David A. Edelman, MD
Choichi Sugawa, MD
Professor of Surgery
Hiromi Ono, MD
James Tyburski, MD
Associate Professor and Residency
Department of Surgery
Wayne State University
Pancreas divisum is the most common congenital variant of pancreatic ductal development. In symptomatic cases, the underlying mechanism is thought to be a relative outflow obstruction at the site of the minor papilla. Therapeutic interventions for symptomatic pancreas divisum aim to relieve obstruction of the minor papilla by improving pancreatic drainage. The authors report a case of symptomatic pancreas divisum that was successfully treated using a combined approach.
Pancreas divisum occurs when the ventral and dorsal ducts of the embryonic pancreas fail to fuse. It is the most common congenital variant of pancreatic ductal development.1,2 A minority of patients with this condition become symptomatic, experiencing recurrent acute pancreatitis, chronic pancreatitis, or chronic abdominal pain.3 The underlying mechanism in these symptomatic cases is thought to be a relative outflow obstruction at the site of the minor papilla.4 We report a case of symptomatic pancreas divisum that was treated using the combined approach of endoscopic sphincterotomy, surgical transduodenal sphincteroplasty, and repeated stenting of the minor papilla over a 5-year period.
A 33-year-old woman was admitted to the hospital for gallstone pancreatitis, which was treated without complication using laparoscopic cholecystectomy. Postoperatively, the patient's pain never resolved and her pancreatitis continued. An endoscopic retrograde cholangiopancreatography (ERCP) performed 1 month later showed pancreatic divisum and duodenitis around the area of the minor papilla, which itself could not be identified (Figure 1). The patient's condition worsened; she was unable to tolerate a regular diet and required large amounts of narcotics for pain control. She was started on long-term total parenteral nutrition (TPN), and a Hickman catheter was placed.
Approximately 1 month later, our institution tried and failed to perform an endoscopic minor papilla sphincterotomy. The minor papilla could not be cannulated because of the swelling and inflammation surrounding it. The patient continued to require considerable narcotics and was losing weight despite home TPN. She eventually went to a regional tertiary referral center and underwent successful endoscopic sphincterotomy and stent placement in her minor papilla. The regional center was able to cannulate the minor papilla only after using methylene blue dye spray followed by an injection of secretin.5
This procedure relieved the patient's symptoms for 2 months, after which she returned to our institution due to an occluded stent, recurrent pancreatitis, and a scarred papilla. The stent was removed, and the patient underwent a surgical transduodenal minor papilla sphincteroplasty a few months later. Free pancreatic juice from the papilla was noted at the completion of the procedure.
The patient's symptoms resolved completely for 208 days following this procedure. After she returned to the hospital with recurrent pancreatitis and diet intolerance, an ERCP showed scarring at the previous minor papilla sphincterotomy site but a normal-sized dorsal duct (Figure 2). Another sphincterotomy and stent placement were performed endoscopically. The patient did well for the following 6 months, and the stent was removed. Over the next 2 years, however, the patient required six hospital admissions. During each admission, she was allowed nothing by mouth for a few days, after which her symptoms improved and she was discharged to home on a regular diet. Her longest hospitalization during this time was for 7 days.
Approximately 3 months after her last stent was removed, the patient returned to our institution reporting that she was moving to another state. Although she was asymptomatic, a repeat ERCP was performed at her request, and a new No. 7 French stent was placed. The minor papilla was not stenosed at the time and the dorsal pancreatic duct was larger than it had been 5 years earlier.
Pancreatic divisum is often an incidental finding and typically does not cause symptoms, although it may be responsible for up to 25% of unexplained recurrent pancreatitis cases.3,6 ERCP is the gold standard test for diagnosing pancreas divisum. Cannulation of the major papilla reveals a short ventral duct (1 to 4 cm in length) that does not cross the midline. There generally is prompt drainage of contrast, and the morphology of the duct, including ductal branches, appears normal.7 Therapeutic interventions for symptomatic pancreas divisum aim to relieve obstruction of the minor papilla by improving pancreatic drainage. Warshaw and associates found that patients with symptomatic pancreas divisum and acute recurrent pancreatitis benefited the most from surgical sphincteroplasty.4 In this study, the mean followup period was 53 months, and the intraoperative identification of a stenotic papilla was associated with an 85% improvement rate after sphincteroplasty.
In a study evaluating endoscopic minor papillotomy, Gerke and colleagues noted immediate improvement in 60% of patients after this procedure; however, 53% of patients had recurrent symptoms after 6 months.8 The long-term response rate to endoscopic minor papillotomy was only 33% after a median follow-up time of 29 months. The authors reported that endoscopic minor papillotomy was most successful in patients with symptomatic pancreatic divisum and acute recurrent pancreatitis. They suggested that prolonged stenting of the dorsal pancreatic duct can produce irreversible ductal damage and should be avoided.
A retrospective study by Lehman and colleagues showed a 43% improvement in 51 patients with symptomatic pancreatic divisum following minor papillotomy over a dorsal duct stent after a mean follow-up of 20 months.9 A randomized controlled trial by Lans and associates showed that symptomatic patients with pancreatic divisum did better with dorsal duct stenting compared with controls 12 months after stent retrieval.10 Heyries and colleagues reported that insertion of a stent into the dorsal duct appeared to increase the efficacy of the initial sphincterotomy.11 In cases where a stent was placed, no difference in improvement was noted between those who underwent sphincterotomy and those who did not. In Heyries and colleagues' study, the median followup period was 39 months, and stents were left in place for 1 year and exchanged every 4 months.
Ertan treated patients with recurrent pancreatitis secondary to pancreas divisum using endoscopic pancreatic stent placement without pancreatic papillotomy.12 The stents were left in place for 2 to 3 months and then exchanged twice every 2 to 3 months. A 40% stent occlusion rate was noted, suggesting that the stents may have been in place too long. Ikenberry and associates showed the occlusion rate of pancreatic stents increased in an almost linear fashion based on the length of time that the stents were left in the duct.13 By 6 weeks, 50% of the stents were occluded, and all stents were occluded after 9 weeks. Collectively, these studies suggest that therapeutic interventions for symptomatic pancreas divisum can improve patients' symptoms by relieving obstruction of the minor papilla.
Our patient had symptomatic pancreatic divisum, which caused recurrent attacks of pancreatitis. Although initial endoscopic treatment improved her symptoms, they continued to recur. The patient ultimately was treated successfully using a combination of surgical transduodenal minor papilla sphincteroplasty followed by serial endoscopic stent placement over a 5-year period. The patient?s dorsal duct became only minimally dilated during this time. If it had become more dilated and the patient was symptomatic, she would have undergone an open ductal drainage procedure. This case demonstrates that stenting of the dorsal pancreatic duct for an extended period of time can cause ductal dilatation.
Our case illustrates the success of using a combined approach to treat severe pancreatitis secondary to pancreas divisum, which included endoscopic minor papillotomy and stenting, surgical transduodenal sphincteroplasty, and 5 years of repeated stenting of the dorsal pancreatic duct. Our report appears to be the first to describe using a stent in the dorsal pancreatic duct for the long-term treatment of this congenital anomaly. No randomized, controlled trials comparing open surgical procedures with endoscopic ones for the treatment of symptomatic pancreas divisum have been conducted. Although more comprehensive studies regarding the treatment of symptomatic pancreatic divisum are needed, it appears that endoscopic stenting of the dorsal pancreatic duct can cause duct dilation and may be used as a favorable long-term treatment.