Clinical significance of pancreas divisum.

Pancreas divisum (PD), a congenital anatomic variant consisting of a separate pancreatic ductal system, is diagnosed in nearly 10% of patients for whom a successful pancreatogram was obtained. The relationship between PD and pancreatic disease is discussed since 1976. Some authors, reporting a higher incidence of pancreatitis in patients having two separate pancreatic ducts, proposed the concept of relative outflow obstruction of pancreatic juice through the accessory papilla. Based on the literature data and on our own experience, this review asserts that there is no definite evidence for PD being associated with a significantly risk of idiopathic pancreatitis. Discrepancies between epidemiological series could be explained by selection biases leading to an apparent association of PD and pancreatitis. An objective assessment of accessory papilla stenosis in patients with PD is not clearly available to give consistent results. Although controversies persist with regard to the actual abnormality of PD and the presence of stenosis into the accessory papilla, several endoscopic and/or surgical procedures have been proposed in an attempt to correct what is thought to be a stenosis of the orifice of the dorsal duct. More than 300 patients have been treated until now with variable and unpredictable results, these treatments are not devoid of complications. We conclude that PD should be considered as a frequent coincidental anatomic variant having no clinical significance in the great majority of patients and not requiring systematic further therapy.     

Management of pancreas divisum

Pancreas divisum is the most common congenital anomaly of the pancreas. Abnormal hedgehog protein signaling appears related to the formation of several pancreatic malformations, including annular pancreas, pancreatic-biliary malunion, pancreatic rests, and pancreas divisum. Pancreas divisum by itself should not necessarily require intervention. A careful evaluation should be performed to exclude other causes of symptoms. If the patient is asymptomatic, no further evaluation is necessary. However, a significant percentage of patients with pancreas divisum and acute recurrent pancreatitis benefit from intervention. Surgical sphincteroplasty and endoscopic interventions appear similar in outcome. Thus, endoscopic intervention with prophylactic temporary stenting is advised as initial therapy. Surgery should be reserved for patients with chronic pancreatitis.     

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.     

Endoscopic approach to pancreas divisum

Pancreas divisum has been claimed to be a harmless congenital variant or to occasionally cause acute relapsing pancreatitis (ARP), chronic pancreatitis (CP), or a chronic abdominal pain (CAP) syndrome. Both surgical and endoscopic approaches to accessory papilla decompression have been promulgated and widely disparate results reported in the literature. We retrospectively reviewed a five-year experience with dorsal pancreatic duct decompression at our institution utilizing a variety of endotherapeutic techniques. Data collected included procedural complications; patient interpretation of pre- and posttherapy pain, frequency, and intensity graded on an analog pain scale; frequency of hospitalization; and patient perception of global improvement to endotherapy. At a mean follow-up of 20 months, there was a statistically significant decrease in pancreatitis incidence in 15 patients with ARP (P=0.016) and 19 patients with CP (P=0.025). The frequency and intensity of chronic pain was also significantly improved (P<0.001) in the latter group. In contrast, only one of five patients with CAP and normal dorsal pancreatography and secretin tests experienced global improvement, and there was no improvement utilizing an analog pain scale (P=0.262) in the group as a whole. There was a 20% incidence of mild procedure or subsequent stent-related pancreatitis and an 11.5% accessory papilla restenosis rate. It is concluded that a subset of carefully selected patients with pancreas divisum may respond to endotherapy but that long-term follow-up will be required to define its ultimate place in the management of symptomatic patients with this anomaly.     

Serous cystadenoma of the pancreas associated with pancreas divisum

Received: December 27, 2000 / Accepted: May 25, 2001     

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.     

Multiple neuroendocrine tumors of the pancreas associated with pancreas divisum

Pancreas divisum is the most common congenital anomaly of the pancreas, characterized by missing fusion of the ventral and dorsal pancreatic duct. It may cause pancreatitis, but is rarely associated with malignancy.We report herein for the first time the rare association, in a symptomless patient, of multiple neuroendocrine tumors of the pancreas with pancreas divisum and a failure of the exocrine system. Diagnosis was made incidentally by routine abdominal ultrasound. Laboratory examinations and a fine-needle aspiration revealed the neuroendocrine nature of the tumor. Spleen-preserving left pancreas resection was performed, with evidence of multiple neuroendocrine tumors of the pancreas with the typical histological characteristics. Eighteen months later the patient is still free of tumor burden.     

Porcelain gallbladder complicated with pancreas divisum

We report a rare case of porcelain gallbladder associated with pancreas divisum (PD). A 60-year-old woman suffered from discomfort in the back of the right side. An abdominal radiograph revealed a calcified spherical mass in the right upper quadrant. Ultrasonography revealed a scattered echo with a posterior acoustic shadow in the gallbladder wall. A plain computed tomography (CT) scan showed flecks of intramural calcification in the wall of the gallbladder. Endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) showed separate openings for the Santorini and Wirsung ducts. The patient underwent cholecystectomy after porcelain gallbladder and pancreas divisum had been diagnosed. The porcelain gallbladder resulted from a stone impacted in the neck of the gallbladder. Patients with PD should be followed carefully, because gallstones often accompany PD, and porcelain gallbladder may result, as in this patient.     

Double anastomoses to jejunum of dissected pancreas portions in pancreas divisum

The effectiveness of operation for pancreas divisum in patients who exhibit unexplained upper abdominal pain or recurrent pancreatitis is often uncertain. Here we report a successful operation in a patient whose two dissected pancreas portions were anastomosed with the looped jejunum. The patient was a 31-year-old woman who had suffered from continuous upper abdominal pain and relapsing pancreatitis for 10 years. She had a history of excessive alcohol intake from the age of 19–25 years. When she was 24 years old, endoscopic retrograde pancreatography had been performed, revealing pancreas divisum. Thereafter, various treatments had been performed, endoscopic accessory papillotomy; the administration of an anti-secretagogue, a cholecystokinin receptor antagonist; and cannulation of a stent tube into the dorsal pancreatic duct. Each of these treatments led to only short-lived relief of the symptoms. When she was 31 years old, the following operation was performed: The pancreas was cut off at the portal vein and the jejunum was pulled up via the retrocolic route; the two dissected pancreas portions were double-anastomosed with the jejunum by an end-to-side procedure. The postoperative course has been smooth. Fifteen months after the operation, the patient has gained 4 kg in weight and is symptom-free.