Intraductal pancreatic carcinoma associated with pancreas divisum.

OBJECTIVE: we report the case of a 36 years old man with a pancreatic cancer associated to a pancreas divisum presenting as acute and relapsing recurrent pancreatitis. The coexistence of intraductal carcinoma and pancreas divisum is uncommon as let us do analyze its clinical, radiological and therapeutical features. CONCLUSIONS: 1. Pancreatic duct obstruction and pancreas divisum as congenital anomaly may be consider potential etiologies for pancreatic cancer. 2. Widespread use of magnetic resonance xholangiopancreatography and endoscopic retrograde xholangiopancreatography examinations together with brush cytology smears will allow more accurate approach to tumor diagnosis and management. 3. The use of pancreatoscopy in patients suffering from chronic pancreatitis may reveal helpful to discover intraductal neoplasms otherwise misdiagnosed.     

Prevalence and clinical evolution of isolated ventral pancreatitis in alcoholic chronic pancreatitis

BACKGROUND: The role of pancreas divisum in chronic pancreatitis is controversial. Ductal changes limited to the pancreatic ventral duct (isolated ventral pancreatitis) have only been described as isolated case reports. METHODS: In a cohort of 542 patients with chronic pancreatitis we determined the frequency of ventral chronic pancreatitis among patients with pancreas divisum and analyzed the clinical presentation of 10 patients with isolated ventral alcohol-induced pancreatitis compared with 10 patients with isolated dorsal alcohol-induced pancreatitis and 30 patients with chronic pancreatitis and without pancreas divisum. Magnetic resonance pancreatography under secretin stimulation was used to evaluate the status of the dorsal pancreatic duct in some patients during follow-up. RESULTS: We identified 62 (11.4%) patients with pancreas divisum and 16 (2.9%) patients with incomplete pancreas divisum. The ventral duct was affected by chronic pancreatitis in 74% of these patients. Isolated ventral or dorsal duct alterations were identified in 14% and 26%, respectively, of patients with pancreas divisum. Patients with isolated ventral pancreatitis had pain symptoms similar to those of the two other groups but had no endocrine or exocrine insufficiency on initial presentation. After a mean follow-up of 44 months, 83% of patients studied with isolated ventral pancreatitis developed alterations of the dorsal ductal part of the gland. CONCLUSION: We conclude that isolated ventral alcohol-induced pancreatitis is one of the first manifestations of generalized pancreatic disease where the anatomic factor of pancreas divisum plays only a marginal role.     

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.     

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.     

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.     

Pancreas Divisum: Incidence, Detection, and Clinical Significance

Pancreas divisum is a congenital anomaly in which the ventral and dorsal pancreas drain separately into the duodenum. It is the most common congenital variant of pancreatic ductal fusion and drainage anomalies. With widespread use of endoscopic retrograde cholangiopancreatography, pancreas divisum is being detected with increasing frequency. Ten cases of pancreas divisum detected among 500 endoscopic retrograde cholangiopancreatography examinations performed between 1979 and 1985 at our institution were critically analyzed. Patients with symptomatic pancreas divisum (group 1) were typically young (mean age 29 yr), usually female, and had no history of significant alcohol abuse. Those with incidental detection of pancreas divisum (group 2) were older (mean age 62 yr), usually male with hepatobiliary disease, and had a history of significant alcohol ingestion. The radiological feature of pancreas divisum is characterized by a short (1-6 cm) and thin (2 mm diameter) pancreatic duct (duct of Wirsung) that branches off into regular arborization and drains only the posterior part of the head of the pancreas. This appearance is quite typical; however, this may be simulated by other conditions such as previous pancreatic trauma, partial pancreatectomy, or pancreatitis with irreversible damage to the duct, pseudocyst, and pancreatic carcinoma. The differentiation between true and false pancreas divisum is important because of its clinical implications.     

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.     

Isolated ventral pancreatitis in a patient with pancreas divisum

A previously healthy 22-yr-old woman was found to have chronic pancreatitis restricted to the ventral pancreas in association with pancreas divisum. When conservative treatment failed, sphincteroplasty of both papillae was performed with good results. This well-documented case represents a clear exception to the commonly held concept that obstruction of the dorsal duct is the essential pathogenic factor in the development of pancreatitis associated with pancreas divisum. The importance of visualizing both the dorsal and ventral pancreatic ducts to direct therapy for patients with idiopathic pancreatitis is discussed. Theories of the pathogenesis of concomitant pancreatitis and pancreas divisum are reviewed.     

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.     

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.     

Pancreas divisum: congenital anatomic variant or anomaly? Contribution of endoscopic retrograde dorsal pancreatography

The relationship between pancreas divisum and pancreatic disease has been studied in a series of 304 patients. This congenital anatomic variant, consisting of a separate pancreatic ductal system, was diagnosed by endoscopic pancreatography and dorsal duct opacification was achieved in 97 of these patients. This anatomic variation was observed with the same frequency in cases of pancreatitis (acute and chronic) (6.9%) and in the series of patients investigated by endoscopic pancreatography taken as a whole (5.7%). Moreover, incidences of pancreatic disease in patients with and without pancreas divisum were not statistically different when compared. These results show that pancreas divisum should not be regarded as an etiologic factor in pancreatitis but should be considered as a coincidental anatomic variant encountered in nearly 10% of the population. The results obtained herein do not support the hypothesis that stenosis of the accessory papilla occurs frequently in cases of pancreas divisum. We conclude that no further therapy should be systematically proposed for patients with pancreas divisum and pancreatitis.     

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.     

Pancreas divisum does not modify the natural course of chronic pancreatitis

Background Pancreas divisum is the most common congenital variant of the pancreas; however, its clinical significance remains controversial. The purpose of our study was to determine the role of pancreas divisum in the development of chronic pancreatitis. Methods We compared the clinical presentation, morphological findings, and course of disease of 30 patients with chronic pancreatitis associated with pancreas divisum (there was coexisting chronic alcohol abuse in 18 cases) to those of 57 patients with chronic pancreatitis and no evidence of pancreas divisum (15 with nonalcoholic pancreatitis and 42 with alcoholic pancreatitis). Results Sex distribution, age at onset of disease, clinical presentation, course of disease, and frequency of complications were not affected by the presence of pancreas divisum. Although the etiology of pancreatitis in patients with pancreas divisum may be attributed to impaired drainage of the majority of the gland through the minor papilla, we observed a relatively low frequency of isolated dorsal duct involvement in our patients irrespective of alcohol use (25% and 28% in patients with and without a history of alcohol abuse, respectively). However, involvement of the ventral duct was commonly observed (75% and 72%, respectively). Conclusions The presence of pancreas divisum in our study did not modify the natural course of chronic nonalcoholic or alcoholic pancreatitis. Pancreas divisum is not likely to play a dominant role in the etiopathogenesis of chronic pancreatitis.     

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.     

胰管支架的临床应用及研究进展

胰管支架是扩张胰管、通畅引流的有效手段。内镜下置入胰管支架多用于缓解慢性胰腺炎患者的疼痛、治疗胰管断裂及其引发的胰腺液体积聚、预防ERCP术后胰腺炎,也可以用于治疗胰腺分裂症、胰腺恶性肿瘤以及定位阴性结石。随着内镜技术的发展,胰管支架在胰管疾病的治疗中占据重要地位。本文主要讨论内镜下胰管支架的临床应用及研究进展。     

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.     

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.     

Dynamic CT scanning of pancreatic duct after secretin provocation in pancreas divisum

Variations in pancreatic duct diameter at CT scanning and serum pancreatic amylase response following secretin administration were studied in 29 patients with pancreas divisum and unexplained upper abdominal pain. Eleven healthy individuals were used as controls. At endoscopie retrograde pancreatography (ERP) six patients had signs of marked and six moderate pancreatitis, whereas there were no pancreatitis changes in 17 of the patients. At CT scanning patients with marked pancreatitis (ERP) had significantly increased pancreatic duct diameter as compared to patients without signs of pancreatitis. The duct was visualized in 52% of all patients before and 71% after secretin stimulation the corresponding figures for healthy controls, being 18% both before and after secretin. In patients without signs of pancreatitis, it was demonstrated in 5/17 (29%) before and 11/17 (65%) after secretin, whereas it was seen in 10/12 (83%) pancreatitis patients both before and after the hormonal provocation. In five of the nonpancreatitis patients in whom the duct was measurable before and at all study intervals (10, 20, and 50 min) after secretin, there was a significant duct dilation response both at 10 min and when comparing the maximal duct diameter after secretin to the initial values. In contrast secretin did not affect the duct caliber in pancreatitis patients. Serum pancreatic amylase increased significantly after secretin administration to healthy controls and nonpancreatitis patients but was uninfluenced in the marked and moderate pancreatitis groups, respectively. However, when all pancreatitis patients were grouped together, the amylase levels were significantly elevated by secretin. In conclusion, secretin provocation caused duct dilation at CT scanning in pancreas divisum patients without signs of pancreatitis at ERP. In this group secretin also increased the number of patients with visualized pancreatic duct. However, in pancreas divisum patients with pancreatitis and in healthy controls no such findings were observed. Secretin stimulation increased samylase in a nonspecific way. If secretin at CT scanning causes dilation of the pancreatic duct compared to its initial measurable width or visualization of an initially not demonstrable duct in patients with unexplained upper abdominal pain, the presence of pancreas divisum without morphologically manifest pancreatitis should be considered.     

胰腺分裂内镜治疗价值

目的探讨内镜下治疗胰腺分裂的疗效和安全性。方法收集2006年6月至2013年6月在南京大学医学院附属鼓楼医院消化科就诊的8例胰腺分裂患者的临床资料,对术中及术后情况进行回顾性分析。结果8例胰腺分裂患者共行经内镜逆行胰胆管造影术28例次,均置入胰管支架,其中7例同时行副乳头括约肌切开术,6例行内镜下扩张术,1例行胰管取石术。术后出现1例次轻度胰腺炎。随访2～47个月,1例死于胰腺癌,3例已拔出支架,3例定期复查,1例失访。患者术后慢性胰腺炎急性发作频率及腹痛程度均较术前明显降低。结论内镜下治疗胰腺分裂安全有效,可减少胰腺炎发作频率,减轻术后腹痛程度,术后并发症发生率低。     

Pancreas divisum

Summary A 36-year-old man, followed for 14 years with recurrent abdominal pain, developed chronic calcific pancreatitis and was found to have pancreas divisum on endoscopic retrograde pancreatography. An intraoperative biopsy showed normal acinar tissue in the head of the pancreas, while the body and tail were replaced by fibrous tissue. His pain resolved following surgical drainage of the dorsal pancreatic duct.Evaluation of the clinical course of this patient and critical review of other such cases in the literature support the role of compromised ductal drainage of the dorsal pancreas in the pathogenesis of chronic pancreatitis in pancreas divisum.