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.     

Patency of the accessory pancreatic duct evaluated by dye‐injection endoscopic retrograde pancreatography: Methods and clinical implication

The accessory pancreatic duct (APD) is sometimes developmentally obliterated near the duodenum. We evaluated patency of the APD by dye‐injection endoscopic retrograde pancreatography (ERP). We injected 2–3 mL contrast medium containing indigocarmine into the main pancreatic duct (MPD) via a selectively cannulated endoscopic catheter. Patency of the APD was evaluated by observing the excretion of dye from the minor duodenal papilla. Of the 291 control cases studied, 43% demonstrated a patent APD. Patency of the APD in patients with acute pancreatitis was only 17%, signi?cantly lower than that of controls (P < 0.01). Mean caliber of patent APD was 1.6 ± 0.5 mm, signi?cantly greater than the 1.1 ± 0.5 mm of non‐patent APD (P < 0.01). Regarding the terminal shape of the APD, spindle‐ and cudgel‐type APD were frequently patent (93% and 88%, respectively, (P < 0.01). With respect to APD course, long‐type APD showed most frequent patency (75%, P < 0.01). Dye‐injection ERP represents a simple and de?nitive method for examining APD function. A patent APD may prevent acute pancreatitis by reducing pressure in the MPD. Patency of the APD might be dependent on duct caliber, course, and terminal shape.     

DOES A PATENT ACCESSORY PANCREATIC DUCT PREVENT ACUTE PANCREATITIS?

Background and Aim: The role of the accessory pancreatic duct (APD) in pancreatic pathophysiology has been unclear. We previously examined the patency of the APD in 291 control cases who had a normal pancreatogram in the head of the pancreas by dye‐injection endoscopic retrograde pancreatography (ERP). APD patency was 43% and was closely related with the shape of the terminal portion of the APD. The present study aimed to clarify the clinical implications of a patent APD. Methods: Based on the underlying data, the patency rate of the APD was estimated from the terminal shape of the APD on ERP in 167 patients with acute pancreatitis. Results: In patients with acute pancreatitis, stick‐type APD, spindle‐type APD, and cudgel‐type APD, which showed a high patency, were rare, and branch‐type APD and halfway‐type or no APD, which showed quite low patency, were frequent in acute pancreatitis patients. Accordingly, the estimated patency of the APD in acute pancreatitis patients was only 21%. There was no significant relationship between the estimated APD patency and etiology or severity of acute pancreatitis. Conclusions: The terminal shapes of the APD with low patency were frequent in acute pancreatitis patients, and estimated APD patency was only 21% in acute pancreatitis. A patent APD may function as a second drainage system to reduce the pressure in the main pancreatic duct and prevent acute pancreatitis.     

Clinical significance of the accessory pancreatic duct

BACKGROUND/AIMS: The accessory pancreatic duct is the smaller and less constant pancreatic duct in comparison with the main pancreatic duct. We investigated the patency of the accessory pancreatic duct and its role in pancreatic pathophysiology. METHODOLOGY: Dye-injection endoscopic retrograde pancreatography was performed in 411 patients. In patients in whom the main pancreatic duct could be selectively cannulated, contrast medium with indigo carmine was injected through the catheter. Excretion of the dye from the minor duodenal papilla was observed endoscopically. RESULTS: Patency of the accessory pancreatic duct was 43% of the 291 control cases. In the 46 patients with acute pancreatitis, 8 (17%) had a patent accessory pancreatic duct. The difference in patency between this group and the normal group was significant (p < 0.01). Especially, patency of the accessory pancreatic duct was only 8% of the 13 patients with acute biliary pancreatitis. In the patients with pancreaticobiliary maljunction, biliary carcinoma occurred in 72% of patients with a nonpatent accessory pancreatic duct, but in contrast, it occurred only in 30% of those with a patent accessory pancreatic duct. This difference was significant (p < 0.05). Lower amylase level in the bile of patients with pancreaticobiliary maljunction with a patent accessory pancreatic duct was frequently observed than those with a nonpatent accessory pancreatic duct. CONCLUSIONS: A patent accessory pancreatic duct may prevent acute pancreatitis by lowering the pressure in the main pancreatic duct. In cases of pancreaticobiliary maljunction with a patent accessory pancreatic duct, the incidence of carcinogenesis of the bile duct might be lower, as the reflux of the pancreatic juice to the bile duct might be reduced by the flow of the pancreatic juice into the duodenum through the accessory pancreatic duct.     

Size,location and patency of the minor duodenal papilla as determined by dye‐injection endoscopic retrograde pancreatography

Background: The accessory pancreatic duct (APD) sometimes is developmentally obliterated near the duodenum. We evaluated patency of the minor duodenal papilla by dye‐injection endoscopic retrograde pancreatography to determine whether patency was related to papillary size and location. Methods: We injected 2–3 mL of contrast material containing indigocarmine into the main pancreatic duct via an endoscopic catheter in 104 patients. It was endoscopically observed whether dye was extruded from the minor papilla. Size of the minor papilla and distance from the orifice of the major duodenal papilla to the apex of the minor papilla were measured endoscopically with measuring forceps. Results: The APD was patent in 56 of 104 cases (54%). Size of the minor papilla varied considerably from 3 to 6 mm, but showed no correlation with patency. Half of the patients with chronic pancreatitis (6/13) had the minor papilla larger than 6 mm. In cases where the terminal APD had a cudgel or tapering‐off configuration, the minor papilla was larger than in cases where the duct had a stick shape. The minor papilla was patent in 9 out of 10 cases (90%) when it was near the major papilla (≤ 1.5 cm). Frequency of a patent minor papilla was 16 out of 33 (48%) when it existed 1.5 to 2.0 cm from the major papilla, and 31 out of 61 (51%) when the distance was more than 2.0 cm. Conclusions: The minor papilla was more frequently patent when it was close to the major papilla (P < 0.05).     

Involvement of pancreatic and bile ducts in autoimmune pancreatitis

AIM:To examine the involvement of the pancreatic andbile ducts in patients with autoimmune pancreatitis.METHODS:Clinical and cholangiopancreatographic find-ings of 28 patients with autoimmune pancreatitis wereevaluated.For the purposes of this study,the pancreaticduct system was divided into three portions:the ventralpancreatic duct;the head portion of the dorsal pancre-atic duct;and the body and tail of the dorsal pancreaticduct.RESULTS:Both the ventral and dorsal pancreatic ductswere involved in 24 patients,while in 4 patients only thedorsal pancreatic duct was involved.Marked stricture ofthe bile duct was detected in 20 patients and their initialsymptom was obstructive jaundice.Six patients showedmoderate stenosis to 30%-40% of the normal diameter,and the other two patients showed no stenosis of thebile duct.Although marked stricture of the bile duct wasdetected in 83%(20/24)of patients who showed nar-rowing of both the ventral and dorsal pancreatic ducts,itwas not observed in the 4 patients who showed involve-ment of the dorsal pancreatic duct alone(P=0.0034).     

Pancreas divisum in pancreaticobiliary maljunction

BACKGROUND/AIMS: Pancreaticobiliary maljunction (PBM) and pancreas divisum (PD) are congenital anomalies that develop in the embryo at an early stage. They are possibly the result of bile and pancreatic duct misarrangement. We investigated the configuration of the pancreatic duct in patients with PBM and its clinical implications. METHODOLOGY: In 84 PBM patients, the configuration of the pancreatic duct and the presence of biliary cancer were documented. Patency of Santorini's duct was determined fluoroscopically or by dye-injection endoscopic retrograde pancreatography. Bile amylase levels were measured in 10 patients. RESULTS: Incomplete PD was detected in 8 (9.5%) of the 84 PBM patients. All of the 8 patients had a patent Santorini's duct, and only 1 patient had gallbladder cancer. The frequency of associated gallbladder cancer and the bile amylase level were significantly lower in PBM patients with a patent Santorini's duct than in PBM patients with a nonpatent Santorini's duct. CONCLUSIONS: PBM is sometimes associated with incomplete PD. In PBM patients with an incomplete PD, the incidence of cancer of the biliary tract may be lower, since pancreatic juice reflux into the bile duct might be reduced by the flow of pancreatic juice into the duodenum through Santorini's duct.     

Biliary carcinoma risk in patients with pancreaticobiliary maljunction and the degree of extrahepatic bile duct dilatation

BACKGROUND/AIMS: Pancreaticobiliary maljunction (PBM) carries a high risk of biliary carcinoma. This study aimed to examine the biliary complications of patients with PBM in relation to the degree of extra-hepatic bile duct dilatation. METHODOLOGY: Ninety-eight cases of PBM could be divided into 5 groups according to the maximum diameter of the extrahepatic bile duct: < or = 10mm, 11-15mm, 16-20mm, 21-30mm, > or = 31mm. The clinicopathological findings of biliary carcinomas associated with PBM were compared with 232 cases of gallbladder carcinoma and 159 cases of bile duct carcinoma that were not associated with PBM. RESULTS: Gallbladder carcinoma occurred in 36 of 65 patients (55%) with PBM whose maximum diameter of the extrahepatic bile duct was < or = 30mm, but no gallbladder carcinoma occurred in patients with PBM whose diameter was > or = 31mm. Bile duct carcinoma occurred in 6 of 52 patients (12%) with PBM whose diameter was > or = 21mm, but no bile duct carcinoma occurred in patients with PBM whose diameter was < or = 20mm. The age at diagnosis of the patients with gallbladder or bile duct carcinoma associated with PBM was significantly younger than those without PBM (p<0.01). CONCLUSIONS: PBM with an extrahepatic bile duct diameter < or = 30mm is associated with a high risk of gallbladder carcinoma. PBM with an extrahepatic bile duct diameter > or = 21mm is associated with a high risk of bile duct carcinoma. Prophylactic cholecystectomy is recommended for patients with PBM without biliary dilatation.     

Pancreatographic findings in idiopathic acute pancreatitis

Background/purpose

Despite extensive evaluation based on clinical history, biochemical tests, and noninvasive imaging studies, the cause of acute pancreatitis cannot be determined in 10 to 30% of patients, and a diagnosis of idiopathic acute pancreatitis is made. The purpose of this study was to clarify the pancreatographic findings in patients with idiopathic acute pancreatitis.

Methods

Endoscopic retrograde cholangiopancreatography (ERCP) was performed in 34 patients with idiopathic acute pancreatitis, and the pancreatographic findings were examined. Patency of the accessory pancreatic duct was examined by dye-injection endoscopic retrograde pancreatography (ERP) in 16 of the 34 patients.

Results

In 11 patients (32%), the following anatomic abnormalities of the pancreatic or biliary system were demonstrated: complete pancreas divisum (n = 5), incomplete pancreas divisum (n = 2), high confluence of pancreaticobiliary ducts (n = 2), choledochocele (n = 1), and giant periampullary diverticulum (n = 1). Pancreatographic findings were normal in 17 patients. Eleven of these patients were examined by dye-injection ERP, and all were found to have nonpatent accessory pancreatic duct.

Conclusions

Anatomic abnormality of the pancreatic or biliary system is one of the major causes of idiopathic acute pancreatitis. Closure of the accessory pancreatic duct may play a role in the development of idiopathic acute pancreatitis in patients with a normal pancreaticobiliary ductal system.     

Cancer of the gallbladder associated with pancreaticobiliary maljunction without bile duct dilatation in a European patient

A rare case of pancreaticobiliary maljunction (PBM) without dilatation of the biliary tract (DBT) associated with gallbladder carcinoma is described herein. A 62‐year‐old European woman with a long history of right upper abdominal pain was diagnosed as having PBM without DBT by endoscopic retrograde cholangiopancreatography and other examinations. Excision of the gallbladder and biliary duct with a Roux‐en‐Y hepaticojejunostomy was performed, and subsequent pathological examination of the surgical specimen showed a well differentiated adenocarcinoma of the gallbladder. She had no clinical symptoms for 58 months postoperatively. PBM allows reflux of pancreatic juice into the biliary tract. Recent findings support the idea that epithelial hyperplasia plays an important role in gallbladder carcinogenesis with PBM, and also support the concept that gene mutations are involved in the carcinogenesis of biliary epithelium in patients with PBM. For these reasons, we advocate that resection of the extrahepatic biliary tract in PBM patients without bile duct dilatation, rather than cholecystectomy alone, is the treatment of choice for preventing bile duct carcinoma.     

Patency of the accessory pancreatic duct in relation to its course and shape: a dye-injection endoscopic retrograde pancreatography study

Objective: The accessory pancreatic duct (APD) exhibits several appearances on pancreatography. We examined the patency of the APD by dye-injection endoscopic retrograde pancreatography (ERP), and studied the relationship between patency and duct course and shape.
Methods: There were 213 patients with satisfactory imaging of the entire normal APD who also underwent dye-injection ERP. The length and maximum diameter of the APD and the length of the main pancreatic duct (MPD) from its orifice to the junction with the APD were measured.
Results: The caliber of the patent APD was 1.6 ± 0.6 mm. This was significantly larger than the caliber (1.1 ± 0.4 mm) of the nonpatent APD (   p < 0.01  ). The length of the MPD from its orifice to the junction with the patent APD was 32.7 ± 12.5 mm. This was significantly longer than the length to the junction with the nonpatent APD (22.5 ± 8.1 mm) (   p < 0.01  ). The APD was classified according to duct course: long type, intermediate type, short type, or ansa type. Patency was most common in the long type APD (74.5%). The terminal shape of the APD was also used to classify the ducts: stick type, branch type, saccular type, cudgel type, or spindle type. Patency was most frequently observed in the spindle and cudgel type ducts.
Conclusions: Patency of the APD might be dependent on duct caliber, course, and terminal shape of the duct.     

Clinical and radiological findings in dominance of Santorini's duct

BACKGROUND/AIMS: Few studies on Santorini's duct dominance, in which the ventral pancreatic duct is narrower than and anastomoses with Santorini's duct have been performed. We examined clinical and radiological findings in cases characterized by dominance of Santorini's duct. METHODS: We reviewed 3,800 cases of endoscopic retrograde cholangiopancreatography. Clinical and pancreatographic findings including caliber, course, terminal shape, and patency of Santorini's duct were examined in cases of Santorini's duct dominance. RESULTS: Twenty-nine cases were diagnosed as Santorini's duct dominant. Chronic pancreatitis, acute relapsing pancreatitis, pancreatic-type pain, and hyperamylasemia not associated with obvious pancreatitis were observed in 3, 1, 5, and 6 cases, respectively. Cholangiopancreatographic findings indicated congenital choledochal cyst (n = 2), branch fusion between the ventral and dorsal pancreatic ducts (n = 23), and normal pancreatic duct system characterized by a straight course through the body and tail to join the ventral pancreatic duct in the neck portion of the pancreas (n = 4). Regarding terminal shapes of Santorini's duct, cudgel type (n = 9) and spindle type (n = 8), which showed frequent patency, were observed significantly more frequently than in controls. Patency of Santorini's duct was observed in 90% (17/19). CONCLUSIONS: Many Santorini's duct-dominant cases exhibited branch fusion between the ventral and dorsal pancreatic ducts. Although Santorini's duct functions well in most cases in which it is dominant, pancreatitis or pancreatic-type pain occurs in half of such cases due to relative impairment of function of the minor duodenal papilla.     

Magnetic resonance cholangiopancreatography study of pancreaticobiliary maljunction and pancreaticobiliary diseases

AIM:To discuss the imaging anatomy about pancreaticobiliary ductal union,occurrence rate of pancreaticobiliary maljunction(PBM)and associated diseases in a Chinese population by using magnetic resonance cholangiopancreatography(MRCP).METHODS:Data were collected from 694 patients who underwent MRCP from January 2010 to December2012.Three hundred and ninety-three patients were male and 301 patients were female.The age range was16-92 years old and the average age was 51.8 years.The recruitment indication of all cases was patients who had clinical symptoms,such as abdominal pain,jaundice,nausea and vomiting,which thus were clinically suspected as relative pancreaticobiliary diseases.All cases were examined by MRCP using single-shot fast spin-echo sequences.In order to obtain MRCP images,the maximum intensity projection was used.RESULTS:According to the anatomy of pancreaticobiliary ductal union based on our analysis of MRCP images,all cases were classified into normal type and abnormal type according to the position of pancreaticobiliary ductal union.The abnormal type could be further divided into P-B type,B-P type and the duodenum type.By analyzing the incidence of biliary stone and inflammation,pancreatitis,biliary duct tumors and pancreatic tumors between normal and abnormal types,significant differences existed.The abnormal group was more likely to suffer from pancreaticobiliary diseases.Comparing three different types of PBM that were associated with pancreaticobiliary diseases by using Fisher’s method,the result showed that there was no significant difference in the incidence of biliary stones,cholecystitis and pancreatic tumors.The incidence of pancreatitis in B-P type and P-B type was higher than that in duodenum type;the incidence of biliary duct tumor in B-P type was higher than that in P-B type;the incidence of biliary duct tumor in duodenum type was lower than that in P-B type.The incidence of congenital choledochus dilatation in normal type and abnormal type was similar,and there was no significant difference between the two types.CONCLUSION:Types of PBM are closely related to the occurrence of pancreaticobiliary diseases.MRCP has important clinical value in the early diagnosis and preventive treatment of pancreaticobiliary diseases.     

Segmental groove pancreatitis accompanied by protein plugs in Santorini's duct

"Groove pancreatitis", a form of segmental pancreatitis affecting the head of the pancreas, is local-ized within the "groove" between pancreas head, duo-denum, and common bile duct. Differentiation between groove pancreatitis and pancreatic head carcinoma is often difficult. We report a case of groove pancreatitis in which a hypoechoic mass between the duodenal wall and pancreas was clearly imaged, and narrowing of the second portion of the duodenum and bile duct stenosis were also found. The diagnosis was confirmed by surgery (pylorus-preserving pancreato duodenectomy). The patient was relieved from abdominal pain post operation. Up to the present, the patient has been good condition. We review the clinicopathologic and radiologic features of groove pancreatitis in the Japanese literature and discuss the possible role of Santorini's duct in its pathogenesis. We consider that impacted protein plugs in Santorini's duct are a pathogenic factor in the development of groove pancreatitis. Therefore, the findings of Santorini's duct on endoscopic retrograde pancreatography are very important in the diagnosis of groove pancreatitis. Groove pancreatitis presents various clinical features, such as biliary stenosis, duodenal stenosis, and pancreatic mass, and often masquerades as pancreatic head carcinoma. This condition should be kept in mind in the differential diagnosis of pancreatic head carcinoma. (Received Apr. 17, 1997; accepted Sept. 26, 1997)     

Spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis.

BACKGROUND: Autoimmune pancreatitis is an evolving entity. METHODS: A patient who had spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis is presented. RESULTS: A 58-year-old diabetic woman with jaundice was referred for pancreatic head carcinoma diagnosed by magnetic resonance imaging (MRI). At laparotomy, a pancreatic head mass (4 x 3 cm) that involved the transverse mesocolon and two other hard masses (1 cm) in the pancreatic body and tail were found. The gallbladder was palpated as a hard tumor mass. Frozen section examination of the gallbladder and pancreatic biopsies revealed cholecystitis and pancreatitis with lymphoplasmacytic infiltration. The common bile duct was brittle and unsuitable for anastomosis. Starting 1 month after the operation, drainage from the biliary catheter decreased gradually and stopped. There was no parenchymal lesion on MRI examination in the 2nd postoperative month. Cholangiography from the percutaneous catheter showed flow of contrast agent into the duodenum. Serum immunoglobulin G, G4 and E levels were increased. CONCLUSION: To the best of our knowledge, this is the first report of spontaneous regression of a pancreatic head mass and biliary obstruction due to autoimmune pancreatitis.     

胰头部囊腺瘤局部切除术前内镜下置入胆胰管支架的安全性及疗效分析

为探讨内镜逆行胰胆管造影（endoscopic retrograde cholangiopancreatography，ERCP）下胆胰管支架置入联合胰腺局部切除术（enucleation，En）治疗胰头部囊腺瘤的安全性与临床疗效，回顾性分析2020年1月—2023年1月杭州市第一人民医院行ERCP+En（ERCP+En组，n=11）与En（En组，n=12）治疗的胰头部囊腺瘤患者临床资料，对比两组一般情况、术中情况、围术期并发症、住院时间及随访结果。两组患者一般资料差异无统计学意义（P>0.05）。ERCP+En组中，ERCP置入胆胰管支架顺利，术后出现高淀粉酶血症3例，经保守治疗好转。两组En术中均无中转开腹、输血发生，术后均无严重并发症。ERCP+En组与En组术后B/C级胰瘘分别为0例和3例（P=0.001），中位住院时间分别为11 d和15 d，差异有统计学意义（U=2.25，P=0.031）；两组中位En时间（145 min比155 min，U=0.03，P=0.952）、中位术中出血量（100 mL比120 mL，U=0.05，P=0.784）差异无统计学意义。中位随访18个月，两组患者均无复发，ERCP+En组无胆胰管狭窄发生，En组中2例发生胰管狭窄，1例发生胆管狭窄。内镜下胆胰管支架置入联合En治疗胰头部囊腺瘤可有效减少术后胰瘘，避免远期胆胰管狭窄等并发症。     

Recent advances in pathophysiology and surgical treatment of congenital dilatation of the bile duct

The introduction of the concept of pancreaticobiliary maljunction (PBM) has led to significant progress in the pathogenetic study and surgical treatment of congenital dilatation of the bile duct (CDBD). Clinical studies of 155 patients with CDBD, and basic experimental studies of the mechanisms of bile duct dilatation and associated pancreatitis were conducted. The CDBD was morphologically classified as either cystic or cylindrical type. Clinical signs, symptoms, and laboratory findings were highly dependent on two factors: the age at onset and the reflux of pancreatic juice into the bile duct through the PBM. The canine experimental model of PBM exhibited not only significant bile duct dilatation but also morphological findings similar to those in the CDBD patients. Subsequent studies in a rat model clarified the involvement of activated pancreatic enzyme phospholipase A₂ in PBM‐associated pancreatitis. For surgical treatment, cystoenterostomy has been superseded by hepatico‐enterostomy after removal of the damaged bile ducts and blocking their communication with the pancreatic duct. Long‐term follow‐up studies have shown excellent results by confirming the surgical correction based on the pathological principles. Regarding biliary reconstruction, jejunal interposition hepatico‐duodenostomy, which is, theoretically, a logical approach was unexpectedly shown to often lead to bile reflux gastritis, so it is now superseded by Roux‐en‐Y hepaticojejunostomy.     

Patency of accessory pancreatic duct and its relation with duodenal inter papillary distance

A patent accessory pancreatic duct (APD), which acts as a safety valve, may prevent complications of endoscopic retrograde cholangiopancreatography. The interpapillary distance is probably the easiest parameter to assess the probability of APD patency. In previous studies, the patency of APD was correlated with other morphometric parameters of the accessory duct. The present study assessed the frequency of APD patency among south Indian adult cadavers of both sexes, and correlated it with the interpapillary distance. Duodeno-pancreas specimens collected from 100 cadavers with no recorded diseases of biliary pancreatic tree were studied by routine dissection method and dye injection technique. APD had a patent communication with the duodenum in 46 specimens, and was more frequent in men. The distance between the two duodenal papillae varied from 1.6–3 cm; 98% of the patent APD specimens had an interpapillary distance of ≥2 cm. We postulate that an duodenal interpapillary distance ≥2 cm suggests patency of APD.     

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.     

Recent advances in pancreaticobiliary maljunction

The purpose of this review is to evaluate our current knowledge of the embryologic etiology of pancreaticobiliary maljunction (PBM), its diagnosis, clinical aspects, and treatment, and to clarify the mechanisms of PBM involvement in carcinogenesis. Although the embryologic etiology of PBM still awaits clarification, an arrest of the migration of the common duct of the biliary and pancreatic ducts inwards in the duodenal wall has hitherto been speculated to result in a long common channel in PBM. However, we propose the hypothesis that the etiology of PBM is caused by a disturbance in the embryonic connections (misarrangement) of the choledochopancreatic duct system in the extremely early embryo. That is, PBM is an anomaly caused by a misarrangement whereby the terminal bile duct joins with a branch of the ventral pancreatic duct system, including the main pancreatic duct. PBM is frequently associated with congenital bile duct cyst (CCBD). However, these two anomalies are thought to have different embryonic etiologies. The diagnostic criteria for PBM are the radiological and anatomical detection of the extramural location of the junction of the pancreatic and biliary ducts in the duodenal wall. However, in PBM patients with a short common duct (less than 1 cm in length), detection of the extramural location is difficult. The clinical features of PBM are intermittent abdominal pain, with or without elevation of pancreatic enzyme levels; and obstructive jaundice, with or without acute pancreatitis, while the clinical features of PBM patients with CCBD are primary bile duct stone and acute cholangitis. The optimum approach for the treatment of PBM is the prevention of the reciprocal reflux of bile and pancreatic juice in the pancreas and the bile duct system. To achieve these aims, the surgical approach is most effective, and complete biliary diversion procedures with bile duct resection (for example, choledochoduodenostomy or choledochojejunostomy of the Roux‐en‐Y type) are most useful. Recently, it has been recognized that the development of biliary ductal carcinoma is associated with PBM. That is, the development of gallbladder cancer occurs frequently in PBM patients without CCBD, and bile duct cancer originating from the cyst wall also occurs in PBM patients with CCBD. It is speculated that the pathogenesis of the bile duct or gallbladder cancer in PBM patients involves the reciprocal reflux of bile and pancreatic juice. Investigations of epithelial cell proliferation in the gallbladder of PBM patients, and of K‐ras mutations andp53 suppressor gene mutations, loss of heterozygosity ofp53, and overexpression of thep53 gene product in gallbladder cancer and noncancerous lesions in PBM patients have been carried out in various laboratories around the world. The results support the conclusion that PBM is a high risk factor for the development of bile duct carcinoma.