Disconnected pancreatic duct syndrome (DPDS) is a pathological condition that causes various symptoms due to the continuous secretion of pancreatic enzymes from the pancreas upstream, which has been separated due to disconnection of the pancreatic duct (DPD) for various reasons.Acute necrotizing pancreatitis includes a certain probability of DPDS appearance, which makes it necessary to provide various treatments for DPDS. Furthermore, DPDS can impact long-term results, such as recurrence and impaired pancreatic function. Although the development of various modalities has contributed to diagnosis and treatment, especially less invasive endoscopic therapy, DPDS is often overlooked, and the diagnosis can be delayed due to the lack of consensus on its definition and classification. This review summarizes the current knowledge and challenges of DPDS and discusses the optimal strategy for its diagnosis and treatment, as well as future perspectives.

Given the lack of established definition, diagnosis, and treatment of DPDS, we conducted a thorough review of the existing literature.

It is emphasized that a standardized definition and classification of DPDS is essential for designing and conducting clinical studies to address current unmet needs in managing patients with DPDS.

Disconnected pancreatic duct syndrome (DPDS) is a rare complication of a common disease. Typically, DPDS occurs in acute necrotizing pancreatitis (ANP), chronic pancreatitis, abdominal surgery, or trauma. We present a case of DPDS from acute non-necrotizing pancreatitis (ANNP). A 41-year-old male with a history of alcohol use and prior AP presented with progressive, severe left-sided abdominal pain that was worse with movement. Labs revealed a lipase of 95 U/L (normal range 11-82 U/L). Computed tomography (CT) of the abdomen/pelvis (A/P) with IV contrast demonstrated a large left-sided pleural effusion, non-necrotic pancreatic pseudocysts, and a large subdiaphragmatic fluid collection. Thoracentesis of the pleural effusion revealed an amylase of 601 U/L confirming pancreatic etiology. A subsequent magnetic resonance cholangiopancreatography (MRCP) confirmed complex peripancreatic ascites, rapid subdiaphragmatic fluid accumulation, and a fistula from the pancreatic tail to retroperitoneum concerning for a rapidly dissecting pancreatic pseudocyst. He ultimately underwent endoscopic retrograde cholangiopancreatography (ERCP) with stent placement in the main pancreatic duct. His left-sided abdominal pain rapidly improved, and the patient was discharged. CT A/P one week after discharge showed a reduced size of subdiaphragmatic fluid collection. DPDS is usually seen in patients with a history of ANP. Our case demonstrates that it can also occur in ANNP, which has not previously been described in the literature. Therefore, a high index of clinical suspicion must be maintained for DPDS even in ANNP given its potential for severe complications.

Disconnected pancreatic duct syndrome (DPDS) is an important and common complication of acute necrotizing pancreatitis. Endoscopic approach has been established as the first-line treatment for pancreatic fluid collections (PFCs) with less invasion and satisfactory outcome. However, the presence of DPDS significantly complicates the management of PFC; besides, there is no standardized treatment for DPDS. The diagnosis of DPDS presents the first step of management, which can be preliminarily established by imaging methods including contrast-enhanced computed tomography, ERCP, magnetic resonance cholangiopancreatography (MRCP), and EUS. Historically, ERCP is considered as the gold standard for the diagnosis of DPDS, and secretin-enhanced MRCP is recommended as an appropriate diagnostic method in existing guidelines. With the development of endoscopic techniques and accessories, the endoscopic approach, mainly including transpapillary and transmural drainage, has been developed as the preferred treatment over percutaneous drainage and surgery for the management of PFC with DPDS. Many studies concerning various endoscopic treatment strategies have been published, especially in the recent 5 years. Nonetheless, existing current literature has reported inconsistent and confusing results. In this article, the latest evidence is summarized to explore the optimal endoscopic management of PFC with DPDS.

Necrotizing pancreatitis may result in a disrupted or disconnected pancreatic duct (DPD) with the potential for long lasting negative impact on a patient's clinical outcome. There is a lack of detailed data on the full clinical spectrum of DPD which is critical for the development of better diagnostic and treatment strategies.

We performed a long-term post-hoc analysis of a prospectively collected nationwide cohort of 896 patients with necrotizing pancreatitis (2005-2015). The median follow-up after hospital admission was 75 months (P25-P75:41-151). Clinical outcomes of patients with and without DPD were compared using regression analyses, adjusted for potential confounders. Predictive features for DPD were explored.

DPD was confirmed in 243 (27%) of the 896 patients and resulted in worse clinical outcomes during both the patient's initial admission and follow-up. During hospital admission, DPD was associated with an increased rate of new-onset intensive care unit admission (adjusted-OR2.52 [95%-CI 1.62-3.93]), new-onset organ failure (adjusted-OR2.26 [95%-CI 1.45-3.55]), infected necrosis (adjusted-OR4.63 [95%-CI 2.87-7.64]) and pancreatic interventions (adjusted-OR7.55 [95%-CI 4.23-13.96]). During long-term follow-up, DPD increased the risk of pancreatic intervention (adjusted-OR9.71 [95%-CI 5.37-18.30], recurrent pancreatitis (adjusted-OR2.08 [95%-CI 1.32-3.29]), chronic pancreatitis (adjusted-OR2.73 [95%-CI 1.47-5.15]) and endocrine pancreatic insufficiency (adjusted-OR1.63 [95%-CI 1.05-2.53]).Central or subtotal pancreatic necrosis on computed tomography (CT), (OR9.49 [95%-CI 6.31-14.29] and a high levels of serum C-reactive protein (CRP) in the first 48 hours after admission (per 10 points increase, OR1.02 [95%-CI 1.00-1.03] were identified as independent predictors for developing DPD.

At least one of every four patients with necrotizing pancreatitis suffer from DPD which is associated with detrimental, short and long-term interventions and complications. Central and subtotal pancreatic necrosis and high levels of serum CRP in the first 48 hours are independent predictors for DPD.

Disconnected duct syndrome (DDS) is an adverse sequela of acute necrotizing pancreatitis in which there is disruption of the pancreatic duct leading to a failure to deliver pancreatic secretions into the duodenum. Its presentation may range from a persistent external pancreatic fistula to a treatment-resistant pancreatic pseudocyst. The diagnosis is often delayed in the acute setting if there is concurrent necrosis as management is often directed to the associated peripancreatic fluid collection. A combination of imaging modalities may be required to evaluate ductal anatomy. No definitive consensus has been achieved regarding the optimal treatment strategy for DDS, and there is a lack of published level I evidence on the topic. Treatment should be tailored to each individual patient, depending on the anatomy of ductal disruption, the presence of sepsis, the degree of physiological derangement, and the patients' performance status. Patients with DDS represent a specific cohort of patients with complex pancreatic disease that requires the input from a diverse multidisciplinary team to ensure that a good clinical outcome can be achieved.

Pancreatic fluid collections often develop as a complication of acute pancreatitis but can be seen in a variety of conditions including chronic pancreatitis, trauma, malignancy or post-operatively. It is important to classify a pancreatic fluid collection in order to optimize treatment strategies and management. Most interventions are targeted towards the management of delayed complications of pancreatitis, including pancreatic pseudocysts and walled-off necrosis (WON), which often develop days to weeks after the initial episode of pancreatitis. Surgical, percutaneous, and endoscopic interventions are all possible methods for treatment of pancreatic fluid collections, however endoscopic drainage with endoscopic ultrasound has become first-line. Advances within endoscopic drainage strategies have also led to innovative changes in the specific stents used for treatment, with possible options including double pigtail plastic stents, fully covered self-expanding metal stents and lumen-apposing metal stents (LAMS).

Disconnected pancreatic duct syndrome (DPDS) is a recognized complication of necrotizing pancreatitis (NP). Manifestations include recurrent peripancreatic fluid collections (R-PFC) and pancreatocutaneous fistulae (PC-Fistulae). Pancreatitis of the disconnected pancreatic segment (DPDS-P) and its relationship to new-onset diabetes after pancreatitis (NODAP) are not well characterized.

We performed a retrospective cohort study of consecutive patients with NP admitted to University of California, San Francisco from January 2011 to June 2019. A diagnosis of a disconnected pancreatic duct (PD) was confirmed using computed tomography and magnetic resonance cholangiopancreatography/endoscopic retrograde cholangiopancreatography. DPDS was defined as a disconnected PD presenting with R-PFC, PC-Fistulae, or DPDS-P. The primary outcome was NODAP, defined as diabetes mellitus (DM) occurring >3 months after NP. Cox proportional hazards regression was used to evaluate the relationship between DPDS and NODAP.

Of 171 patients with NP in this study, the mean clinical follow-up was 46 ± 18 months and the imaging follow-up was 38 ± 20 months. Twenty-seven patients (16%) developed DPDS-P at a median of 28 months. New-onset DM occurred in 54 of the 148 patients (36%), with 22% developing DM within 3 months of NP and 14% developing NODAP at a median of 31 months after AP. DPDS-P was associated with NODAP when compared with non-DPDS patients (adjusted hazard ratio 5.63 95% confidence interval: 1.69-18.74, P = 0.005) while R-PFCs and PC-Fistulae were not.

DPDS and NODAP occurred in 28% and 14% of the patients, respectively. Pancreatitis of the disconnected pancreas occurred in 16% of the patients and was associated with higher rates of NODAP when compared with patients with other manifestations of DPDS and patients without DPDS.

Pancreatic duct disruption or disconnection is a potentially severe complication of necrotizing pancreatitis. With no existing treatment guidelines, it is unclear whether there is any consensus among experts in clinical practice. We evaluated current expert opinion regarding the diagnosis and treatment of pancreatic duct disruption and disconnection in an international case vignette study.

An online case vignette survey was sent to 110 international expert pancreatologists. Expert selection was based on publications in the last 5 years and/or participation in development of IAP/APA and ESGE guidelines on acute pancreatitis. Consensus was defined as agreement by at least 75% of the experts.

The response rate was 51% (n = 56). Forty-four experts (79%) obtained a MRI/MRCP and 52 experts (93%) measured amylase levels in percutaneous drain fluid to evaluate pancreatic duct integrity. The majority of experts favored endoscopic transluminal drainage for infected (peri)pancreatic necrosis and pancreatic duct disruption (84%, n = 45) or disconnection (88%, n = 43). Consensus was lacking regarding the treatment of patients with persistent percutaneous drain production, and with persistent sterile necrosis.

This international survey of experts demonstrates that there are many areas for which no consensus existed, providing clear focus for future investigation.

Pleural effusion secondary to a pancreatico-pleural fistula is a very rare presentation in children, with limited reports in the literature. We describe two differing presentations of pleural effusions resulting from chronic pancreatitis (CP) with successful resolution of the pleural effusion. These cases highlight the need for consideration of this rare paediatric diagnosis, and the variety of investigations, management strategies, and complications that can occur in the setting of CP in children.

Severe pancreatitis may result in a disrupted pancreatic duct, which is associated with a complicated clinical course. Diagnosis of a disrupted pancreatic duct is not standardized in clinical practice or international guidelines. We performed a systematic review of the literature on imaging modalities for diagnosing a disrupted pancreatic duct in patients with acute pancreatitis.

A systematic search was performed in PubMed, Embase and Cochrane library databases to identify all studies evaluating diagnostic modalities for the diagnosis of a disrupted pancreatic duct in acute pancreatitis. All data regarding diagnostic accuracy were extracted.

We included 8 studies, evaluating five different diagnostic modalities in 142 patients with severe acute pancreatitis. Study quality was assessed, with proportionally divided high and low risk of bias and low applicability concerns in 75% of the studies. A sensitivity of 100% was reported for endoscopic ultrasound and endoscopic retrograde cholangiopancreatography. The sensitivity of magnetic resonance cholangiopancreatography with or without secretin was 83%. A sensitivity of 92% was demonstrated for a combined cohort of secretin-magnetic resonance cholangiopancreatography and magnetic resonance cholangiopancreatography. A sensitivity of 100% and specificity of 50% was found for amylase measurements in drain fluid compared with ERCP.

This review suggests that various diagnostic modalities are accurate in diagnosing a disrupted pancreatic duct in patients with acute pancreatitis. Amylase measurement in drain fluid should be standardized. Given the invasive nature of other modalities, secretin-magnetic resonance cholangiopancreatography or magnetic resonance cholangiopancreatography would be recommended as first diagnostic modality. Further prospective studies, however, are needed.

Disconnected pancreatic duct syndrome (DPDS) is a complication of acute necrotizing pancreatitis in the neck and body of the pancreas often manifesting as persistent pancreatic fluid collection (PFC) or external pancreatic fistula (EPF). This systematic review and pairwise meta-analysis aimed to review the definitions, clinical presentation, intervention, and outcomes for DPDS.

The PubMed, EMBASE, MEDLINE, and SCOPUS databases were systematically searched until February 2020 using the PRISMA framework. A meta-analysis was performed to assess the success rates of endoscopic and surgical interventions for the treatment of DPDS. Success of DPDS treatment was defined as long-term resolution of symptoms without recurrence of PFC, EPF, or pancreatic ascites.

Thirty studies were included in the quantitative analysis comprising 1355 patients. Acute pancreatitis was the most common etiology (95.3%, 936/982), followed by chronic pancreatitis (3.1%, 30/982). DPDS commonly presented with PFC (83.2%, 948/1140) and EPF (13.4%, 153/1140). There was significant heterogeneity in the definition of DPDS in the literature. Weighted success rate of endoscopic transmural drainage (90.6%, 95%-CI 81.0-95.6%) was significantly higher than transpapillary drainage (58.5%, 95%-CI 36.7-77.4). Pairwise meta-analysis showed comparable success rates between endoscopic and surgical intervention, which were 82% (weighted 95%-CI 68.6-90.5) and 87.4% (95%-CI 81.2-91.8), respectively (P = 0.389).

Endoscopic transmural drainage was superior to transpapillary drainage for the management of DPDS. Endoscopic and surgical interventions had comparable success rates. The significant variability in the definitions and treatment strategies for DPDS warrant standardisation for further research.

Disconnectedpancreatic duct syndrome (DPDS), a severe complication of acute necrotizing pancreatitis (ANP), may require surgery, usually by distal splenopancreatectomy, thus increasing the risk of diabetes. We describe a new technique reconnecting the distal pancreas to the digestive tract.

This technique was proposed after failure of non-surgical treatment and at least 3 months after the onset of ANP in non-diabetic or non-insulin dependent diabetic patients with a distal pancreas of at least 5 cm. The ruptured zone was identified and the distal side was anastomosed to the stomach or the jejunum.

From 2013 to June 2019, 36 patients (median age = 49 years) with DPDS underwent a "French reconnection" procedure, indicated for chronic pain/recurrent pancreatitis (n = 35; 97%), persistent pancreatic fistula (n = 33; 91%), or digestive compression/fistulisation (n = 9; 25%). Median preoperative weight loss was 10 kg (4-27), the median number of hospitalisations per patient was 5(1-8) and 24(67%) patients had received endoscopic/percutaneous treatment. Surgery was performed in median 279(90-2000) days after ANP, laparoscopically in 9(25%) patients. The remnant pancreas (median length = 70 mm; range = 50-130) was anastomosed to the stomach (n = 30) or the jejunum (n = 6). There were 13(36%) postoperative grade B/C pancreatic fistulas and 3(10%) bleedings including one death (mortality = 3%). The median hospital stay was 18 (7-121) days. After a median follow-up of 24 (4-53) months, all pancreatic fistulas had healed and the clinical success rate was 91%. Median BMI increased from 22 to 25 kg/m². In patients with normal pancreatic function, postoperative de novo endocrine and severe exocrine insufficiencies were observed in 4/27 (15%) and 7/22 (32%), respectively.

The "French reconnection" procedure, as an alternative to distal splenopancreatectomy for the treatment of DPDS, provides good control of symptoms and decreases the risk of pancreatic insufficiency.

Pancreatic fluids collections are local complications related to acute or chronic pancreatitis and may require intervention when symptomatic and/or complicated. Within the last decade, endoscopic management of these collections via endoscopic ultrasound-guided transmural drainage has become the gold standard treatment for encapsulated pancreatic collections with high clinical success and lower morbidity compared to traditional surgery and percutaneous drainage. Proper understanding of anatomic landmarks, including assessment of the main pancreatic duct and any associated lesions - such as disruptions and strictures - are key to achieving clinical success, reducing the need for reintervention or recurrence, especially in cases with suspected disconnected pancreatic duct syndrome. Additionally, proper review of imaging and anatomic landmarks, including collection location, are pivotal to determine type and size of pancreatic stenting as well as approach using long-term transmural indwelling plastic stents. Pancreatography to adequately assess the main pancreatic duct may be performed by two methods: Either non-invasively using magnetic resonance cholangiopancreatography or endoscopically via retrograde cholangiopan-creatography. Despite the critical need to understand anatomy via pancrea-tography and assess the main pancreatic duct, a standardized approach or uniform assessment strategy has not been described in the literature. Therefore, the aim of this review was to clarify the role of pancreatography in the endoscopic management of encapsulated pancreatic collections and to propose a new classification system to aid in proper assessment and endoscopic treatment.

Disconnected Pancreatic Duct Syndrome (DPDS) is an important but often overlooked complication of acute necrotising pancreatitis (ANP) that occurs due to necrosis of the main pancreatic duct (PD). This segmental necrosis leads on to disconnection between the viable upstream pancreatic parenchyma and the duodenum. The disconnected and functional segment of pancreas continues to secrete pancreatic juice that is not drained into the gastrointestinal tract and lead on to recurrent pancreatic fluid collections (PFC), refractory external pancreatic flstulae and chronic abdominal pain/recurrent pancreatitis. Because of lack of awareness of this important complication of ANP, the diagnosis of DPDS is usually delayed. The delay in diagnosis increases the morbidity of the disease as well as increase the cost of treatment and duration of hospital stay. Surgery has remained the cornerstone for management of patients with DPDS. The conventional surgical approaches have been either resection or internal drainage procedures. Surgery for DPDS in the setting of ANP is often difficult due to presence of local inflammation and extensive venous collaterals in the operative field due to splenic vein thrombosis and therefore is associated with significant morbidity. Advancement in therapeutic endoscopy, especially advent of therapeutic endoscopic ultrasound has opened an exciting new field of minimally invasive therapeutic options for management of DPDS. The present review discusses the current understanding of the clinical manifestations, imaging features and management strategies in patients with DPDS.

Endoscopic cystogastrostomy for mature pancreatic collections has long been recognized. However, FDA approval of newer lumen-apposing metallic stents in 2014 has now brought pancreatic necrosectomy to the endoscopic realm. Endoscopic drainage of Walled-off necrosis and direct endoscopic necrosectomy are technically challenging procedures with higher rates of complications. Collaborative clinical decision making both pre- and post-procedurally between the radiologist, endoscopist, and the surgeon can greatly improve outcomes in necrotizing pancreatitis. Herein, we review the basic pathophysiology that underlies progressive radiographic findings in NP, value of preprocedural imaging, current management algorithms, newer tools, and techniques as well as potential post-procedure complications on imaging follow-up after endoscopic interventions in necrotizing pancreatitis.

External pancreatic fistulae (EPF) associated with complete pancreatic duct (PD) disruption leading on to disconnected pancreatic duct syndrome (DPDS) is a therapeutic nightmare usually requiring surgery. However, spontaneous closure also has been reported. There is paucity of data on the natural history of EPF associated with DPDS.

To retrospectively study outcome of conservative treatment in patients with low output (<200 ml/day) EPF with DPDS following percutaneous or surgical intervention in acute necrotising pancreatitis (ANP).

The data of patients of low output EPF with DPDS treated conservatively in our unit over last 5 years was retrospectively analysed. Their clinical course, complications as well as time taken for fistula closure was retrieved.

33 patients (27 males; mean age: 40.5 ± 9.3 years) of low output EPF and DPDS were studied. 31 patients developed EPF following percutaneous drainage (PCD) and 2 patients developed fistula following surgery. The drain fluid amylase ranged from 1600 to 32,000 IU/l and site of disruption was neck, proximal body and distal body in 4, 16 and 13 patients respectively. EPF closed spontaneously in all patients within 88.2 ± 63.46 days. PCD slipped out in 2 patients and led to formation of pseudocyst in 1 patient that was treated endoscopically. There has been no recurrence in any patient over follow up of 32.5 ± 21.9 months.

Low output EPF developing post PCD or surgery in patients with DPDS following ANP closely spontaneously in majority of patients within 3 months with good long term outcome.

Necrotizing pancreatitis may lead to loss of integrity of the pancreatic duct, resulting in leakage of pancreatic fluid. Pancreatic duct disruption or disconnection is associated with a prolonged disease course and particular complications. Since a standard treatment for this condition is currently lacking, we performed a systematic review of the literature to compare outcomes of various treatment strategies.

A systematic review was performed according to the PRISMA guidelines in the PubMed, EMBASE and Cochrane databases. Included were articles considering the treatment of patients with disrupted or disconnected pancreatic duct resulting from acute necrotizing pancreatitis.

Overall, 21 observational cohort studies were included comprising a total of 583 relevant patients. The most frequently used treatment strategies included endoscopic transpapillary drainage, endoscopic transluminal drainage, surgical drainage or resection, or combined procedures. Pooled analysis showed success rates of 81% (95%-CI: 60-92%) for transpapillary and 92% (95%-CI: 77-98%) for transluminal drainage, 80% (95%-CI: 67-89%) for distal pancreatectomy and 84% (95%-CI: 73-91%) for cyst-jejunostomy. Success rates did not differ between surgical procedures (cyst-jejunostomy and distal pancreatectomy (risk ratio = 1.06, p = .26)) but distal pancreatectomy was associated with a higher incidence of endocrine pancreatic insufficiency (risk ratio = 3.06, p = .01). The success rate of conservative treatment is unknown.

Different treatment strategies for pancreatic duct disruption and duct disconnection after necrotizing pancreatitis show high success rates but various sources of bias in the available studies are likely. High-quality prospective, studies, including unselected patients, are needed to establish the most effective treatment in specific subgroups of patients, including timing of treatment and long-term follow-up.

The incidence of acute pancreatitis continues to increase worldwide, and it is one of the most common gastrointestinal causes for hospital admission in the USA. In the past decade, substantial advancements have been made in our understanding of the pathophysiological mechanisms of acute pancreatitis. Studies have elucidated mechanisms of calcium-mediated acinar cell injury and death and the importance of store-operated calcium entry channels and mitochondrial permeability transition pores. The cytoprotective role of the unfolded protein response and autophagy in preventing sustained endoplasmic reticulum stress, apoptosis and necrosis has also been characterized, as has the central role of unsaturated fatty acids in causing pancreatic organ failure. Characterization of these pathways has led to the identification of potential molecular targets for future therapeutic trials. At the patient level, two classification systems have been developed to classify the severity of acute pancreatitis into prognostically meaningful groups, and several landmark clinical trials have informed management strategies in areas of nutritional support and interventions for infected pancreatic necrosis that have resulted in important changes to acute pancreatitis management paradigms. In this Review, we provide a summary of recent advances in acute pancreatitis with a special emphasis on pathophysiological mechanisms and clinical management of the disorder.

Endoscopic ultrasound-guided drainage of symptomatic walled-off pancreatic necrosis (WON) usually has been performed with double pigtail plastic stents (DPS) and more recently, with lumen-apposing metal stents (LAMS). However, LAMS are significantly more expensive and there are no comparative studies with DPS. Accordingly, we compared our experience with combined endoscopic and percutaneous drainage (dual-modality drainage [DMD]) for symptomatic WON using LAMS versus DPS.

Patients who underwent DMD of WON between July 2011 and June 2016 using LAMS were compared with a matched group treated with DPS. Technical success, clinical success, need for reintervention and adverse events (AE) were recorded.

A total of 50 patients (31 males, 25 patients treated with LAMS and 25 patients treated with DPS) were matched for age, sex, computed tomography severity index, and disconnected pancreatic ducts. Technical success was achieved in all patients. Mean days hospitalized post-intervention (14.5 vs. 13.1, P  = 0.72), time to resolution of WON (77 days vs. 63 days, P  = 0.57) and mean follow-up (207 days vs. 258 days, P  = 0.34) were comparable in both groups. AEs were similar in both groups (6 vs. 8, P  = 0.53). Patients treated with LAMS had significantly more reinterventions per patient (1.5 vs. 0.72, P  = 0.01).

 In treatment of symptomatic WON using DMD, LAMS did not shorten time to percutaneous drain removal and was not associated with fewer AEs.