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Background and Aim Pancreaticobiliary anomalies are rare and often present with cryptic signs and symptoms, thus delaying appropriate treatment. Methods Endoscopic retrograde cholangiopancreatography (ERCP) was used to define pancreaticobiliary anomalies. A retrospective review was performed of 5522 ERCPs conducted at a tertiary care center from 1972 to 2015. Results There were 249 (4.5%) patients with pancreaticobiliary anomalies, including 179 patients with pancreas divisum (PD), 44 patients with choledochal cyst (CC) (Todani's classification Type I: extrahepatic cyst 31, Type III; choledochocele 9, Type V: Caroli's disease 4), 20 patients with anomalous pancreaticobiliary ductal union (APDU), and 6 patients with other abnormalities. Of 179 patients with pancreas divisum, 8 (4.5%) required minor sphincterotomies for multiple unexplained acute pancreatitis. Of the 31, 15 (48%) Type I CC patients underwent an operation. In patients with Type III CC (choledochocele), seven of the nine were treated by endoscopic sphincterotomy, and two patients were treated by surgery. Four patients with Type V CC (Caroli's disease) were managed nonoperatively. Of the 20 patients with APDU, 8 (40%) required operative intervention. Six patients were found to have other anomalies: two with pancreas bifidum, one with a duplication of the gallbladder, one with a cystic duct diverticulum, one with an annular pancreas, and one with an abnormal cystic duct origin. These patients were treated based on their etiology. Conclusion Pancreaticobiliary anomalies are rare and can be defined using ERCP. The appreciation of these abnormalities is important for the proper diagnosis and treatment of these rare biliary and pancreatic disorders., Pancreaticobiliary anomalies were analyzed using endoscopic retrograde cholangiopancreatography (5522 patients) in an urban medical center in the United States. A total of 249 (4.5%) patients were found to have a pancreaticobiliary anomaly. There were 179 patients with pancreas divisum, 44 patients with choledochal cyst, 20 patients with anomalous pancreaticobiliary ductal union, and 6 patients with other abnormalities.

Clinical literature indicates that pancreas divisum (PD) is present in 3-22% of the population and may be associated with an increased risk of pancreatitis. PD is a developmental variant wherein the duct systems derived from the dorsal and ventral pancreatic buds are not fused. Hence secretions from the head, neck, body, and tail, which develop from the dorsal bud, must pass through the minor duodenal papilla. The smaller uncinate process, derived from the ventral bud, drains through the major duodenal papilla. The purpose of this study was: (1) to do a cadaveric dissection to confirm whether PD is common in donors who had not been selected because they had pancreatitis and (2) to determine the frequency of PD descriptions in anatomy, embryology, pathology, and surgery books in our libraries. For our anatomical study, pancreata of eight human donors were dissected. Dye was injected into the ducts so that any communications between main and accessory ducts could be easily located. For our literature review, 22 anatomy, 14 embryology, 11 pathology, and 26 surgery books were examined for mention of PD. PD was unambiguously identified in two donor cadavers. However, only 14% of the anatomy plus embryology books compared to 70% of the surgery plus pathology books describe PD. Cadaveric dissection confirms that PD is indeed prevalent. The prevalence of PD with its increased risk of pancreatitis merits inclusion of this topic in textbooks of anatomy and embryology.

Objective The aim of this study was to determine the diagnosis and endoscopic management of pancreas divisum with results from long-term experience at our institution. Methods A prospectively collected database of all patients who underwent endoscopic retrograde cholangiopancreatography (ERCP) with the diagnosis of pancreas divisum at our institution from January 2001 to April 2010 was retrospectively analyzed. Results A total of 45 patients were identified with pancreas divisum with 62 ERCP procedures. The major indication for ERCP included pancreatitis in 33 patients (73.3%), of whom 18 had idiopathic recurrent acute pancreatitis, 8 had recurrent pancreatic-type pain and 7 had chronic pancreatitis. The median size of the incision of sphincterotomy was 5 mm (range 3–8 mm). In all 37 patients (82.2%) underwent placement of stent into the dorsal pancreatic duct, with a median stent size of 5 Fr by 7 cm (range 3–10 Fr by 3–12 cm). The overall response rate was 75.8%. Conclusions The frequency of finding pancreas divisum during ERCP varies among institutions and is low compared with the autopsy series, given that many patients may remain asymptomatic or might not undergo ERCP even if the symptoms develop. Our study is the first to describe specific procedure-related details during therapeutic endoscopy for pancreas divisum.

Background and Aims: External pancreatic fistulas (EPFs) are a therapeutic challenge. The present study was conducted to evaluate the efficacy of endoscopic transpapillary nasopancreatic drainage (NPD) in patients with EPF. Methods: Over 12 years, 23 patients (19 males) with EPF underwent attempted endoscopic transpapillary NPD. The end points were fistula closure with healing of pancreatic duct disruption on nasopancreatogram, or need for surgery. Results: All 23 patients had persistent drain output (>50 mL/day) for >6 weeks. The mean output volume of the fistula was 223 mL (range: 60 mL to 750 mL). Sixteen patients had partial and seven patients had complete pancreatic duct disruption. The NPD could be successfully placed in 21/23 (91.3%) patients. Disruption was bridged in 15 of 16 patients with partial duct disruption. EPF healed in 2–8 weeks of placement of NPD in all of the patients with partial duct disruption that was bridged and there was no recurrence at a mean follow-up of 38 months. The EPF resolved in only 2/6 (33%) patients with complete duct disruption. Conclusions: External pancreatic fistulas developing following percutaneous drainage of pancreatic fluid collections or surgical necrosectomy can be effectively treated by transpapillary nasopancreatic drain placement especially when there is partial ductal disruption and the disruption can be bridged.

To clarify the anatomy of the pancreatic duct system and to investigate its embryology, we reviewed 256 pancreatograms with normal pancreatic head, 81 with pancreas divisum and 74 with pancreaticobiliary maljunction. Accessory pancreatograms were divided into two patterns. The long-type accessory pancreatic duct forms a straight line and joins the main pancreatic duct at the neck portion of the pancreas. The short-type accessory pancreatic duct joins the main pancreatic duct near its first inferior branch. The short-type accessory pancreatic duct is less likely to have a long inferior branch arising from the accessory pancreatic duct. The length of the accessory pancreatic duct from the orifice to the first long inferior branch was similar in the short- and long-type accessory pancreatic ducts. The first long inferior branch from the long-type accessory pancreatic duct passes though the main pancreatic duct near the origin of the inferior branch from the main pancreatic duct. Immunohistochemically, in the short-type accessory pancreatic duct, the main pancreatic duct between the junction with the short-type accessory pancreatic duct and the neck portion was located in the ventral pancreas. The long-type accessory pancreatic duct represents a continuation of the main duct of the dorsal pancreatic bud. The short-type accessory pancreatic duct is probably formed by the proximal main duct of the dorsal pancreatic bud and its long inferior branch.

Objectives To describe the clinical picture and outcome, and to assess the etiological factors of acute and recurrent pancreatitis in children. Methods Thirty-six (65.5%) patients with acute and 19 (34.5%) with recurrent pancreatitis were studied. Mean age was 126 (41.3 SD) months; 27 (49.1%) were females. Setting A pediatric referral hospital. Period 2000-2005. Design Cross-sectional. Variables Clinical and laboratory data and etiological factors. Statistics Chi2-test, Fisher test, OR, confidence interval, Student t-test and Mann-Whitney U-test. Results The most frequent symptom in acute and recurrent pancreatitis was abdominal pain, followed by vomiting and ileus. The severity of pancreatitis and complications were similar in both groups. Biliary stones, family history of pancreatitis, drug ingestion and hypercalcemia occurred in both groups. Abdominal trauma and acute hepatitis A occurred in patients with acute pancreatitis; triglyceride>5.65 mmol/L, pancreas divisum and DeltaF508 mutation occurred in patients with recurrent pancreatitis. No difference was observed when frequency factors between study groups were compared. Conclusions The clinical picture and etiological factors were similar in both groups. Since one out of every three children with acute pancreatitis in this series presented recurrences, it was not considered to be a 'benign disease'. Fifteen different etiological factors were identified in two-thirds of the cases.

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.

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. © 2005 Blackwell Publishing Asia Pty Ltd

The morphological characteristics and incidence of pancreas divisum (PD) in Niigata were investigated by studying 16,646 cases diagnosed by ERCP. These cases had been patients in 16 hospitals located in Niigata prefecture. PD was found in 114 (63 cases confirmed, 51 cases unconfirmed) of these 16,646 cases (0.68%). An unconfirmed case was defined as being a patient in whom only the short ventral pancreatic duct was visible through the main papilla, and the confirmed cases were classified into the 3 types according to the ductal systems of the dorsal and ventral pancreas. The classification of these 3 types was as follows. Type 1: Nonfusion between the ventral (VPD) and dorsal pancreatic ducts (DPD), which was visualized through the main papilla (MP) and the accessory papilla (AP), separately. Type 2: Only DPD was visualized through the AP. In spite of the cannulation of the MP, the VPD was not demonstrated. Type 3: Miscellaneous cases. The frequency of Type 1, Type 2, and Type 3 was 58.7% (37 cases), 28.6% (18 cases), and 12.7% (8 cases), respectively. Type 3 consisted of two subtypes. One (7 cases) was regarded as being when the patient had a very fine communicating branch between VPD and DPD, whether an obvious communicating branch could be detected or not by ERCP. The other subtype (only one case) showed very strange findings in which through the AP a short pancreatic duct distributed to the head of the pancreas and to the uncinate process was visualized and in which through the MP a long pancreatic duct to the tail was demonstrated, separately.

The aim of this study was to indirectly evaluate functional aspects of the accessory papilla, by measuring the diameter of the dorsal duct in non-fusion (pancreas divisum). In 56 patients with definite non-fusion, the diameter of the duct on ERCP was measured with a caliper rule, in the ventral duct (V1, V2), in the head of the dorsal duct (Dh1, Dh2), the body (Dbl, Db2), and the tail (Dt1, Dt2). The controls were 61 consecutive patients with a normally fused pancreatic duct system. The mean value ± SD were V1:2.0±1.1, V2:1.7±0.8, Dh1:2.5±0.6, Dh2:2.6±0.8, Dbl:2.7±0.8, Db2: 2.1 ±0.5, Dt1: 1.7±0.5, Dt2: 0.9±0.2 in the non-fusion group. The values in the control group were 3.4±0.9, 3.0±0.8, 1.0±0.3, 1.3±0.4, 2.6±0.6, 2.3±0.6, 1.6 ± 0.3, 0.9 ± 0.2, respectively. Results showed that the diameter of the pancreatic duct was significantly related to the age of the patient in the body and tail of the non-fusion group but not in the controls (p

Five cases of pancreas divisum, clinically diagnosed by endoscopic retrograde choledochopancreatography (ERCP), were studied histopathologically. They consisted of four biopsied cases and one patient who underwent pancreatoduodenectomy under the diagnosis of a suspected pancreatic carcinoma. Fibrosis was found in three out of the five cases, two cases with fibrosis in the dorsal pancreas and one in the ventral pancreas. In the two dorsal fibrosis pancreas cases, fibrosis was distributed in the intra- and interlobular areas and was accompanied by atrophy or disappearance of the acinar cells. These findings were compatible with chronic obstructive pancreatitis, distal to a stricture of the pancreatic duct. In the one ventral fibrosis pancreas case, i. e., the pancreatoduodenectomized case, fibrosis was distributed mainly in the interlobular area associated with hemosiderin deposition. Hemosiderin deposition was also observed in inflammatory foci at the posterior aspect of the ventral pancreas. Hence, such interlobular fibrosis developed in relation to the inflammatory foci, and was not caused by pancreas divisum. Therefore, in pancreas divisum, abnormal findings were observed in the dorsal pancreas tissue, and they were compatible with chronic obstructive pancreatitis. (Dig Endosc 1994; 6 : 74–79)

It has been suggested that acute pancreatitis occurs more commonly in patients with pancreas divisum and that these patients may respond to surgery aimed at improving pancreatic ductal drainage. We have studied them incidence of pancreas divisum in patients referred for endoscopic retrograde cholangio pancreatography (ERCP) and the results of surgical sphincteroplasty in a separate series of such patients. Twenty-three patients with pancreas divisum were identified among 336 successful pancreatograms (Group A), an incidence of 6.8%. The incidence of pancreas divisum in patients having ERCP for documented pancreatitis was 13% (11 of 86) compared with 4.8% (12 of 250) in those having ERCP for other indications. This difference was statistically significant (P < 0.05). However, pancreas divisum was not the sole risk factor for pancreatitis in the majority of our patients; most also had one of the commonly recognized causes for their pancreatitis. There is dispute about the indications for surgery in patients with recurrent acute pancreatitis and pancreas divisum, but without any other risk factor. We have reviewed the results of operations on 13 patients with pancreas divisum (Group B) treated in four different hospitals. Surgical sphincteroplasty was carried out on 10 patients with documented pancreatitis and seven of these had good results. Three patients who had operations for pain without documented pancreatitis were not improved.

Aman, aged 41, was seen in the Emergency Service because of the acute onset of pain in the right upper quadrant of the abdomen that was associated with nausea. He described similar episodes on two previous occasions. On examination of the abdomen, the only abnormality was mild tenderness on palpation over the right upper quadrant. Screening blood tests including liver function tests and serum amylase were within the reference range. His pain settled with analgesia and he was given an outpatient appointment for an upper abdominal ultrasound scan. The gallbladder was poorly seen but the possibility was raised of a contracted gallbladder with multiple stones. Because of continuing minor symptoms, he was advised to proceed with elective laparoscopic cholecystectomy.At operation, it was not possible to identify the gallbladder. The appearance of the gallbladder fossa is shown in Figure 1. Magnetic resonance cholangiopancreatography (MRCP) was performed after surgery and showed congenital absence of the cystic duct and gallbladder (Figure 2). An incidental finding was that of pancreas divisum. Agenesis of the gallbladder is a rare congenital anomaly with an estimated prevalence of between 1 and 10 per 10,000 people in the general population. The anomaly is usually sporadic although there are occasional reports of two affected members within families. Embryologically, the anomaly is presumed to arise because of a defect in the development of the gallbladder bud that arises from the caudal portion of the hepatic bud. Most patients do not have a cystic duct stump. At surgery, gallbladder agenesis should only be diagnosed after a careful search of ectopic locations, particularly an intrahepatic or left-sided gallbladder. Intraoperative cholangiography may also be helpful if the bile duct can be readily identified. However, extensive surgical dissection of the area should be avoided as this may result in injury to hilar structures. MRCP either before or after surgery may also be helpful in those patients with uncertain results from ultrasound or computed tomography scans. In the above patient, possible causes for pain include an atypical irritable bowel syndrome, a motility disorder of the sphincter of Oddi and perhaps pancreas divisum. There are also rare reports of agenesis of the gallbladder with primary bile duct stones.