In a subset of patients with acute necrotizing pancreatitis, segmental necrosis affecting the main pancreatic duct may result in a discontinuity between the left-sided pancreas and the duodenum. Such an interruption in the setting of a viable upstream portion of the gland can give rise to the disconnected pancreatic duct syndrome (DPDS). By maintaining its secretory function, the disconnected segment may lead to persistent external pancreatic fistulae, recurrent pancreatic fluid collections, and/or obstructive recurrent acute or chronic pancreatitis of the isolated parenchyma. There are currently no universally accepted guidelines for the diagnosis or treatment of DPDS, and because the condition is underrecognized, the diagnosis is often delayed. DPDS is associated with a prolonged disease course and poses a burden on patients' quality of life as well as high health care resource utilization. The aim of our review is to summarize current knowledge, discuss diagnostic approaches, outline management options, and raise awareness of this challenging complication of necrotizing pancreatitis.

2nd Department of Internal Medicine Gastroenterology and Geriatrics University Hospital Olomouc Faculty of Medicine Palacky University Olomouc Czech Republic

Division of Gastroenterology Hepatology and Nutrition University of Minnesota Minneapolis MN USA

Zobrazit více v PubMed

Banks P.A., Bollen T.L., Dervenis C., et al. Classification of acute pancreatitis - 2012: revision of the Atlanta classification and definitions by international consensus. Gut. 2013;62:102–111. doi: 10.1136/gutjnl-2012-302779. PubMed DOI

Freeman M.L., Werner J., van Santvoort H.C., et al. Interventions for necrotizing pancreatitis: summary of a multidisciplinary consensus conference. Pancreas. 2012;41(8):1176–1194. doi: 10.1097/MPA.0b013e318269c660. PubMed DOI

Arvanitakis M., Dumonceau J.M., Albert J., et al. Endoscopic management of acute necrotizing pancreatitis: European Society of Gastrointestinal Endoscopy (ESGE) evidence-based multidisciplinary guidelines. Endoscopy. 2018;50(5):524–546. doi: 10.1055/a-0588-5365. PubMed DOI

Trikudanathan G., Wolbrink D.R.J., van Santvoort H.C., et al. Current concepts in severe acute and necrotizing pancreatitis: an evidence-based approach. Gastroenterology. 2019;156(7):1994–2007.e3. doi: 10.1053/j.gastro.2019.01.269. PubMed DOI

Trikudanathan G., Attam R., Arain M.A., et al. Endoscopic interventions for necrotizing pancreatitis. Am J Gastroenterol. 2014;109(7):969–981. doi: 10.1038/ajg.2014.130. quiz 982. PubMed DOI

Tenner S., Baillie J., DeWitt J., Vege S.S. American College of Gastroenterology guideline: management of acute pancreatitis. Am J Gastroenterol. 2013;108(9):1400–1415. doi: 10.1038/ajg.2013.218. 1416. PubMed DOI

Besselink M., van Santvoort H., Freeman M., et al. Working Group IAP/APA Acute Pancreatitis Guidelines. IAP/APA evidence-based guidelines for the management of acute pancreatitis. Pancreatology. 2013;13(4 Suppl 2):e1–e15. doi: 10.1016/j.pan.2013.07.063. PubMed DOI

Trikudanathan G., Arain M., Attam R., et al. Interventions for necrotizing pancreatitis: an overview of current approaches. Expert Rev Gastroenterol Hepatol. 2013;7(5):463–475. doi: 10.1586/17474124.2013.811055. PubMed DOI

Sabo A., Goussous N., Sardana N., et al. Necrotizing pancreatitis: a review of multidisciplinary management. JOP. 2015;16(2):125–135. doi: 10.6092/1590-8577/2947. PubMed DOI

Portelli M., Jones C.D. Severe acute pancreatitis: pathogenesis, diagnosis and surgical management. Hepatobiliary Pancreat Dis Int. 2017;16(2):155–159. doi: 10.1016/S1499-3872(16)60163-7. PubMed DOI

Crockett S.D., Wani S., Gardner T.B., et al. American Gastroenterological Association Institute guideline on initial management of acute pancreatitis. Gastroenterology. 2018;154(4):1096–1101. doi: 10.1053/j.gastro.2018.01.032. PubMed DOI

Bang J.Y., Wilcox C.M., Navaneethan U., et al. Impact of disconnected pancreatic duct syndrome on the endoscopic management of pancreatic fluid collections. Ann Surg. 2018;267(3):561–568. doi: 10.1097/SLA.0000000000002082. PubMed DOI

Jang J.W., Kim M.H., Oh D., et al. Factors and outcomes associated with pancreatic duct disruption in patients with acute necrotizing pancreatitis. Pancreatology. 2016;16(6):958–965. doi: 10.1016/j.pan.2016.09.009. PubMed DOI

Lawrence C., Howell D.A., Stefan A.M., et al. Disconnected pancreatic tail syndrome: potential for endoscopic therapy and results of long-term follow-up. Gastrointest Endosc. 2008;67(4):673–679. doi: 10.1016/j.gie.2007.07.017. PubMed DOI

Nealon W.H., Bhutani M., Riall T.S., et al. A unifying concept: pancreatic ductal anatomy both predicts and determines the major complications resulting from pancreatitis. J Am Coll Surg. 2009;208:790–799. doi: 10.1016/j.jamcollsurg.2008.12.027. PubMed DOI

Kozarek R.A., Traverso L.W. Pancreatic fistulas: etiology, consequences, and treatment. Gastroenterologist. 1996;4(4):238–244. PubMed

Murage K.P., Ball C.G., Zyromski N.J., et al. Clinical framework to guide operative decision making in disconnected left pancreatic remnant (DLPR) following acute or chronic pancreatitis. Surgery. 2010;148(4):847–856. doi: 10.1016/j.surg.2010.07.039. PubMed DOI

Neoptolemos J.P., London N.J., Carr-Locke D.L. Assessment of main pancreatic duct integrity by endoscopic retrograde pancreatography in patients with acute pancreatitis. Br J Surg. 1993;80(1):94–99. doi: 10.1002/bjs.1800800131. PubMed DOI

Varadarajulu S., Wilcox C.M. Endoscopic placement of permanent indwelling transmural stents in disconnected pancreatic duct syndrome: does benefit outweigh the risks? Gastrointest Endosc. 2011;74(6):1408–1412. doi: 10.1016/j.gie.2011.07.049. PubMed DOI

Uomo G., Molino D., Visconti M., et al. The incidence of main pancreatic duct disruption in severe biliary pancreatitis. Am J Surg. 1998;176(1):49–52. doi: 10.1016/s0002-9610(98)00097-x. PubMed DOI

Dua M.M., Jensen C.W., Friedland S., et al. Isolated pancreatic tail remnants after transgastric necrosectomy can be observed. J Surg Res. 2018;231:109–115. doi: 10.1016/j.jss.2018.05.020. PubMed DOI

Maatman T.K., Roch A.M., Lewellen K.A., et al. Disconnected pancreatic duct syndrome: spectrum of operative management. J Surg Res. 2020;247:297–303. doi: 10.1016/j.jss.2019.09.068. PubMed DOI

Vanek P., Falt P., Urban O. Disconnected pancreatic duct syndrome - a neglected complication of acute pancreatitis. Gastroenterol Hepatol. 2020;74:204–211. doi: 10.14735/amgh2020204. DOI

Vanek P., Trikudanathan G., Freeman M.L. Diagnosing disconnected pancreatic duct syndrome: many disconnects, few answers. Dig Dis Sci. 2021;66(5):1380–1382. doi: 10.1007/s10620-020-06538-2. PubMed DOI

Chen Y., Jiang Y., Qian W., et al. Endoscopic transpapillary drainage in disconnected pancreatic duct syndrome after acute pancreatitis and trauma: long-term outcomes in 31 patients. BMC Gastroenterol. 2019;19(1):54. doi: 10.1186/s12876-019-0977-1. PubMed DOI PMC

Verma S., Rana S.S. Disconnected pancreatic duct syndrome: updated review on clinical implications and management. Pancreatology. 2020;20(6):1035–1044. doi: 10.1016/j.pan.2020.07.402. PubMed DOI

Varadarajulu S., Rana S.S., Bhasin D.K. Endoscopic therapy for pancreatic duct leaks and disruptions. Gastrointest Endosc Clin N Am. 2013;23(4):863–892. doi: 10.1016/j.giec.2013.06.008. PubMed DOI

Rana S.S., Shah J., Sharma R.K., Gupta R. Clinical and morphological consequences of permanent indwelling transmural plastic stents in disconnected pancreatic duct syndrome. Endosc Ultrasound. 2020;9(2):130–137. doi: 10.4103/eus.eus_8_20. PubMed DOI PMC

Tann M., Maglinte D., Howard T.J., et al. Disconnected pancreatic duct syndrome: imaging findings and therapeutic implications in 26 surgically corrected patients. J Comput Assist Tomogr. 2003;27(4):577–582. doi: 10.1097/00004728-200307000-00023. PubMed DOI

Smoczyński M., Jagielski M., Jabłońska A., et al. Transpapillary drainage of walled-off pancreatic necrosis - a single center experience. Wideochir Inne Tech Maloinwazyjne. 2015;10(4):527–533. doi: 10.5114/wiitm.2015.55677. PubMed DOI PMC

Larsen M., Kozarek R.A. Management of disconnected pancreatic duct syndrome. Curr Treat Options Gastroenterol. 2016;14(3):348–359. doi: 10.1007/s11938-016-0098-7. PubMed DOI

Kamal A., Singh V.K., Akshintala V.S., et al. CT and MRI assessment of symptomatic organized pancreatic fluid collections and pancreatic duct disruption: an interreader variability study using the revised Atlanta classification 2012. Abdom Imaging. 2015;40(6):1608–1616. doi: 10.1007/s00261-014-0303-x. PubMed DOI

Fulcher A.S., Turner M.A., Yelon J.A., et al. Magnetic resonance cholangiopancreatography (MRCP) in the assessment of pancreatic duct trauma and its sequelae: preliminary findings. J Trauma. 2000;48(6):1001–1007. doi: 10.1097/00005373-200006000-00002. PubMed DOI

Irani S., Gluck M., Ross A., et al. Resolving external pancreatic fistulas in patients with disconnected pancreatic duct syndrome: using rendezvous techniques to avoid surgery (with video) Gastrointest Endosc. 2012;76(3):586–593.e1–3. doi: 10.1016/j.gie.2012.05.006. PubMed DOI

Rana S.S., Sharma R., Kang M., Gupta R. Natural course of low output external pancreatic fistula in patients with disconnected pancreatic duct syndrome following acute necrotising pancreatitis. Pancreatology. 2020;20(2):177–181. doi: 10.1016/j.pan.2019.12.011. PubMed DOI

Agarwal N., Pitchumoni C.S. Acute pancreatitis: a multisystem disease. Gastroenterologist. 1993;1(2):115–128. PubMed

Timmerhuis H.C., van Dijk S.M., Verdonk R.C., et al. Various modalities accurate in diagnosing a disrupted or disconnected pancreatic duct in acute pancreatitis: a systematic review. Dig Dis Sci. 2020;66(5):1415–1424. doi: 10.1007/s10620-020-06413-0. PubMed DOI PMC

Sandrasegaran K., Tann M., Jennings S.G., et al. Disconnection of the pancreatic duct: an important but overlooked complication of severe acute pancreatitis. Radiographics. 2007;27(5):1389–1400. doi: 10.1148/rg.275065163. PubMed DOI

Fischer T.D., Gutman D.S., Hughes S.J., et al. Disconnected pancreatic duct syndrome: disease classification and management strategies. J Am Coll Surg. 2014;219(4):704–712. doi: 10.1016/j.jamcollsurg.2014.03.055. PubMed DOI

Boxhoorn L., Timmerhuis H.C., Verdonk R.C., et al. Diagnosis and treatment of pancreatic duct disruption or disconnection: an international expert survey and case vignette study. HPB (Oxford) 2021;23(8):1201–1208. doi: 10.1016/j.hpb.2020.11.1148. PubMed DOI

Beger H.G., Rau B., Mayer J., et al. Natural course of acute pancreatitis. World J Surg. Feb 1997;21(2):130–135. doi: 10.1007/s002689900204. PubMed DOI

Nadkarni N.A., Kotwal V., Sarr M.G., Swaroop Vege S. Disconnected pancreatic duct syndrome: endoscopic stent or surgeon’s knife? Pancreas. 2015;44(1):16–22. doi: 10.1097/MPA.0000000000000216. PubMed DOI

Pelaez-Luna M., Vege S.S., Petersen B.T., et al. Disconnected pancreatic duct syndrome in severe acute pancreatitis: clinical and imaging characteristics and outcomes in a cohort of 31 cases. Gastrointest Endosc. 2008;68(1):91–97. doi: 10.1016/j.gie.2007.11.041. PubMed DOI

Woods R.W., Akshintala V.S., Singh V.K., et al. CT severity of post-ERCP pancreatitis: results from a single tertiary medical center. Abdom Imaging. 2014;39(6):1162–1168. doi: 10.1007/s00261-014-0147-4. PubMed DOI

Karjula H., Nordblad Schmidt P., Makela J., et al. Prophylactic pancreatic duct stenting in severe acute necrotizing pancreatitis: a prospective randomized study. Endoscopy. 2019;51(11):1027–1034. doi: 10.1055/a-0865-1960. PubMed DOI

Miller F.H., Keppke A.L., Dalal K., et al. MRI of pancreatitis and its complications: part 1, acute pancreatitis. AJR Am J Roentgenol. 2004;183(6):1637e44 doi: 10.2214/ajr.183.6.01831637. PubMed DOI

Drake L.M., Anis M., Lawrence C. Accuracy of magnetic resonance cholangiopancreatography in identifying pancreatic duct disruption. J Clin Gastroenterol. 2012;46(8):696–699. doi: 10.1097/MCG.0b013e31825003b3. PubMed DOI

Gillams A.R., Kurzawinski T., Lees W.R. Diagnosis of duct disruption and assessment of pancreatic leak with dynamic secretin-stimulated MR cholangiopancreatography. AJR Am J Roentgenol. 2006;186(2):499–506. doi: 10.2214/AJR.04.1775. PubMed DOI

Bang J.Y., Navaneethan U., Hasan M.K., et al. EUS correlates of disconnected pancreatic duct syndrome in walled-off necrosis. Endosc Int Open. 2016;4(8):E883–E889. doi: 10.1055/s-0042-112586. PubMed DOI PMC

Jin S., Shi X.J., Wang S.Y., et al. Drainage fluid and serum amylase levels accurately predict development of postoperative pancreatic fistula. World J Gastroenterol. 2017;23(34):6357–6364. doi: 10.3748/wjg.v23.i34.6357. PubMed DOI PMC

Yokoi Y., Kikuyama M., Kurokami T., Sato T. Early dual drainage combining transpapillary endotherapy and percutaneous catheter drainage in patients with pancreatic fistula associated with severe acute pancreatitis. Pancreatology. 2016;16(4):497–507. doi: 10.1016/j.pan.2016.03.007. PubMed DOI

Bakker O.J., van Baal M.C., van Santvoort H.C., et al. Endoscopic transpapillary stenting or conservative treatment for pancreatic fistulas in necrotizing pancreatitis: multicenter series and literature review. Ann Surg. 2011;253(5):961–967. doi: 10.1097/SLA.0b013e318212e901. PubMed DOI

van Dijk S.M., Timmerhuis H.C., Verdonk R.C., et al. Treatment of disrupted and disconnected pancreatic duct in necrotizing pancreatitis: a systematic review and meta-analysis. Pancreatology. 2019;19(7):905–915. doi: 10.1016/j.pan.2019.08.006. PubMed DOI

Ross A.S., Irani S., Gan S.I., et al. Dual-modality drainage of infected and symptomatic walled-off pancreatic necrosis: long-term clinical outcomes. Gastrointest Endosc. 2014;79(6):929–935. doi: 10.1016/j.gie.2013.10.014. PubMed DOI

Puli S.R., Graumlich J.F., Pamulaparthy S.R., et al. Endoscopic transmural necrosectomy for walled-off pancreatic necrosis: a systematic review and meta-analysis. Can J Gastroenterol Hepatol. 2014;28(1):50–53. doi: 10.1155/2014/539783. PubMed DOI PMC

Arvanitakis M., Delhaye M., Bali M.A., et al. Pancreatic-fluid collections: a randomized controlled trial regarding stent removal after endoscopic transmural drainage. Gastrointest Endosc. 2007;65:609–619. doi: 10.1016/j.gie.2006.06.083. PubMed DOI

Tellez-Avina F.I., Casasola-Sanchez L.E., Ramirez-Luna M.A., et al. Permanent indwelling transmural stents for endoscopic treatment of patients with disconnected pancreatic duct syndrome: long-term results. J Clin Gastroenterol. 2018;52(1):85–90. doi: 10.1097/MCG.0000000000000754. PubMed DOI

Dhir V., Adler D.G., Dalal A., et al. Early removal of biflanged metal stents in the management of pancreatic walled-off necrosis: a prospective study. Endoscopy. 2018;50(6):597–605. doi: 10.1055/s-0043-123575. PubMed DOI

Basha J., Lakhtakia S., Nabi Z., et al. Impact of disconnected pancreatic duct on recurrence of fluid collections and new-onset diabetes: do we finally have an answer? Gut. 2021;70(3):447–449. doi: 10.1136/gutjnl-2020-321773. PubMed DOI PMC

Chavan R., Nabi Z., Lakhtakia S., et al. Impact of transmural plastic stent on recurrence of pancreatic fluid collection after metal stent removal in disconnected pancreatic duct: a randomized controlled trial. Endoscopy. Feb 18, 2022 doi: 10.1055/a-1747-3283. Epub ahead of print. PubMed DOI

Varadarajulu S., Noone T.C., Tutuian R., et al. Predictors of outcome in pancreatic duct disruption managed by endoscopic transpapillary stent placement. Gastrointest Endosc. 2005;61(4):568–575. doi: 10.1016/s0016-5107(04)02832-9. PubMed DOI

François E., Kahaleh M., Giovannini M., Matos C., Devière J. EUS-guided pancreaticogastrostomy. Gastrointest Endosc. 2002;56(1):128–133. doi: 10.1067/mge.2002.125547. PubMed DOI

Hayat U., Freeman M.L., Trikudanathan G., et al. Endoscopic ultrasound-guided pancreatic duct intervention and pancreaticogastrostomy using a novel cross-platform technique with small-caliber devices. Endosc Int Open. 2020;8(2):E196–E202. doi: 10.1055/a-1005-6573. PubMed DOI PMC

Bellin M.D., Freeman M.L., Gelrud A., et al. Total pancreatectomy and islet autotransplantation in chronic pancreatitis: recommendations from PancreasFest. Pancreatology. 2014;14(1):27–35. doi: 10.1016/j.pan.2013.10.009. PubMed DOI PMC

Pearson E.G., Scaife C.L., Mulvihill S.J., et al. Roux-en-Y drainage of a pancreatic fistula for disconnected pancreatic duct syndrome after acute necrotizing pancreatitis. HPB (Oxford) 2012;14(1):26–31. doi: 10.1111/j.1477-2574.2011.00397.x. PubMed DOI PMC

Thiruvengadam N.R., Forde K.A., Miranda J., et al. Disconnected pancreatic duct syndrome: pancreatitis of the disconnected pancreas and its role in the development of diabetes mellitus. Clin Transl Gastroenterol. 2022;13(2) doi: 10.14309/ctg.0000000000000457. PubMed DOI PMC

Gastrovesical fistula as a rare complication following endoscopic transluminal drainage of walled-off necrosis-a case report