Endoscopic Retrograde Cholangiopancreatography Combined with Percutaneous Drainage in Treating Disconnected Pancreatic Duct Syndrome

DPDS was first described by Kozarek¹. The most common underlying cause of disconnected pancreatic duct syndrome (DPDS) is acute necrotizing pancreatitis. The incidence of DPDS in patients with necrotizing pancreatitis ranges from 16.0% to 46.3%^2,3. Clinical manifestations include ongoing pancreatic duct leaks; episodes of recurrent obstructive pancreatitis; recurrent fluid collections around the pancreas, including pseudocysts; and impairment of pancreatic exocrine and endocrine functions. Additionally, patients may develop pancreatic ascites or pancreatic pleural effusion; pseudoaneurysms causing hemorrhage in peripancreatic vessels; and portal hypertension related to pancreatic pathology.

In patients with DPDS caused by acute necrotizing pancreatitis, the disease progression is complex and prolonged, often involving a sequence of pancreatic transmural necrosis, pancreatic duct interruption, and encapsulated pancreatic necrosis⁴. Currently, there is no standard treatment plan for DPDS patients; therefore, early diagnosis and treatment are crucial.

DPDS treatment mainly includes conservative treatment, percutaneous drainage (PCD), endoscopic treatment, and surgical treatment. Currently, endoscopic treatment, which includes endoscopic retrograde cholangiopancreatography (ERCP) and endoscopic ultrasound (EUS), has become a popular approach for DPDS. Compared to EUS-guided transmural drainage, ERCP more closely matches the natural drainage of the duodenal major papilla⁵. However, some studies have found that the failure rate of ERCP alone in treating DPDS can reach up to 75%⁶, and only about one-third of patients succeed with PCD drainage alone⁷. The combination of ERCP and PCD can significantly improve treatment outcomes⁸.

In this study, we focused on treating DPDS with ERCP and PCD, and increased the surgical success rate through the double guidewire technique and localization with methylene blue staining. We presented a case to illustrate our surgical techniques and intraoperative guidance methods; we also assessed the patient's treatment outcomes. A 64-year-old male patient had a lengthy history of pancreatitis and pancreatic fistula. The patient underwent laparoscopic surgery to remove necrotic tissue and drain fluid from acute biliary necrotizing pancreatitis 3 months prior to the hospital visit. The surgery also included gastrostomy and jejunal feeding tube placement. Additionally, the patient received three procedures of PCD to remove necrotic pancreatic tissue. After the surgery, the PCD was draining about 300 mL of pancreatic fluid each day. Though acute pancreatitis was resolved, the PCD was still draining clear pancreatic fluid. The patient underwent ERCP and PCD through the double guidewire technique and localization with methylene blue staining. The follow-up 12 months post-procedure confirmed sufficient and effective drainage and complete healing of the sinus tract, minimizing the risk of complications.

Through this report, we aim to demonstrate the feasibility and effectiveness of this combined approach and provide valuable insights into the endoscopic treatment of DPDS. Ultimately, the findings of this study may help guide clinical decision-making and improve the success rate of ERCP in this challenging area of medicine.

This study was approved by the Ethics Committee of the Fifth Affiliated Hospital of Southern Medical University. Written informed consent was obtained from the patient before the procedure.

1. Patient selection

1. Confirm the indication for surgery. Here, the PCD was draining about 300 mL of pancreatic fluid each day after the initial surgery.

2. Informed consent

1. Discuss the surgical procedure, risks, benefits, and alternatives with the patient, and ensure that informed consent is obtained.

3. Preoperative workup

1. Perform routine laboratory tests, including complete blood count and coagulation profiles.
2. Perform preoperative enhanced computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP) studies to localize pancreatic fluid collections and evaluate the main pancreatic duct (MPD).

4. Operative setup

1. Administer general anesthesia following standard protocols.
2. Position the patient in the left lateral position, which is the regular position of ERCP.
3. Prepare the duodenoscope, X-ray machine, electronic bile duct scope, and surgical materials.

5. Surgical technique

1. Insert the duodenoscope.
   1. Pass the endoscope through the throat, esophagus, and stomach in sequence.
   2. Identify the sunset sign-a characteristic endoscopic feature-at the pylorus, then enter the duodenum and conduct a thorough examination to assess for ulcers or other pathologies of the digestive tract.
   3. Next, locate the main duodenal papilla and position the endoscope appropriately to facilitate further evaluation.
2. Intubate the patient.
   1. Insert the catheter at the 1 o'clock position. After gently pushing the guidewire through the catheter, pull back on the syringe plunger to aspirate and check for pancreatic ductal fluid.
   2. Then, use an X-ray to confirm that the guidewire is in the pancreatic duct (Figure 1A). Then, inject the appropriate contrast agent to clearly visualize the MPD.
      NOTE: During the intubation process, the lead surgeon and the assistant need to coordinate their actions closely. The orientation of the intubation and the assistant's tactile sensitivity are both very important. It is important to avoid forceful intubation or forceful guidewire insertion.
   3. Make a 2 mm incision on the major duodenal papilla at the 1 o'clock position with a scalpel. Then, slowly inflate a balloon catheter to dilate the papilla for 1 minute, maintaining the inflation pressure at 2 atmospheres.
3. Explore using an electronic bile duct scope.
   1. Inject an appropriate contrast agent through the PCD tube until the DPDS cavity is fully visualized. Confirm the anatomical relationship between the MPD and the DPDS cavity using X-ray imaging (Figure 1A).
   2. Next, insert a zebra guidewire. Gradually dilate the sinus tract to 12 French (Fr) along the guidewire using a sheath.
   3. After inserting the endoscope through the sheath, carefully explore the DPDS cavity and locate the endoscopic guidewire.
   4. During the exploration with the bile duct scope, continuously inject saline and use intermittent negative pressure washing to keep a clear view. Then, if the DPDS cavity is straightened, use a stiff bile duct scope.
4. Bridge the disrupted pancreatic duct.
   1. First, repeatedly adjust the scalpel and guidewire under the endoscope, and confirm via X-ray if it can access the DPDS cavity. If unsuccessful, slowly inject methylene blue into the DPDS cavity wall via the MPD (Figure 1B).
   2. Use a choledochoscope to examine the first stained area of the cavity wall (Figure 1C). Gently break the stained cavity wall with a zebra guidewire and a stone retrieval basket until the methylene blue flowing smoothly into the DPDS cavity is seen.
   3. Then, adjust the endoscopic knife and guidewire to enter the cavity through the perforation (Figure 1D). Finally, guide the endoscope wire out of the body (Figure 1E).
   4. Perform preoperative imaging examinations to determine the thickness of the DPDS cyst wall and its relationship to the MPD. If the MPD is near the cyst cavity and the cyst wall is thin, first use an endoscopic knife and a guidewire to break the cyst wall.
   5. When the cyst wall thickens and results in poor staining with methylene blue, repeatedly thin the cyst wall with an endoscopic scalpel and guidewire. If that does not work, directly puncture the cyst wall with the tail end of a black loach guidewire through a peroral cholangiopancreatoscopy.
   6. After the endoscopic guidewire enters the cystic cavity, further advance it through the cyst to the body surface. If that does not work, retrieve the endoscopic guidewire using a basket for stone retrieval under direct visualization using a choledochoscope. Then pull it out to the body surface.
5. Place the pancreatic duct stent.
   1. Once the bridging is successful, use a 10Fr sheath following the tip of the endoscopic guidewire to dilate the tear in the cyst wall where the DPDS meets the MPD under X-ray guidance. This will make it easier to place the pancreatic duct stent and widen the passage.
   2. Choose a pancreatic duct stent (7 Fr, 10 cm) based on the diameter of the main duct and how far the duodenal papilla is from the cyst cavity, making sure that the stent goes into the DPDS cyst cavity by over 2 cm (Figure 1F).
6. Place a bile duct stent and a PCD tube.
   1. After inserting the catheter into the bile duct at the 11 o'clock position, make a 2 mm incision in the duodenal papilla in the 11 o'clock direction with a scalpel, then slowly dilate the papilla using a balloon for 1 min, keeping the pressure at 3 standard atmospheres, and place a plastic stent (7 Fr, 10 cm) in the bile duct.
   2. Insert a silicone drainage tube (18Fr) 8 cm into the cystic space under X-ray guidance through the original skin sinus tract of the PCD (Figure 1F) and then secure it. During the procedure, take care to avoid displacing the previously placed stent in the pancreatic duct.

6. Postoperative care

1. Monitor vital signs, fluid balance, complete blood count, and pancreatitis markers in the postoperative period. If acute pancreatitis and elevated infection markers occur, initiate antimicrobial therapy and symptomatic treatment, including inhibition of pancreatic enzyme activity.
2. Encourage early ambulation and remove the PCD drainage tube on the third day after the operation.
3. Conduct follow-up for any discomfort, such as abdominal pain or fever, and address any postoperative complications.
   1. After 6 months, perform the second ERCP to replace the pancreatic duct plastic stent (10 Fr, 12 cm).
   2. After 12 months, perform the third ERCP to place a nasal pancreatic duct catheter (8.5 Fr) for temporary drainage.
      NOTE: After the third ERCP and placement of the stent, the overall treatment duration was 1 year for the case described here. Ultimately, the DPDS was completely resolved.

From March 2023 to May 2025, ERCP + PCD was performed on 2 patients in our department. The key to a successful surgery lies in whether the junction between the MPD and the cyst can be successfully breached. In one patient, the endoscopic guidewire was successfully navigated into the cyst cavity after several attempts during treatment. However, in this patient of this study, despite several attempts with the endoscopic guidewire, it could not enter the cyst cavity. The cyst wall junction was marked with methylene blue, and then, the cyst wall was breached using a stone retrieval basket and a zebra guidewire with a choledochoscope. This method combines internal and external approaches and successfully achieves physiological drainage of the major duodenal papilla. For instance, Figure 2A illustrates the inside-out approach, showcasing the rupture of the cyst wall at the lateral side of the cyst cavity of the DPDS. Figure 2B illustrates the outside-in approach, showcasing the rupture of the cyst wall on the inner side of the DPDS cyst cavity.

The overall procedure duration of 180 min is reasonable for complete DPDS, and the blood loss during the operation was only 1 mL. The patient was discharged on the seventh day without major adverse events, such as hemorrhage or acute pancreatitis, underscoring the safety of this technique (Table 1). Figure 3A,B further demonstrates the situation of DPDS after 6 months and 12 months of traffic diversion, confirming sufficient and effective drainage and complete healing of the sinus tract, thereby minimizing the risk of complications.

Figure 1: ERCP combined with the choledochoscope process. (A) Confirm the anatomical relationship between MPD and DPDS, represented by white arrows. (B) Inject methylene blue (white arrow). (C) Examine the earliest stained area (white arrows) on the cavity wall. (D) Adjust the endoscopic guidewire to enter the DPDS cavity (white arrows). (E) Guide the endoscopic guidewire (white arrow) out of the body. (F) Place a pancreatic duct stent (7 Fr, 10 cm) and an 18 Fr PCD tube, represented by white arrows. Please click here to view a larger version of this figure.

Figure 2: Two different paths to break the DPDS cyst wall. (A) Break the cyst wall from MPD into the cyst cavity. (B) Break the cyst wall from the cyst cavity into MPD. Please click here to view a larger version of this figure.

Figure 3: Follow-up. (A) Effective drainage confirmed (white arrows) after 6 months. (B) The disappearance of the cavity (white arrows) was confirmed, and the complete cure of DPDS after 12 months. Please click here to view a larger version of this figure.

Table 1: The details of the surgery.

Currently, endoscopic treatment is a popular option for treating patients with DPDS, which mainly includes ERCP, EUS, ERCP with PCD, and ERCP with EUS. According to different drainage methods, it is mainly divided into transpapillary drainage and transmural drainage.Transpapillary drainage is physiologic and more attractive. Compared with transwall drainage, transpapillary drainage does not require creating an alternative nonanatomical route of drainage. ERCP combined with PCD is one of the methods of transpapillary drainage, which is mainly used in those with larger fluid collections, with a lot of solid debris, as well as in patients where PCD treatment has failed².

PCD has become an effective treatment for the first stage of acute necrotizing pancreatitis. Moreover, we can repeatedly remove the necrotic tissue of the pancreas through the PCD sinus tract. After the pancreatic fluid drained by PCD becomes clear, the second stage of treatment can be initiated. Rana et al.⁹ found that patients whose PCD have a low-flow pancreatic fistula (<200 mL/day) after acute necrotizing pancreatitis can spontaneously close within 3 months without intervention. However, PCD, which is an iatrogenic external pancreatic fistulae (EPF) in the setting of DPDS, is difficult to manage and causes infective and nutritional complications resulting in prolonged hospital stay and necessitating multiple interventions¹⁰.

ERCP is considered the gold standard for the diagnosis of DPDS, which directly describes the type of MPD disruption. However, the approach to endoscopic treatment of DPDS depends on the presence or absence of co-existing pancreatic fluid collection (PFC)⁴. Multiple studies have been conducted that show the successful resolution of PFC and MPD disruption depends on the type of disruption^10,11. Compared with complete MPD disruption, the success rate of ERCP for partial MPD disruption is much higher, and the transpapillary drainage of ERCP is more effective in patients with partial PD disruption¹².

Due to the poor results of endoscopic transpapillary therapy or PCD, many patients with DPDS were more likely to require multi-modality complex hybrid endoscopic approaches, such as ERCP combined with PCD and EUS combined with PCD or ERCP. A recent retrospective study reports that 167 patients (46.3%) had DPDS in 361 patients with PFC, and 31.1% DPDS patients require multi-modality complex hybrid therapy². Compared with various EUS-guided mainstream methods, ERCP combined with PCD is mostly a case report^4,8,9. From March 2023 to May 2025, we successfully treated two patients with complete DPDS caused by necrotizing pancreatitis using ERCP combined with PCD, with no complications, including pancreatic leakage and acute pancreatitis, occurring after the operation. However, based on our personal experience, the total treatment time for patients with complete DPDS is at least as long as 1 year. Starting from the first successful treatment of ERCP combined with PCD, ERCP needs to be performed every six months to replace the pancreatic duct stent. However, 1 year later, the disrupted pancreatic duct has been completely drained and unobstructed. If preoperative MRCP confirms the patency of MPD and the disappearance of PFC, the pancreatic duct stent can be removed through the third ERCP.

However, this protocol has several obvious limitations. For instance, ERCP combined with PCD is infrequently used and technically challenging, which requires an experienced interventional gastroenterologist and pancreatic surgery expert to provide appropriate treatment for DPDS patients and reduce surgical complications¹³. As a pancreatic surgeon, surgery remains the cornerstone for the management of DPDS patients, as the treatment outcomes with endoscopic transpapillary drainage are poor. Additionally, the presence of PFC suggests an internal drainage disorder of the secretions of a disconnected pancreas. Experienced pancreatic experts and interventional gastroenterologists are needed to evaluate the specific anatomical relationship between the stomach, MPD, PFC, and the surrounding blood vessels based on imaging examinations before the operation⁴. If the diameter of the MPD is less than 2 mm, the thickness of the PFC sac wall exceeds 1 cm, there is still pus flowing out of the PCD, and the distance between the MPD and the PFC exceeds 1 cm, transmural drainage is an alternative option before other more invasive procedures, such as surgical operation. Moreover, the entire treatment process lasts for up to 1 year, and two more ERCPs are required to replace the pancreatic duct stent from the successful first stent bridging. During the treatment period, in addition to well-known complications such as postoperative pancreatitis and PCD or intestinal infection into sterile MPD, transpapillary drainage also increases the risk of stent-induced catheter and parenchymal changes similar to chronic pancreatitis.

Overall, this report has confirmed the feasibility, effectiveness, and safety of ERCP combined with PCD in treating patients with complete DPDS. However, given that the number of the above-mentioned cases is relatively small, further studies are needed to confirm more details and address the remaining difficulties. Future research could focus on direct-view bridging technology, which combines cholangiopancreatoscopy and choledochoscopy through ERCP, along with PCD, to improve visualization in more challenging DPDS cases.