Skip to main content
Log in

Pancreas Divisum

  • Published:
Current Gastroenterology Reports Aims and scope Submit manuscript

Abstract

We review important new clinical observations in pancreas divisum (PD) made since 2007. PD is common and has the same prevalence in the general population and idiopathic pancreatitis (IP). Up to 53% of patients with PD and IP have underlying idiopathic chronic pancreatitis (CP), and in rigorous prospective clinical follow-up and/or natural history studies, many with idiopathic recurrent acute pancreatitis (IRAP) have idiopathic CP. According to retrospective studies, PD does not modify the natural course of nonalcoholic or alcoholic CP. CFTR and/or SPINK1 gene mutations associate with IP (idiopathic CP and IRAP) independently of the presence of PD. More than one third of patients with pancreatitis or presumed pancreaticobiliary pain respond to placebo. Authors of uncontrolled studies report a significant symptomatic response to surgery and endotherapy in patients with IP and PD, but the response remains unproven and is largely limited to those with IRAP and not idiopathic CP or chronic pain.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Subscribe and save

Springer+ Basic
EUR 32.99 /Month
  • Get 10 units per month
  • Download Article/Chapter or Ebook
  • 1 Unit = 1 Article or 1 Chapter
  • Cancel anytime
Subscribe now

Buy Now

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

Papers of particular interest, published recently, have been highlighted as: • Of importance

  1. • Fogel EL, Toth TG, Lehman GA, et al.: Does endoscopic therapy favorably affect the outcome of patients who have recurrent acute pancreatitis and pancreas divisum? Pancreas (2007) 34(1):21–45. To address an important and long-standing controversy in clinical pancreatology, this two-part discussion focuses on data and arguments for and against endoscopic therapy for patients with recurrent acute pancreatitis and PD. Coauthors Fogel, Toth, and Lehman report “The Case for Endoscopic Therapy” and coauthors DiMagno and DiMagno report “The Cases Against Endoscopic Therapy.” The section by DiMagno and DiMagno also includes multiple new analyses of the world’s literature to report prevalence data of PD in idiopathic pancreatitis based on whether data were derived from autopsy, endoscopic, or MRCP literature.

    Article  PubMed  Google Scholar 

  2. Layer P, Yamamoto H, Kalthoff L, et al.: The different courses of early- and late-onset idiopathic and alcoholic chronic pancreatitis. Gastroenterology (1994) 107(5):1481–1487.

    CAS  PubMed  Google Scholar 

  3. DiMagno MJ, Wamsteker EJ, Lee A: Chronic Pancreatitis. BMA House, London (2009). https://online.epocrates.com/u/291167/Chronic+pancreatitis

  4. Cotton PB: Congenital anomaly of pancreas divisum as cause of obstructive pain and pancreatitis. Gut (1980) 21(2):105–114.

    Article  CAS  PubMed  Google Scholar 

  5. Sahel J, Cros RC, Bourry J, Sarles H: Clinico-pathological conditions associated with pancreas divisum. Digestion (1982) 23(1):1–8.

    Article  CAS  PubMed  Google Scholar 

  6. Richter JM, Schapiro RH, Mulley AG, Warshaw AL: Association of pancreas divisum and pancreatitis, and its treatment by sphincteroplasty of the accessory ampulla. Gastroenterology (1981) 81(6):1104–1110.

    CAS  PubMed  Google Scholar 

  7. Burtin P, Person B, Charneau J, Boyer J: Pancreas divisum and pancreatitis: a coincidental association? Endoscopy (1991) 23(2):55–58.

    Article  CAS  PubMed  Google Scholar 

  8. Delhaye M, Engelholm L, Cremer M: Pancreas divisum: congenital anatomic variant or anomaly? Contribution of endoscopic retrograde dorsal pancreatography. Gastroenterology (1985) 89(5):951–958.

    CAS  PubMed  Google Scholar 

  9. Sugawa C, Walt AJ, Nunez DC, Masuyama H: Pancreas divisum: is it a normal anatomic variant? Am J Surg (1987) 153(1):62–67.

    Article  CAS  PubMed  Google Scholar 

  10. Bernard JP, Sahel J, Giovannini M, Sarles H: Pancreas divisum is a probable cause of acute pancreatitis: a report of 137 cases. Pancreas (1990) 5(3):248–254.

    Article  CAS  PubMed  Google Scholar 

  11. • Liao Z, Gao R, Wang W, et al.: A systematic review on endoscopic detection rate, endotherapy, and surgery for pancreas divisum. Endoscopy (2009) 41(5):439–444. A systematic review of ERCP detection rates for PD reported a 2.9% prevalence of PD out of 31,413 ERCPs from 17 studies. PD prevalence varied by different geographic regions, but these apparent regional differences are likely attributable to the degree that endoscopists search for PD (by examining both ductal systems), to referral bias, or to other factors.

    Article  CAS  PubMed  Google Scholar 

  12. Ammann RW: Alcoholic chronic pancreatitis: its relation to alcoholic acute pancreatitis. Gastroenterol Clin Biol (1996) 20(3):312–314.

    CAS  PubMed  Google Scholar 

  13. Comfort MW, Gambill EE, Baggenstoss AH: Chronic relapsing pancreatitis: a study of 29 cases without associated disease of the biliary or gastro-intestinal tract (continued). Gastroenterology (1946) 6(376–408.

    CAS  PubMed  Google Scholar 

  14. Barthet M, Valantin V, Spinosa S, et al.: Clinical course and morphological features of chronic calcifying pancreatitis associated with pancreas divisum. Eur J Gastroenterol Hepatol (1995) 7(10):993–998.

    Article  CAS  PubMed  Google Scholar 

  15. • Spicak J, Poulova P, Plucnarova J, et al.: Pancreas divisum does not modify the natural course of chronic pancreatitis. J Gastroenterol (2007) 42(2):135–139. In a population with predominantly alcoholic chronic pancreatitis, groups with and without PD showed no significant differences among gender, age at onset of disease, clinical presentation, course of disease, and frequency of complications.

    Article  PubMed  Google Scholar 

  16. Draganov P, Forsmark CE: “Idiopathic” pancreatitis. Gastroenterology (2005) 128(3):756–763.

    Article  PubMed  Google Scholar 

  17. Vila JJ, Vicuna M, Irisarri R, et al.: Diagnostic yield and reliability of endoscopic ultrasonography in patients with idiopathic acute pancreatitis. Scand J Gastroenterol (2010) 45(3):375–381.

    Article  CAS  PubMed  Google Scholar 

  18. Yadav D, Lowenfels AB: Trends in the epidemiology of the first attack of acute pancreatitis: a systematic review. Pancreas (2006) 33(4):323–330.

    Article  PubMed  Google Scholar 

  19. Garg PK, Tandon RK, Madan K: Is biliary microlithiasis a significant cause of idiopathic recurrent acute pancreatitis? A long-term follow-up study. Clin Gastroenterol Hepatol (2007) 5(1):75–79.

    Article  PubMed  Google Scholar 

  20. Jacob L, Geenen JE, Catalano MF, Geenen DJ: Prevention of pancreatitis in patients with idiopathic recurrent pancreatitis: a prospective nonblinded randomized study using endoscopic stents. Endoscopy (2001) 33(7):559–562.

    Article  CAS  PubMed  Google Scholar 

  21. Yusoff IF, Raymond G, Sahai AV: A prospective comparison of the yield of EUS in primary vs. recurrent idiopathic acute pancreatitis. Gastrointest Endosc (2004) 60(5):673–678.

    Article  PubMed  Google Scholar 

  22. Whitcomb DC, Gorry MC, Preston RA, et al.: Hereditary pancreatitis is caused by a mutation in the cationic trypsinogen gene. Nat Genet (1996) 14(2):141–145.

    Article  CAS  PubMed  Google Scholar 

  23. Simon P, Weiss FU, Zimmer KP, et al.: Spontaneous and sporadic trypsinogen mutations in idiopathic pancreatitis. JAMA (2002) 288(17):2122.

    Article  PubMed  Google Scholar 

  24. Cohn JA, Friedman KJ, Noone PG, et al.: Relation between mutations of the cystic fibrosis gene and idiopathic pancreatitis. N Engl J Med (1998) 339(10):653–658.

    Article  CAS  PubMed  Google Scholar 

  25. Sharer N, Schwarz M, Malone G, et al.: Mutations of the cystic fibrosis gene in patients with chronic pancreatitis. N Engl J Med (1998) 339(10):645–652.

    Article  CAS  PubMed  Google Scholar 

  26. Witt H, Luck W, Hennies HC, et al.: Mutations in the gene encoding the serine protease inhibitor, Kazal type 1 are associated with chronic pancreatitis. Nat Genet (2000) 25(2):213–216.

    Article  CAS  PubMed  Google Scholar 

  27. Rossi L, Pfutzer RH, Parvin S, et al.: SPINK1/PSTI mutations are associated with tropical pancreatitis in Bangladesh. A preliminary report. Pancreatology (2001) 1(3):242–245.

    Article  CAS  PubMed  Google Scholar 

  28. Noone PG, Zhou Z, Silverman LM, et al.: Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. Gastroenterology (2001) 121(6):1310–1319.

    Article  CAS  PubMed  Google Scholar 

  29. DiMagno EP: Gene mutations and idiopathic chronic pancreatitis: clinical implications and testing. Gastroenterology (2001) 121(6):1508–1512.

    Article  CAS  PubMed  Google Scholar 

  30. Bishop MD, Freedman SD, Zielenski J, et al.: The cystic fibrosis transmembrane conductance regulator gene and ion channel function in patients with idiopathic pancreatitis. Hum Genet (2005) 1–10.

  31. Choudari CP, Imperiale TF, Sherman S, et al.: Risk of pancreatitis with mutation of the cystic fibrosis gene. Am J Gastroenterol (2004) 99(7):1358–1363.

    Article  CAS  PubMed  Google Scholar 

  32. Aoun E, Muddana V, Papachristou GI, Whitcomb DC: SPINK1 N34S is strongly associated with recurrent acute pancreatitis but is not a risk factor for the first or sentinel acute pancreatitis event. Am J Gastroenterol (2010) 105(2):446–451.

    Article  CAS  PubMed  Google Scholar 

  33. Steinberg WM, Chari ST, Forsmark CE, et al.: Controversies in clinical pancreatology: management of acute idiopathic recurrent pancreatitis. Pancreas (2003) 27(2):103–117.

    Article  PubMed  Google Scholar 

  34. Pezzilli R, Morselli-Labate AM, Mantovani V, et al.: Mutations of the CFTR gene in pancreatic disease. Pancreas (2003) 27(4):332–336.

    Article  CAS  PubMed  Google Scholar 

  35. Kopelman H, Durie P, Gaskin K, et al.: Pancreatic fluid secretion and protein hyperconcentration in cystic fibrosis. New England Journal of Medicine (1985) 312(6):329–334.

    Article  CAS  PubMed  Google Scholar 

  36. DiMagno MJ, Lee SH, Hao Y, et al.: A proinflammatory, antiapoptotic phenotype underlies the susceptibility to acute pancreatitis in cystic fibrosis transmembrane regulator (-/-) mice. Gastroenterology (2005) 129(2):665–681.

    PubMed  Google Scholar 

  37. DiMagno MJ, Lee SH, Owyang C, Zhou SY: Inhibition of acinar apoptosis occurs during acute pancreatitis in the human homologue DeltaF508 cystic fibrosis mouse. Am J Physiol Gastrointest Liver Physiol (2010) 299(2):G400–412.

    Article  CAS  PubMed  Google Scholar 

  38. Rosendahl J, Witt H, Szmola R, et al: Chymotrypsin C (CTRC) variants that diminish activity or secretion are associated with chronic pancreatitis. Nat Genet (2008) 40(1):78–82.

    Article  CAS  PubMed  Google Scholar 

  39. Felderbauer P, Hoffmann P, Einwachter H, et al.: A novel mutation of the calcium sensing receptor gene is associated with chronic pancreatitis in a family with heterozygous SPINK1 mutations. BMC Gastroenterol (2003) 3(1):34.

    Article  PubMed  Google Scholar 

  40. Muddana V, Lamb J, Greer JB, et al.: Association between calcium sensing receptor gene polymorphisms and chronic pancreatitis in a US population: role of serine protease inhibitor Kazal 1type and alcohol. World J Gastroenterol (2008) 14(28):4486–4491.

    Article  CAS  PubMed  Google Scholar 

  41. Groman JD, Meyer ME, Wilmott RW, et al.: Variant cystic fibrosis phenotypes in the absence of CFTR mutations. N Engl J Med (2002) 347(6):401–407.

    Article  CAS  PubMed  Google Scholar 

  42. Knowles MR, Durie PR: What is cystic fibrosis? N Engl J Med (2002) 347(6):439–442.

    Article  PubMed  Google Scholar 

  43. Ko SB, Mizuno N, Yatabe Y, et al.: Corticosteroids correct aberrant CFTR localization in the duct and regenerate acinar cells in autoimmune pancreatitis. Gastroenterology (2010) 138(5):1988–1996.

    Article  CAS  PubMed  Google Scholar 

  44. Gelrud A, Sheth S, Banerjee S, et al: Analysis of cystic fibrosis gener product (CFTR) function in patients with pancreas divisum and recurrent acute pancreatitis. Am J Gastroenterol (2004) 99(8):1557–1562.

    Article  PubMed  Google Scholar 

  45. Garg PK, Khajuria R, Kabra M, et al.: Association of SPINK1 gene mutation and CFTR gene polymorphisms in patients with pancreas divisum presenting with idiopathic pancreatitis. J Clin Gastroenterol, 2009. 43(9): p. 848–52.

    Article  CAS  PubMed  Google Scholar 

  46. Wilcox CM: Endoscopic therapy for pain in chronic pancreatitis: Is it time for the naysayers to throw in the towel? Gastrointest Endosc (2005) 61(4):582–586.

    Article  PubMed  Google Scholar 

  47. Toskes PP, Forsmark CE, DeMeo MT, et al: An open-label trial of octreotide for the pain of chronic pancreatitis. Gastroenterology (1994) 106(A326.

    Google Scholar 

  48. Shiratori K, Takeuchi T, Satake K, Matsuno S: Clinical evaluation of oral administration of a cholecystokinin-A receptor antagonist (loxiglumide) to patients with acute, painful attacks of chronic pancreatitis: a multicenter dose-response study in Japan. Pancreas (2002) 25(1):e1–5.

    Article  PubMed  Google Scholar 

  49. Bhardwaj P, Garg PK, Maulik SK, et al.: A randomized controlled trial of antioxidant supplementation for pain relief in patients with chronic pancreatitis. Gastroenterology (2009) 136(1):149–159 e142.

    Google Scholar 

  50. Cahen DL, Gouma DJ, Nio Y, et al.: Endoscopic versus surgical drainage of the pancreatic duct in chronic pancreatitis. N Engl J Med (2007) 356(7):676–684.

    Article  CAS  PubMed  Google Scholar 

  51. Cooperman AM, Siegel J, Hammerman H: Pancreas divisum—advocates and agnostics. J Clin Gastroenterol (1989) 11(5):489–491.

    Article  CAS  PubMed  Google Scholar 

  52. • Chacko LN, Chen YK, Shah RJ: Clinical outcomes and nonendoscopic interventions after minor papilla endotherapy in patients with symptomatic pancreas divisum. Gastrointest Endosc (2008) 68(4):667–673. The authors report that minor papilla endotherapy of patients with PD results in a clinical response or improvement in 76% with IRAP, but only 42% of those with chronic pancreatitis and 33% with chronic abdominal pain. The authors conclude that endoscopic therapy is a reasonable first step in the absence of chronic pain, but also acknowledge that short-term follow-up may be inadequate to assess response in some patients with IRAP who have long pain-free intervals and/or develop chronic pain.

    Article  PubMed  Google Scholar 

  53. Ahmed F, Sherman S: Minor papilla endotherapy in patients with symptomatic pancreas divisum. Gastrointest Endosc (2008) 68(4):674–675.

    Article  PubMed  Google Scholar 

  54. Delhaye M, Matos C, Arvanitakis M, Deviere J: Pancreatic ductal system obstruction and acute recurrent pancreatitis. World J Gastroenterol (2008) 14(7):1027–1033.

    Article  CAS  PubMed  Google Scholar 

  55. Lowes JR, Lees WR, Cotton PB: Pancreatic duct dilatation after secretin stimulation in patients with pancreas divisum. Pancreas (1989) 4(3):371–374.

    Article  CAS  PubMed  Google Scholar 

  56. Ng WK, Tarabain O: Pancreas divisum: a cause of idiopathic acute pancreatitis. CMAJ (2009) 180(9):949–951.

    PubMed  Google Scholar 

Download references

Disclosure

Conflicts of interest: M. DiMagno is supported by National Institutes of Health grants K08 DK073298 and R21 AA017271; E-J. Wamsteker—none.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Matthew J. DiMagno.

Rights and permissions

Reprints and permissions

About this article

Cite this article

DiMagno, M.J., Wamsteker, EJ. Pancreas Divisum. Curr Gastroenterol Rep 13, 150–156 (2011). https://doi.org/10.1007/s11894-010-0170-8

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s11894-010-0170-8

Keywords

Navigation