed.