Peer Reviewed

Case in Point

Symptomatic Pancreas Divisum: Recurrent Acute Pancreatitis in a 23-Year-Old Man

Volume 58 - Issue 10 - October 2018

  • Management

    Treatment of symptomatic pancreas divisum is traditionally endotherapy with stenting and/or sphincterotomies. Patients with minimal symptoms may be treated with standard medical therapy (low-fat diet, analgesia, pancreatic enzymes, etc), those with recurrent acute pancreatitis represent the best candidates for endotherapy in pancreas divisum with a predicted sustained response rate of approximately 75%.6 Those with chronic pancreatitis are associated with a 40% to 60% sustained response rate, and those with chronic abdominal pain respond poorly (20%-40%).6

    Evidence is accumulating that pancreatic sphincterotomies are useful in some settings for treating pancreas divisum, with some studies showing a success rate of more than 90% in adolescents.12 The efficacy of surgery compared with endotherapy remains controversial in the treatment of symptomatic pancreas divisum. One review compared the efficacy of endotherapy and surgery for pancreas divisum.22 No statistically significant difference in the response rates was found, and the review concluded that endotherapy is a reasonable first-line therapy for pancreas divisum. Endotherapy has been most likely to be effective at reducing the recurrence of recurrent acute pancreatitis, but less than half of patients with pancreas divisum and chronic pancreatitis or chronic abdominal pain are likely to experience a significant reduction in symptoms from endotherapy.1 However, a systematic review and quantitative analysis showed that symptom improvement with surgery was achieved to a significantly higher degree than endoscopy (72% vs 62.3% respectively), and endoscopy was associated with a higher concentration rate than surgery (31.3% vs 23.8% respectively).5 However, no clinical trial or study currently exists comparing symptom reduction in pancreas divisum via surgery or endoscopic therapy.5 Given the lower rate of beneficial outcomes for endotherapy in chronic pancreatitis or chronic abdominal pain, other treatment options such as surgery or radiologic involvement may produce better outcomes.

    Conclusion

    Pancreas divisum is the most common congenital anatomic anomaly of the pancreas and has the highest prevalence among the white population. Pancreas divisum occurs when the dorsal and ventral pancreatic ducts fail to fuse embryologically in week 8 of development, and the individual is left without any communication between the 2 ducts (complete divisum) or a small branch connecting the 2 ducts (incomplete divisum). Those born with pancreas divisum are usually asymptomatic, while fewer than 10% experience symptomatic pancreas divisum. Symptomatic pancreas divisum has been associated with recurrent acute pancreatitis, chronic pancreatitis, and chronic abdominal pain. Controversy remains about whether pancreas divisum is a causal factor or a predisposing factor to pancreatitis, but recent studies increasingly suggest that pancreas divisum is a predisposing factor to pancreatitis, especially in patients with mutations in CFTR or SPINK1.

    Should a patient present with the signs, symptoms, and laboratory test results suggesting pancreatitis, alcohol, gallstones, and hypercalcemia and hypertriglyceridemia should be excluded prior to investigating for a mechanical obstruction such as pancreas divisum. The first-line imaging modality for suspected pancreas divisum is S-MRCP followed by ERCP for possible stenting or sphincterotomy. The aim of treatment in symptomatic pancreas divisum is to mitigate the progression to chronic pancreatitis. Endotherapy has not been shown to have as strong benefit in chronic pancreatitis, so other surgical or radiologic interventions may be more appropriate. n

    John H. Rosenberg, BS, is a third-year medical student at the Creighton University School of Medicine in Omaha, Nebraska.

    John H. Werner, BS, is a third-year medical student at the Creighton University School of Medicine in Omaha, Nebraska.

    Shailendra K. Saxena, MD, PhD, is a professor in the Department of Family Medicine at the Creighton University School of Medicine in Omaha, Nebraska.

    References:

    1. Kanth R, Samji NS, Inaganti A, et al. Endotherapy in symptomatic pancreas divisum: a systematic review. Pancreatology. 2014;14(4):244-250.
    2. Jeong WJ. Acute pancreatitis after endoscopic biopsy of the minor duodenal papilla in an individual with pancreas divisum. Endoscopy. 2016;48(suppl 1):E238-E239.
    3. Mistry JH, Yadav A, Nundy S. Ampullary carcinoma in a patient with agenesis of the dorsal pancreas: a case report. Indian J Surg. 2015;77(suppl 1):​32-34.
    4. Pierro L, Corbelli E, Gagliardi M, Albertini G, Rabiolo A, Bandello F. Chorioretinal coloboma in a patient with pancreas divisum: clinical and imaging features. Eur J Ophthalmol. 2016;26(6):e158-e160.
    5. Hafezi M, Mayschak B, Probst P, Büchler MW, Hackert T, Mehrabi A. A systematic review and quantitative analysis of different therapies for pancreas divisum. Am J Surg. 2017;214(3):525-537.
    6. Kamisawa T. Clinical significance of the minor duodenal papilla and accessory pancreatic duct. J Gastroenterol. 2004;39(7):605-615.
    7. DiMagno MJ, DiMagno EP. Chronic pancreatitis. Curr Opin Gastroenterol. 2010;26(5):490-498.
    8. Oh B, Lee M. Combined delivery of HMGB-1 box A peptide and S1PLyase siRNA in animal models of acute lung injury. J Control Release. 2014;175:​25-35.
    9. Lu Y, Xu B, Chen L, Bie L-k, Gong B. Endoscopic intervention through endoscopic retrograde cholangiopancreatography in the management of symptomatic pancreas divisum: a long-term follow-up study. Gut Liver. 2016;​10(3):476-482.
    10. Butte JM, Llanos LO. Pancreas divisum. Rev Chilena Cir. 2005;57(2):169-174.
    11. Nishi T, Kawabata Y, Ishikawa N, et al. Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature. BMC Gastroenterol. 2015;15(1):1-7.
    12. Brugge WR. Endoscopic approach to the diagnosis and treatment of pancreatic disease. Curr Opin Gastroenterol. 2013;29(5):559-565.
    13. Rana SS, Gonen C, Vilmann P. Endoscopic ultrasound and pancreas divisum. JOP. 2012;13(3):252-257.
    14. Bertin C, Pelletier A-L, Vullierme MP, et al. Pancreas divisum is not a cause of pancreatitis by itself but acts as a partner of genetic mutations. Am J Gastroenterol. 2012;107(2):311-317.
    15. Izzo P, Di Cello P, Pugliese F, et al. Pancreas divisum: correlation between anatomical abnormalities and bile precipitation in the gallbladder in seven patients. G Chir. 2016;37(4):155-157.
    16. Comfort MW, Gambill EE, Baggenstoss AH. Chronic relapsing pancreatitis; a study of 29 cases without associated disease of the biliary or gastrointestinal tract. Gastroenterology. 1946;6:376-408.
    17. Ammann RW, Akovbiantz A, Largiader F, Schueler G. Course and outcome of chronic pancreatitis: longitudinal study of a mixed medical-surgical series of 245 patients. Gastroenterology. 1984;86(5 pt 1):820-828.
    18. Bakman Y, Freeman ML. Update on biliary and pancreatic sphincterotomy. Curr Opin Gastroenterol. 2012;28(5):420-426.
    19. Gupta A, Rai P, Singh V, Gupta RK, Saraswat VA. Intrahepatic biliary duct branching patterns, cystic duct anomalies, and pancreas divisum in a tertiary referral center: a magnetic resonance cholangiopancreaticographic study. Indian J Gastroenterol. 2016;35(5):379-384.
    20. Brown NG, Howell DA, Brauer BC, Walker J, Wani S, Shah RJ. Minor papilla endotherapy in patients with ventral duct obstruction: identification and management. Gastrointest Endosc. 2017;85(2):365-370.
    21. Rustagi T, Njei B. Magnetic resonance cholangiopancreatography in the diagnosis of pancreas divisum: a systematic review and meta-analysis. Pancreas. 2014;43(6):823-828.
    22. 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.