AUTHOR AND EDITOR INFORMATION

Section 1 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

INTRODUCTION

Section 2 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

Background

Pancreas divisum is the most common congenital anomaly of the pancreas. It occurs in approximately 7% of autopsy series (range, 1-14%). The frequency of finding this condition varies greatly among endoscopic retrograde cholangiopancreatography (ERCP) series (4-25%), depending on the population studied and the degree to which complete pancreatography is pursued.

Pathophysiology

Pancreas divisum develops prenatally. It is caused by failure of the ducts of the dorsal and ventral buds to fuse during embryological development, at approximately the eighth intrauterine week of life. In more than 90% of individuals, the proximal one third of the dorsal pancreatic duct regresses as it fuses with the ventral duct, forming the main pancreatic duct.

In the remainder, one of the following occurs. In cases of classic pancreas divisum, the small ventral duct, or duct of Wirsung, drains via the major papilla and the large dorsal duct, or duct of Santorini, drains via minor papilla. Cases of incomplete pancreas divisum are the same as classic divisum, except a small branch connects the ventral and dorsal pancreas. In cases of pancreas divisum with nonpatent major papilla, the entire pancreatic ductal system drains via the minor papilla. In cases of reversed pancreas divisum, the small dorsal duct drains via minor papilla and the large ventral duct drains via major ampulla. This variant has no physiologic significance except in rare cases of pancreatic cancer that do not involve the main pancreatic duct.

Symptoms of pancreatic divisum probably are due to high intrapancreatic dorsal ductal pressure caused by a small minor papilla orifice.

Frequency

United States

It occurs in approximately 7% of autopsy series (range, 1-14%). The frequency of finding this condition varies greatly among ERCP series (4-25%), depending on the population studied and the degree to which complete pancreatography is pursued.

CLINICAL

Section 3 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

History

• Pancreas divisum usually is a coincidental finding, and most individuals with this anomaly are asymptomatic.
• It can be of clinical relevance in the following situations:
• • At ERCP, the small ventral duct must be differentiated from various forms of main pancreatic duct cutoff, such as pancreatic cancer and pancreatic pseudocysts.
  • Dorsal pancreatic duct diseases can be missed because only the ventral pancreas portion of the pancreas can be viewed via standard major papilla cannulation.
  • Patients with symptomatic pancreas divisum present with recurrent pancreatitis and may eventually develop chronic pancreatitis. Symptoms can be mild with only occasional epigastric pain occurring postprandially; but, more often, the symptoms are severe with clinically significant disability.
• Few patients present with recurrent pancreatic pain without any objective evidence of pancreatitis, such as amylase elevation.

Physical

• The findings from an abdominal examination usually are normal.
• Epigastric tenderness and a palpable pseudocyst might be present during episodes of pancreatitis.

DIFFERENTIALS

Section 4 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

Other Problems to be Considered

Chronic abdominal pain
Sphincter of Oddi dysfunction

WORKUP

Section 5 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

Imaging Studies

• CT scan and ultrasound of the abdomen are not sensitive enough to aid in diagnosing pancreas divisum. However, they can detect dorsal duct dilatation and other changes of chronic pancreatitis.
• Magnetic resonance pancreatography is being used with increasing frequency as a noninvasive alternative to diagnostic ERCP in the evaluation of the pancreatic duct and various pathological conditions of the pancreas, including pancreas divisum.

Procedures

• Pancreas divisum is diagnosed by the aid of pancreatography.
• • Upon ERCP, the major papilla is often difficult to cannulate, and the duct of Wirsung (ventral duct, see Media file 1) appears shortened and of small diameter (like a cutoff of the duct) with rapid filling of small accessory ducts.
  • This should not be confused with a mass lesion such as a malignancy or pancreatic pseudocyst.
  • At this point, the accessory papilla should be cannulated. A variety of maneuvers have been used to facilitate cannulation. These include simple maneuvers, such as using the long scope position and accessories to include a taper cannula, slick wires, secretagogues, such as Kinevac or secretin, and the application of vital dyes.
  • This should reveal the duct of Santorini (dorsal duct, see Media file 2) running the entire length of the pancreas.
  • In symptomatic patients with recurrent episodes of pancreatitis, the ERCP could reveal changes in the pancreatic duct characteristic of chronic pancreatitis.
• Endoscopic ultrasound may be helpful in the identification of pancreas divisum by revealing the absence of a "stalk sign," where the bile duct and pancreatic duct can be seen to run parallel through the pancreatic head.
• Manometry of the minor papilla is performed infrequently. Symptomatic patients with high basal sphincter pressures might benefit from endoscopic or surgical interventions. However, more studies are needed in this area.

TREATMENT

Section 6 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

Medical Care

• Because this anomaly usually is asymptomatic, treatment is offered to symptomatic patients after conducting a complete workup for other causes of pancreatitis and abdominal pain.
• Patients with mild symptoms can be managed conservatively. On the other hand, patients with recurrent episodes of pancreatitis or chronic pain may need intervention, which can be performed endoscopically or surgically, to alleviate papillary stenosis.
• Endoscopic minor papilla sphincterotomy and stenting are discussed as follows:
• • Data regarding minor papilla endoscopic therapy are limited, and randomized trials have not been performed.
  • Sphincterotomy can be performed using a papillotome to make a 4- to 6-mm incision in the 10- to 12-o'clock position or over a stent used as a guide.
  • In a small prospective series, patients with recurrent pancreatitis benefited most from endoscopic therapy, with a 75% improvement rate.
  • Patients with chronic pain syndrome and established chronic pancreatitis benefited least from this intervention.
  • The potential adverse effects of prolonged pancreatic stenting, such as pancreatitis, stent occlusion or migration, pancreatic duct perforation, and pseudocyst formation, leave this method largely experimental and not generally recommended.
  • Endoscopic trial therapy in the appropriate patient probably is the most helpful. Response to such therapy implies that the minor papilla was too narrow and probably responsible for the patient's symptoms.

Surgical Care

• All patients selected for surgical treatment should have confirmation of pancreas divisum by ERCP.
• The choice of operation depends on the clinical picture and extent of the disease.
• Surgical minor papilla sphincterotomy and sphincteroplasty are discussed as follows:
• • Surgical sphincterotomy series and sphincteroplasty series seem to have similar outcomes.
  • When patient categorization is detailed, meaning recurrent, acute pancreatitis, chronic pain alone, or chronic pancreatitis is present, patients with recurrent attacks of pancreatitis usually respond better to surgical therapy, mirroring the endoscopic therapy series results.
  • Patients with established chronic pancreatitis are better candidates for a Puestow procedure or pancreatic resection.

MULTIMEDIA

Section 7 of 8  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

Media file 1:  Cholangiopancreatogram showing small ventral duct (duct of Wirsung) and normal biliary tree upon cannulation of the major papilla
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Media type:  X-RAY

Media file 2:  Pancreatogram showing the dominant dorsal duct (duct of Santorini) upon cannulation of the minor papilla
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Media type:  X-RAY

REFERENCES

Section 8 of 8

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Multimedia
• References

• Bhutani MS, Hoffman BJ, Hawes RH. Diagnosis of pancreas divisum by endoscopic ultrasonography. Endoscopy. Feb 1999;31(2):167-9. [Medline].
• Fulcher AS, Turner MA. MR pancreatography: a useful tool for evaluating pancreatic disorders. Radiographics. Jan-Feb 1999;19(1):5-24; discussion 41-4; quiz 148-9. [Medline].
• Lehman GA, Sherman S. Diagnosis and therapy of pancreas divisum. Gastrointest Endosc Clin N Am. Jan 1998;8(1):55-77. [Medline].
• Mergener K, Kozarek RA. Therapeutic pancreatic endoscopy. Endoscopy. Mar 2005;37(3):201-7.
• Mulholland MW, Moosa AR, Liddle RA. Pancreas: anatomy and structural anomalies. In: Yamada T, ed. Textbook of Gastroenterology. Philadelphia, Pa: JB Lippincott; 1995.
• Neuhaus H. Therapeutic pancreatic endoscopy. Endoscopy. Jan 2004;36(1):8-16. [Medline].
• Somogyi L, Forsmark CE. Pancreatic duct stenting in benign pancreatic disease. Semin Gastrointest Dis. Apr 1998;9(2):73-9. [Medline].

Article Last Updated: Feb 21, 2007