What is the diagnosis?
Hint: Note the history of chronic pancreatitis with pancreatic pseudocyst and intermittent melena.
| Your Colleagues Responded: | |||
| Arteriovenous malformation |  7%
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| Esophageal varices | 17%
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| Hemosuccus pancreaticus | Correct Answer | 63%
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| Dieulafoy lesion | 11%
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Discussion
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Figure 1.
Fresh blood oozing from the ampulla of Vater as seen with a side-viewing endoscope. (Note: The previously placed pancreatic duct stent was intact at the onset of bleeding but became dislodged during endoscopic evaluation.) |
Figure 1.
The diagnosis of hemosuccus pancreaticus was made at the time the upper endoscopy was performed following hemodynamic stabilization of the patient. Using a side-viewing endoscope, active bleeding from the ampulla of Vater was visualized (see Figure 1). In the clinical setting of chronic pancreatitis with a large communicating pancreatic pseudocyst following the recent ERCP, this finding established the diagnosis.
Hemosuccus pancreaticus, also known as wirsungorrhagia or pseudohemobilia,[1] is a rare syndrome of bleeding into the pancreatic duct manifested by blood loss through the ampulla of Vater. The first case was described in 1931 by Lower and Ferrell[2] and, in 1969, Vankemmel proposed the term "wirsungorrhagia" (currently used in France).[3] In 1970, Sandblom published 3 cases and coined the term "hemosuccus pancreaticus" to describe the similarity of the disorder to the clinical syndrome of hemobilia.[4]
Overall, hemosuccus pancreaticus is a rare clinical entity with a frequency of only 1 out of 1,500 gastrointestinal (GI) bleeding cases, and less than 100 cases have been reported in the medical literature.[3,5] It most commonly occurs in the setting of chronic pancreatitis with and without pancreatic pseudocysts. It is also seen with acute pancreatitis, neuroendocrine tumors, ectopic pancreas, pancreas divisum, and pancreatolithiasis, as well as being reported as a complication of ERCP and following traumatic abdominal pseudoaneurysm formation.[3,6,7] Hemosuccus pancreaticus usually develops following the rupture of an aneurysm or pseudoaneurysm, which develops in the setting of both pressure necrosis and autodigestion from pancreatic enzymes that lead to progressive vessel wall thinning.[8,9] The splenic artery is most commonly affected (60-65% of cases), followed by the gastroduodenal artery.[9] Pancreaticoduodenal artery involvement occurs in only 10-15% of cases, with hepatic artery and left gastric artery involvement also having been reported.[9] Mortality rates as high as 57% have been reported with pseudocyst-associated rupture of pseudoaneurysms.[9]
Establishing the diagnosis requires clinical suspicion in patients with a past medical history of chronic pancreatitis who present with GI bleeding and severe anemia. This may be manifested primarily as intermittent melena without associated hematemesis, although frank hematochezia may occur.[6,10] More insidious presentations have been described with anemia and vague abdominal discomfort, which may indicate intraperitoneal bleeding and/or bleeding within the pseudocyst. Other exceptional forms of presentation include jaundice, nausea with and without vomiting, and a palpable pulsating mass.[2,3,4]
The differential diagnosis of hemosuccus pancreaticus is broad and includes other causes of acute upper GI bleeding. Depending on the clinical presentation of the individual patient, other considerations include peptic ulcer disease, esophageal varices, arteriovenous malformations, Mallory-Weiss tears, and tumors. Since bleeding may be intermittent with an initial endoscopic evaluation, relatively obscure causes may also be included in the differential, such as Dieulafoy lesion, aortoenteric fistula, and true hemobilia of biliary origin.[8]
Following hemodynamic stabilization of the patient, the initial work-up should be aimed at identifying the source of bleeding. Esophagogastroduodenoscopy (EGD) can rule out other causes of upper GI bleeding and may identify the presence of blood clots in the duodenum in the setting of pseudohemobilia[8]; however, active bleeding from the ampulla of Vater is rarely seen because of the intermittent nature of the bleeding. Use of a side-viewing endoscope may help with the visualization of active bleeding from the ampulla of Vater. CT scanning, CT angiography, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) may provide information regarding the presence of a fistula between a peripancreatic aneurysm or pseudoaneurysm and the pancreatic duct, as well as identify the presence of a "sentinel clot"[11] (focal, high-density clotted blood) in the pancreatic duct during episodes of intermittent bleeding.[12] Doppler studies performed percutaneously or by endoscopic ultrasonography may be useful in identifying the presence of pancreatic pseudocysts as well as any aneurysmal mass. ERCP may demonstrate the presence of clots in the pancreatic duct as well as pancreatic duct dilation and pseudocyst filling, if present. Finally, a pancreatoscopy can be performed using a mother-daughter system endoscope in select centers. Technetium Tc 99m–labeled red blood cell scintigraphy may help identify the location of the bleeding during periods of active bleeding.[12]
Angiography is potentially useful as a part of early diagnostic and therapeutic management strategies, especially in the setting of significant GI bleeding of obscure origin (which is typical in the setting of hemosuccus pancreaticus).[12] Selective angiography of the celiac trunk and the superior mesenteric artery allows for characterization of the anatomic origin of a hemorrhage, as well as identification of any aneurysms or pseudoaneurysms that may be present. It also allows for therapeutic intervention with gel foam or coil embolization of the involved arterial segments.[6,10,12] Additionally, interventional radiologic therapy with the use of a bare metal stent across a splenic artery aneurysm has been described.[10]
While specific management of bleeding primarily involves interventional radiologic therapies, surgical intervention must be considered if less invasive strategies are unsuccessful at controlling bleeding. Surgical management includes arterial ligation of involved vessels, as well as resection of the pancreatic head or tail and pseudocysts. Also, aneurysm resection with possible splenectomy may be indicated in cases of splenic artery aneurysms.[6,10,12] Additionally, both intraoperative ultrasonography and pancreatoscopy have been utilized in identifying the origin of bleeding during surgery.[6]
Selective angiography was performed on the patient in this case. The angiography revealed active arterial extravasation arising from the pancreaticoduodenal arcade. Coil embolization of the gastroduodenal artery was performed successfully. Follow-up images demonstrated excellent results, with significant stagnation of flow in the gastroduodenal artery and segmental branches without any significant arterial blush noted. Repeat angiography performed the following day demonstrated no further bleeding. The bleeding in this case was caused by vessel rupture within the pancreaticoduodenal arcade, most likely associated with the large pancreatic pseudocyst in the setting of chronic pancreatitis. While recent pseudocyst management may have played some role in the development of pseudohemobilia, the extent that each intervention contributed to the patient's presentation is uncertain. The patient remained clinically stable throughout the remainder of his hospital course and was discharged to home with continued outpatient follow-up of his chronic pancreatitis.
Test
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References
- Risti B, Marincek B, Jost R, Decurtins M, Ammann R. Hemosuccus pancreaticus as a source of obscure upper gastrointestinal bleeding: three cases and literature review. AM J Gastroenterol. 1995;90:1878-80.
- Clay RP, Farnell MB, Lancaster JR, Weiland LH, Gostout CJ. Hemosuccus pancreaticus. An unusual cause of upper gastrointestinal bleeding. Ann Surg. 1985;202:75-9.
- Etienne S, Pessaux P, Tuech JJ, et al. Hemosuccus pancreaticus: a rare cause of gastrointestinal bleeding. Gastrointerol Clin Biol. 2005;29:237-42.
- Sandblom P. Gastrointestinal hemorrhage through the pancreatic duct. Ann Surg. 1970;171:61-6.
- Tabrizian P, Newell P, Reiter BP, Heimann TM. Successful multimodality treatment for hemosuccus pancreaticus. Am J Gastroenterol. 2009;104:1060.
- Toyoki Y, Hakamada K, Narumi S, Nara M, Ishido K, Sasaki M. Hemosuccus pancreaticus: problems and pitfalls in diagnosis and treatment. World J Gastroenterol. 2008;14:2776-9.
- Kaman L, Sanyal S, Manekuru SR, Singh R. Pseudoaneurysm of the superior pancreaticoduodenal artery, a rare cause of hemosuccus pancreaticus: report of a case. Surg Today. 2004;34:181-4.
- Sugiki T, Hatori T, Imaizumi T, et al. Two cases of hemosuccus pancreaticus in which hemostasis was achieved by transcatheter arterial embolization. J Hepatobiliary Pancreat Surg. 2003;10:450-4.
- Kapoor S, Rao P, Pal S, Chattopadhyay TK. Hemosuccus pancreaticus: an uncommon cause of gastrointestinal hemorrhage. A case report. JOP. 2004; 5:373-6.
- Benz CA, Jakob P, Jakobs R, Riemann JF. Hemosuccus pancreaticu--a rare cause of gastrointestinal bleeding: diagnosis and interventional radiological therapy. Endoscopy. 2000;32:428-31.
- Orwig D, Federle MP. Localized clotted blood as evidence of visceral trauma on CT: the sentinel clot sign. AJR Am J Roentgenol. 1989;153:747-9.
- Koizumi J, Inoue S, Yonekawa H, Kunieda T. Hemosuccus pancreaticus: diagnosis with CT and MRI and treatment with transcatheter embolization. Abdom Imaging. 2002;27:77-81.
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Author(s)
Juan Carlos Munoz, MD
Clinical Assistant Professor of Medicine, Department of Gastroenterology, University of Florida, Jacksonville, FL
Disclosure: Juan Carlos Munoz, MD, has disclosed no relevant financial information.
William J. Salyers, Jr., MD, MPH
Gastroenterology Fellow, University of Florida College of Medicine, Jacksonville, FL
Disclosure: William J. Salyers, Jr., MD, MPH, has disclosed no relevant financial information.
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Laurie E. Scudder, MS, NP
Accreditation Coordinator, Continuing Professional Education Department, MedscapeCME; Clinical Assistant Professor, School of Nursing and Allied Health, George Washington University, Washington, DC; Nurse Practitioner, School-Based Health Centers, Baltimore City Public Schools, Baltimore, Maryland
Disclosure: Laurie E. Scudder, MS, NP, has disclosed no relevant financial relationships.
Editor(s)
Erik D. Schraga, MD
Director, eMedicine Case of the Week; Clinical Instructor of Emergency Medicine, Stanford/Kaiser Emergency Medicine Residency Program, Department of Emergency Medicine, Kaiser Permanente, Santa Clara Medical Center, Santa Clara, CA
Disclosure: Erik D. Schraga, MD, has disclosed no relevant financial relationships.
D. Brady Pregerson, MD
Dept. of Emergency Medicine, Cedars-Sinai Medical Center, Los Angeles, CA; Dept. of Emergency Medicine, Tri-City Medical Center, Oceanside, CA
Disclosure: D. Brady Pregerson. MD, has disclosed a relevant financial relationship with ERPocketbooks.com.
Tomasz Guzowski, MD, FRCPC
Internal Medicine and Gastroenterology, Stanton Territorial Hospital, Yellowknife, Canada; Assistant Professor of Medicine, University of Alberta
Disclosure: Tom Guzowski, MD, has dislcosed no relevant financial relationships.
Luis M. Soler, BA
Associate Editor, eMedicine/Medscape, New York, NY
Disclosure: Luis M. Soler, BA, has disclosed no relevant financial relationships.
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