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Chronic Pancreatitis with Hemosuccus Pancreaticus. The Diagnostic Contribution of Computed Tomography and Contrast Enhanced Ultrasonography – Case Report
Authors
C. Caraiani1, L. Chiorean2, O. Pascu3, L. Ciobanu3, A. Seicean3, N. Al Hajjar4, T. Zaharie5, R. Badea6
Affiliations
Affiliation addresses are listed at the end of the article.
Schlüsselwörter
Zusammenfassung
Abstract
"
!
!
Haemosuccus pancreaticus ist definiert als eine obere gastrointestinale Blutung aus dem Ductus Wirsungianus durch die Papilla Vateri. Diese seltene aber bedrohliche Blutung ist durch ihre typischerweise intermittierende Symptomatik charakterisiert und nur schwer zu diagnostizieren und therapieren. Eine frühzeitige Diagnose ermöglicht bessere Behandlungsoptionen mit einer Reduktion der Mortalität. In der hier vorgestellten Kasuistik wird die Bedeutung der Bildgebung diskutiert. Die Computertomografie erbrachte den Nachweis von Blut im Pankreasgang und die Kontrastmittelsonografie konnte eine aktive Blutung ausschließen.
Hemosuccus pancreaticus is defined as upper gastrointestinal hemorrhage from the ampulla of Vater via the pancreatic duct. It is a rare disease, with non-specific presentation, challenging to diagnose and difficult to treat, with high mortality rates in untreated patients with massive bleeding. Given the intermittent nature of the bleeding, delays in diagnosis frequently occur. Timely diagnosis and treatment seem to result in markedly reduced mortality, therefore we emphasize the diagnostic contribution of imaging techniques by presenting the case of a patient with chronic pancreatitis in whom computed tomography established the diagnosis of blood in the Wirsung duct and contrast-enhanced ultrasound brought its added value by excluding the active bleeding.
Introduction
tumors, pancreas divisum or iatrogenic or accidental trauma in chronic pancreatitis, chronic exposure of the arterial wall to pancreatic enyzmes and scarring and granulation of the pseudocysts may trigger the gastrointestinal hemorrhage. Trauma can cause mild pancreatitis with perivascular inflammation which leads to pseudoaneurysm formation. Surgery may cause a direct injury to the arterial wall, which weakens the artery leading to pseudoaneurysm formation. Pseudoaneurysms can rupture in pseudocysts or pancreatic parenchyma leading to HP. The bleeding intensity ranges from intermittent occult to massive acute, possibly deadly: HP is a life-threatening condition if presenting with massive bleeding, with more than 90 % mortality in untreated cases [7]. In most cases HP presents with spontaneously resolving acute epigastric pain radiating to the back, followed by intermittent hematochezia, melena or, less frequently, hematemesis within 30 – 40 minutes. The pain can have a crescendodecrescendo course and it is caused by increased
● Haemosuccus pancreaticus ● Computertomografie ● gastrointestinale Blutung ● Kontrastmittelsonografie ● chronische Pankreatitis ● Pankreasgang " " " " "
Key words
● hemosuccus pancreaticus ● computed tomography ● gastrointestinal hemorrhage ● contrast-enhanced " " " "
ultrasound
● chronic pancreatitis ● pancreatic duct " "
received accepted
17.4.2014 11.9.2014
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1385320 Z Gastroenterol 2014; 52: 1263–1267 © Georg Thieme Verlag KG Stuttgart · New York · ISSN 0044-2771 Correspondence Liliana Chiorean Department of Radiology and Computed Tomography, “Octavian Fodor” Institute of Gastroenterology and Hepatology Str. Croitorilor 19 – 21 400 162 Cluj Napoca Romania Tel.: ++40/07/44 78 64 98 Fax: ++40/264/43 98 89 [email protected]
!
Hemosuccus pancreaticus (HP), also known as Wirsungorrhage, hemowirsungia, Santorinirrhage, pseudohematobilia and hemoductal pancreatitis [1] represents bleeding from the ampulla of Vater via the pancreatic duct. The term HP was introduced by Sandblom in 1970 [2], but the first case had been described by Lower and Farrell back in 1931, being presented as splenic artery aneurysm communicating with Wirsung’s duct [3]. It is a rare occurrence, representing the least frequent cause of acute upper gastrointestinal bleeding (1/ 1500) [2], predominantly occurring in men (male to female ratio of 7:1), with a mean age of onset of 50 to 60 years, highly correlating with chronic alcohol ingestion [4]. HP usually occurs as a complication of acute or chronic pancreatitis [5], the most common cause being arterial pseudoaneurysm formation secondary to chronic pancreatitis [1, 6]. Other causes of HP include vascular malformations, pancreatic
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
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Chronische Pankreatitis mit Haemosuccus pancreaticus. Über den diagnostischen Beitrag der Computertomografie und Kontrastmittelsonografie – eine Kasuistik
Kasuistik
intraductal pressure due to blood and clots in the pancreatic duct [1, 8]. Due to its rarity and because of the intermittent symptoms the diagnosis is extremely difficult. The gold standard is angiography which can demonstrate the exact anatomic site of bleeding. When present, the arterial pseudoaneurysms and pseudocysts can be demonstrated on ultrasound and contrast enhanced computed tomography (CT) [9]. If the patient is relatively stable, angiographic embolotherapy is the first choice for treatment. When it fails or when the patient presents uncontrolled hemorrhage and shock then surgical treatment is indicated [4, 10].
Case Report !
Consent Written informed consent was obtained from patient for the publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editorin-Chief of this journal. A 49-year-old man, without significant medical or surgical history, was admitted to our clinic as an emergency for acute abdominal pain, melena and severe anemia. The pain was intense and radiating to the back. On physical examination, the patient was pale, with cold skin, profuse sweating and tachycardia. No signs of chronic hepatopathy or splenomegaly were noted. Laboratory data revealed severe anemia (hemoglobin 6.0 g/dl; reference range: 12 − 16 g/dl), hypocalcemia and hypoproteinemia. Emergency endoscopy was performed, and no abnormal lesions were found in the stomach or duodenum. The papilla of Vater had a normal aspect. On endoscopic ultrasound a pseudoaneur-
ysm of the splenic artery, located at the level of pancreatic body, was suspected. The Wirsung's duct at this level was unevenly dilated, with hypoechoic content. Contrast-enhanced computed tomography (CT) revealed an inhomogeneous structure of the pancreas, showing three cystic lesions: one cephalic, with a diameter of 25 mm and a sponta" Fig. 1a), neously hyperdense content (aspect of fresh blood) (● another one at the level of the pancreatic body, 13 mm in diameter, and the third one at the level of the pancreatic tail, having a 15 mm diameter. The cystic lesions showed intense contrast agent enhancement during the arterial phase, more obvious " Fig. 1b) – the computed toseen in the caudally located lesion (● mographic appearance was in favor of active bleeding at the time the examination performed. Also, a fistula (communication?) appeared to be present between the corporeal lesion and the sple" Fig. 1c). Communications between the cystic lesions nic artery (● and the pancreatic duct were highly suspected because the Wirsung's duct appeared similar to an arterial vessel, showing SAU " Fig. 1b). with intense enhancement during the arterial phase (● The main suspicion was of intraductal papillary mucinous neoplasia of the secondary ducts, the one located at the level of the pancreatic body communicating with the splenic artery, and with active bleeding at the level of the pancreatic duct. The cystic lesions could also represent small, intrapancreatic pseudocysts as stigmata of chronic pancreatitis. We noted that there were no calcifications seen in the pancreatic parenchyma. The splenic vein was permeable, compressed by the caudal cystic lesion. The liver and the spleen had a normal CT aspect. Contrast-enhanced ultrasound (CEUS) was performed with a GE Logiq 7 ultrasound system, using the 1.5 – 5.0 MHz transducer and i. v. contrast agent SonoVue (Bracco), and tissue harmonic imaging in the presence of a low mechanical index (MI). We in-
Fig. 1 Abdominal CT, a native examination, axial section at the level of pancreatic head, showing an inhomogenous structure with spontaneous hyperdense content, having significance of fresh blood; b contrast-enhanced CT, arterial phase, axial section at the level of pancreatic body and tail – cystic lesion (white arrow) and pancreatic duct (red arrow), both with hyperdense content representing active bleeding; c contrast-enhanced CT, arterial phase, coronal reconstruction – a fistula between the corporeal lesion and the splenic artery is highly suspected.
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
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jected 1.6 ml of contrast agent, followed by 10 ml of saline solution. The intention was to demonstrate in real time the active bleeding. Three hypoechoic lesions were seen, the biggest one with cephalic location, all having a cystic nature, with inhomoge" Fig. 2a, b). The lesions showed no enhancement neous content (● " Fig. 2c). There was also a dilataafter contrast agent injection (● tion of the pancreatic duct, but no contrast agent enhancement inside the Wirsung's duct was seen, refuting active bleeding at " Fig. 2 d). the time of examination (● Subsequently, the patient underwent laparotomy, as interventional radiology techniques were not available in our institution. The intraoperative diagnosis was of upper digestive tract hemorrhage due to a pancreatic body tumor, with effraction at the level of the splenic artery and fistula between the Wirsung’s duct and the splenic artery. Corporeo-caudal spleno-pancreatectomy with primary ligation of the splenic artery at its origin was the surgical choice. Macroscopically, the pancreatic body presented an inhomogeneous lesion of 13/8 mm, microscopically corresponding to a dilated and ulcerated main pancreatic duct, containing important hematic infiltrations, fibrin exudation and leukocytes, surrounded by hyalinization of the conjunctive tissue and moderate mixed inflammatory infiltrate. In the pancreatic tail there was an area of 17/13 mm with yellow necrotic content, microscopically corresponding to a dilated ductal system, with focal ulcerations and hemorrhagic areas. Also, there was marked acinar atrophy and massive fibrosis. The conclusion was of chronic pancreatitis, most probably of obstructive cause. The patient’s postoperative course was uneventful.
Discussion !
Diagnosing HP is usually difficult as it has an intermittent character [11]. The imaging diagnosis is made by visualization of bleeding inside the pancreatic duct, either by CT or by angiography [12]. Direct visualization of the fistulous trajectory, by the means
of CT, as seen in our case, is not frequent. The intermittent character of the hemorrhage is the reason why bleeding has been clearly demonstrated by CT scan but not visualized at/by endoscopy or CEUS. The most common cause of HP is a fistula between a splenic artery pseudoaneurysm and the main pancreatic duct. Pseudoaneurysms form when enzyme-rich pancreatic fluid leads to autodigestion and weakening of adjacent artery walls [13]. Pseudocysts or ductal calculi can also lead to HP by eroding the walls of blood vessels. In most cases, rupture of a pseudoaneurysm inside the ductus pancreaticus causes an initially slow, self-limited hemorrhage followed, hours or days later, by massive and life-threatening bleeding. Bleeding is associated with epigastric pain and with the elevation of serum lipase and amylase, thought to be caused by the rapid distension of the pancreatic duct. Blood passing into the duodenum through the ampula of Vater causes hematemesis and melena. The initial self-limited character of the hemorrhage may be due to tamponade of the bleeding vessel by the increased pressure in the pseudocyst [14]. This evolution emphasizes the importance of a correct and quick diagnosis of HP, allowing for the right treatment. If the pseudocyst does not communicate with the ductus pancreaticus, bleeding will cause rapid enlargement of the pseudocyst, pain and a sudden fall in hemoglobin [13]. The initial diagnostic work-up requires upper gastrointestinal endoscopy to rule out other causes of bleeding. Bleeding from the biliary tree or pancreatic duct should be suspected if no other source of bleeding can be identified at digestive endoscopy, particularly if blood is detected in the second part of the duodenum. Bleeding visualized through the ampulla of Vater on duodenoscopy is suggestive, but can require prolonged observation with a side-viewing duodenoscope. CT is a very valuable diagnostic tool, being able to show simultaneous opacification of an aneurysmal artery and pseudocyst or persistence of contrast within a pseudocyst. The characteristic finding of clotted blood in the pancreatic duct, known as the sentinel
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
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Fig. 2 Pancreatic ultrasound examination, transversal sections. a, b Gray scale – three hypoechoic lesions are seen, the biggest one located at the level of pancreatic head, all having inhomogeneous content; c, d contrast-enhanced ultrasound, arterial time – the pancreatic lesions presented no enhancement after contrast-agent injection, nor did the pancreatic duct. The CEUS aspect was refuting active bleeding at the time of examination.
Kasuistik
clot, may seldom be demonstrated on unenhanced sequences [15]. Also, CT can show pancreatic calcifications due to an underlying chronic pancreatitis. Ultrasound also has an important contribution, due to its good availability and non-invasiveness. By visualizing pancreatic pseudocysts or aneurysms of the peripancreatic arteries it can raise the suspicion of a chronic pancreatitis with HP as the cause of the gastrointestinal bleeding. Contrast-enhanced ultrasound may be useful in assessing the presence or absence of active bleeding Ultimately, angiography is the diagnostic reference standard, but there is a clear tendency for it to be replaced by CT angiography. Because direct visualization of the hemorrhage by endoscopy and imaging techniques is not always possible due to the intermittent nature of the bleeding, diagnosis needs correlation of medical history, clinical examination, and biological and imaging signs. Our patient had no medical history of chronic pancreatitis, nor complaints of chronic abdominal pains. He has not been biologically evaluated for chronic pancreatitis since there was no clinical suspicion; a suspicion was raised by the CT and ultrasound appearance, due to the presence of t pancreatic lesions suspicious for pseudocysts. The diagnosis of chronic pancreatitis in our case has been histologically established. HP is a life-threating condition. Several papers report a mortality of 90 % in untreated and 12.5 % in treated patients [14, 16]. Because of potentially catastrophic complications of hemorrhage, a pseudoaneurysm should be treated whether or not it has caused bleeding [14]. Due to both its diagnostic value in determining the exact site of bleeding and therapeutic value in stopping bleeding, radiological procedures are the first choice of treatment. Surgery is reserved as a second line therapy, after the failure of interventional radiology procedures. Patients with hemosuccus pancreaticus are often not eligible for surgery due to associated pathological conditions or therapies (such as anticoagulants). The main radiological technique used in the treatment of haemosuccus pancreaticus is selective transcatheter embolization. The mediums we can choose for/to perform the embolization can be either temporary or permanent obstructive materials such as steel spirals or cyanoacrylate adhesives. An alternative to transcatheter embolization can be stent insertion. The main advantage of stent insertion is that the stent does not cause obstruction of the vessel and does not cause ischemia of the corresponding tissue. Supportive treatment alone is not recommended as the mortality exceeds 90 %. Once the hemodynamic situation of the patient is stable, interventional radiology will be the first therapeutic option with positive outcome in 79 − 100 % of cases [17, 18]. Surgical treatment is indicated for uncontrolled hemorrhage, persistent shock and re-bleeding after initial embolization. Most surgical procedures have a good outcome with a chance of re-bleeding of 0 − 5 % [10].
Conclusion !
HP is a rare pathology and represents a diagnostic and therapeutic challenge. The most common cause of HP is chronic pancreatitis leading to pseudoaneurysm formation. Ultrasonography can be used to detect signs of chronic pancreatitis. CEUS can be a helpful tool in the diagnosis of active bleeding, and, due to its non-invasive nature, allows repeated examinations and can be used for the follow-up. CT may demonstrate extravasation of contrast media in the pseudocyst or in the pancreatic duct, and
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
sometimes may reveal the source of bleeding. Also, it is an excellent modality for demonstrating an underlying pancreatic pathology. Overall, for a correct diagnosis, a high index of suspicion is required in patients with pancreatitis and gastrointestinal bleeding. Interventional radiology is the treatment of choice for these patients, with surgery as second line option. Supportive treatment alone does not represent a therapeutic option.
Abbreviations HP CEUS CT
haemosuccus pancreaticus contrast-enhanced ultrasound computed-tomography
Affiliations 1
2
3
4
5
6
Department of Radiology and Computed Tomography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania Department of Ultrasonography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, Cluj-Napoca, Romania Department of Gastroenterology, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania Department of Surgery, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, ClujNapoca, Romania Department of Pathology, “Octavian Fodor” Institute of Gastroenterology and Hepatology, Cluj-Napoca, Romania Department of Ultrasonography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania
Acknowledgements !
Dr. Cosmin Caraiani is a fellow of POSDRU grant no.159/1.5/S/ 138 776 “Model colaborativ institutional pentru translatarea cercetarii stiintifice biomedicale in practica clinica − TRANSCENT”
References 01 Han B, Song ZF, Sun B. Hemosuccus pancreaticus: a rare cause of gastrointestinal bleeding. Hepatobiliary Pancreat Dis Int 2012; 11: 479 – 488 02 Sandblom P. Gastrointestinal hemorrhage through the pancreatic duct. Ann Surg 1970; 171: 61 – 66 03 Toyoki Y, Hakamada K, Narumi S et al. Hemosuccus pancreaticus: Problems and pitfalls in diagnosis and treatment. World J Gastroenterol 2008; 14: 2776 – 2779 04 Lermite E, Regenet N, Tuech JJ et al. Diagnosis and treatment of hemosuccus pancreaticus: development of endovascular management. Pancreas 2007; 34: 229 – 232 05 Koren M, Kinova S, Bedeova J et al. Hemosuccus pancreaticus. Bratisl Lek Listy 2008; 109: 37 – 41 06 Schreiber-Dietrich D, Ignee A, Braden B et al. Pseudoaneurysmata viszeraler Gefäße als Komplikation der chronischen Pankreatitis, eine seltene Ursache der gastrointestinalen Blutung. Endo heute 2009; 22: 1–5 07 Kothari RA, Leelakrishnan V, Krishnan M. Hemosuccus pancreaticus: a rare cause of gastrointestinal bleeding. Ann Gastroenterol 2013; 26: 175 – 177 08 Péroux JL, Arput JP, Saint-Paul MC et al. Wirsungorrhagia complicating chronic pancreatitis associated with a neuroendocrine tumor of the pancreas. Gastroenterol Clin Biol 1994; 18: 1142 – 1145 09 Burke JW, Erickson SJ, Kellum CD et al. Pseudoaneurysms complicating chronic pancreatitis: detection with CT. Radiology 1986; 161: 447 – 450 10 Sethi H, Peddu P, Prachalias A et al. Selective embolization for bleeding visceral artery pseudoaneurysms in patients with pancreatitis. Hepatobiliary Pancreat Dis Int 2010; 9: 634 – 638
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15 Koizumi J, Inoue S, Yonekawa H et al. Hemosuccus pancreaticus: diagnosis with CT and MRI and treatment with transcatheter embolization. Abdominal Imaging 2002; 27: 77 – 81 16 Grisendi A, Lonardo A, Della CasaG et al. Hemoductal pancreatitis secondary to gastroduodenal artery-ruptured pseudoaneurysm: a rare cause of hematemesis. Am. J. Gastroenterol 1991; 86: 1654 – 1657 17 Woods MS, Traverso LW, Kozarek RA et al. Successful treatment of bleeding pseudoaneurysms of chronic pancreatitis. Pancreas 1995; 10: 22 – 30 18 Tabrizian P, Newell P, Reiter BP et al. Successful multimodality treatment for hemosuccus pancreaticus. Am J Gastroenterol 2009; 104: 1060
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11 De Mas R, Kohler B, Ante D et al. Hämosuccus pancreaticus nach Ruptur eines A. hepatica-aneurysmas. Z Gastroenterol 1989; 27: 736 – 738 12 Sakorafas GH, Sarr MG, Farley DR et al. Hemosuccus pancreaticus complicating chronic pancreatitis: an obscure cause of upper gastrointestinal bleeding. Langenbecks Arch Surg 2000; 385: 124 – 128 13 Elton E, Howell DA, Amberson SM et al. Combined angiographic and endoscopic management of bleeding pancreatic pseudoaneurysms. Gastrointest Endosc 1997; 46: 544 – 549 14 Benz CA, Jakob P, Jakobs R et al. Hemosuccus pancreaticus – a rare cause of gastrointestinal bleeding: Diagnosis and Interventional Radiologic therapy. Endoscopy 2000; 32: 428 – 431
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Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
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Chronic Pancreatitis with Hemosuccus Pancreaticus. The Diagnostic Contribution of Computed Tomography and Contrast Enhanced Ultrasonography – Case Report
Authors
C. Caraiani1, L. Chiorean2, O. Pascu3, L. Ciobanu3, A. Seicean3, N. Al Hajjar4, T. Zaharie5, R. Badea6
Affiliations
Affiliation addresses are listed at the end of the article.
Schlüsselwörter
Zusammenfassung
Abstract
"
!
!
Haemosuccus pancreaticus ist definiert als eine obere gastrointestinale Blutung aus dem Ductus Wirsungianus durch die Papilla Vateri. Diese seltene aber bedrohliche Blutung ist durch ihre typischerweise intermittierende Symptomatik charakterisiert und nur schwer zu diagnostizieren und therapieren. Eine frühzeitige Diagnose ermöglicht bessere Behandlungsoptionen mit einer Reduktion der Mortalität. In der hier vorgestellten Kasuistik wird die Bedeutung der Bildgebung diskutiert. Die Computertomografie erbrachte den Nachweis von Blut im Pankreasgang und die Kontrastmittelsonografie konnte eine aktive Blutung ausschließen.
Hemosuccus pancreaticus is defined as upper gastrointestinal hemorrhage from the ampulla of Vater via the pancreatic duct. It is a rare disease, with non-specific presentation, challenging to diagnose and difficult to treat, with high mortality rates in untreated patients with massive bleeding. Given the intermittent nature of the bleeding, delays in diagnosis frequently occur. Timely diagnosis and treatment seem to result in markedly reduced mortality, therefore we emphasize the diagnostic contribution of imaging techniques by presenting the case of a patient with chronic pancreatitis in whom computed tomography established the diagnosis of blood in the Wirsung duct and contrast-enhanced ultrasound brought its added value by excluding the active bleeding.
Introduction
tumors, pancreas divisum or iatrogenic or accidental trauma in chronic pancreatitis, chronic exposure of the arterial wall to pancreatic enyzmes and scarring and granulation of the pseudocysts may trigger the gastrointestinal hemorrhage. Trauma can cause mild pancreatitis with perivascular inflammation which leads to pseudoaneurysm formation. Surgery may cause a direct injury to the arterial wall, which weakens the artery leading to pseudoaneurysm formation. Pseudoaneurysms can rupture in pseudocysts or pancreatic parenchyma leading to HP. The bleeding intensity ranges from intermittent occult to massive acute, possibly deadly: HP is a life-threatening condition if presenting with massive bleeding, with more than 90 % mortality in untreated cases [7]. In most cases HP presents with spontaneously resolving acute epigastric pain radiating to the back, followed by intermittent hematochezia, melena or, less frequently, hematemesis within 30 – 40 minutes. The pain can have a crescendodecrescendo course and it is caused by increased
● Haemosuccus pancreaticus ● Computertomografie ● gastrointestinale Blutung ● Kontrastmittelsonografie ● chronische Pankreatitis ● Pankreasgang " " " " "
Key words
● hemosuccus pancreaticus ● computed tomography ● gastrointestinal hemorrhage ● contrast-enhanced " " " "
ultrasound
● chronic pancreatitis ● pancreatic duct " "
received accepted
17.4.2014 11.9.2014
Bibliography DOI http://dx.doi.org/ 10.1055/s-0034-1385320 Z Gastroenterol 2014; 52: 1263–1267 © Georg Thieme Verlag KG Stuttgart · New York · ISSN 0044-2771 Correspondence Liliana Chiorean Department of Radiology and Computed Tomography, “Octavian Fodor” Institute of Gastroenterology and Hepatology Str. Croitorilor 19 – 21 400 162 Cluj Napoca Romania Tel.: ++40/07/44 78 64 98 Fax: ++40/264/43 98 89 [email protected]
!
Hemosuccus pancreaticus (HP), also known as Wirsungorrhage, hemowirsungia, Santorinirrhage, pseudohematobilia and hemoductal pancreatitis [1] represents bleeding from the ampulla of Vater via the pancreatic duct. The term HP was introduced by Sandblom in 1970 [2], but the first case had been described by Lower and Farrell back in 1931, being presented as splenic artery aneurysm communicating with Wirsung’s duct [3]. It is a rare occurrence, representing the least frequent cause of acute upper gastrointestinal bleeding (1/ 1500) [2], predominantly occurring in men (male to female ratio of 7:1), with a mean age of onset of 50 to 60 years, highly correlating with chronic alcohol ingestion [4]. HP usually occurs as a complication of acute or chronic pancreatitis [5], the most common cause being arterial pseudoaneurysm formation secondary to chronic pancreatitis [1, 6]. Other causes of HP include vascular malformations, pancreatic
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
Downloaded by: National University of Singapore. Copyrighted material.
Chronische Pankreatitis mit Haemosuccus pancreaticus. Über den diagnostischen Beitrag der Computertomografie und Kontrastmittelsonografie – eine Kasuistik
Kasuistik
intraductal pressure due to blood and clots in the pancreatic duct [1, 8]. Due to its rarity and because of the intermittent symptoms the diagnosis is extremely difficult. The gold standard is angiography which can demonstrate the exact anatomic site of bleeding. When present, the arterial pseudoaneurysms and pseudocysts can be demonstrated on ultrasound and contrast enhanced computed tomography (CT) [9]. If the patient is relatively stable, angiographic embolotherapy is the first choice for treatment. When it fails or when the patient presents uncontrolled hemorrhage and shock then surgical treatment is indicated [4, 10].
Case Report !
Consent Written informed consent was obtained from patient for the publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editorin-Chief of this journal. A 49-year-old man, without significant medical or surgical history, was admitted to our clinic as an emergency for acute abdominal pain, melena and severe anemia. The pain was intense and radiating to the back. On physical examination, the patient was pale, with cold skin, profuse sweating and tachycardia. No signs of chronic hepatopathy or splenomegaly were noted. Laboratory data revealed severe anemia (hemoglobin 6.0 g/dl; reference range: 12 − 16 g/dl), hypocalcemia and hypoproteinemia. Emergency endoscopy was performed, and no abnormal lesions were found in the stomach or duodenum. The papilla of Vater had a normal aspect. On endoscopic ultrasound a pseudoaneur-
ysm of the splenic artery, located at the level of pancreatic body, was suspected. The Wirsung's duct at this level was unevenly dilated, with hypoechoic content. Contrast-enhanced computed tomography (CT) revealed an inhomogeneous structure of the pancreas, showing three cystic lesions: one cephalic, with a diameter of 25 mm and a sponta" Fig. 1a), neously hyperdense content (aspect of fresh blood) (● another one at the level of the pancreatic body, 13 mm in diameter, and the third one at the level of the pancreatic tail, having a 15 mm diameter. The cystic lesions showed intense contrast agent enhancement during the arterial phase, more obvious " Fig. 1b) – the computed toseen in the caudally located lesion (● mographic appearance was in favor of active bleeding at the time the examination performed. Also, a fistula (communication?) appeared to be present between the corporeal lesion and the sple" Fig. 1c). Communications between the cystic lesions nic artery (● and the pancreatic duct were highly suspected because the Wirsung's duct appeared similar to an arterial vessel, showing SAU " Fig. 1b). with intense enhancement during the arterial phase (● The main suspicion was of intraductal papillary mucinous neoplasia of the secondary ducts, the one located at the level of the pancreatic body communicating with the splenic artery, and with active bleeding at the level of the pancreatic duct. The cystic lesions could also represent small, intrapancreatic pseudocysts as stigmata of chronic pancreatitis. We noted that there were no calcifications seen in the pancreatic parenchyma. The splenic vein was permeable, compressed by the caudal cystic lesion. The liver and the spleen had a normal CT aspect. Contrast-enhanced ultrasound (CEUS) was performed with a GE Logiq 7 ultrasound system, using the 1.5 – 5.0 MHz transducer and i. v. contrast agent SonoVue (Bracco), and tissue harmonic imaging in the presence of a low mechanical index (MI). We in-
Fig. 1 Abdominal CT, a native examination, axial section at the level of pancreatic head, showing an inhomogenous structure with spontaneous hyperdense content, having significance of fresh blood; b contrast-enhanced CT, arterial phase, axial section at the level of pancreatic body and tail – cystic lesion (white arrow) and pancreatic duct (red arrow), both with hyperdense content representing active bleeding; c contrast-enhanced CT, arterial phase, coronal reconstruction – a fistula between the corporeal lesion and the splenic artery is highly suspected.
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
Downloaded by: National University of Singapore. Copyrighted material.
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jected 1.6 ml of contrast agent, followed by 10 ml of saline solution. The intention was to demonstrate in real time the active bleeding. Three hypoechoic lesions were seen, the biggest one with cephalic location, all having a cystic nature, with inhomoge" Fig. 2a, b). The lesions showed no enhancement neous content (● " Fig. 2c). There was also a dilataafter contrast agent injection (● tion of the pancreatic duct, but no contrast agent enhancement inside the Wirsung's duct was seen, refuting active bleeding at " Fig. 2 d). the time of examination (● Subsequently, the patient underwent laparotomy, as interventional radiology techniques were not available in our institution. The intraoperative diagnosis was of upper digestive tract hemorrhage due to a pancreatic body tumor, with effraction at the level of the splenic artery and fistula between the Wirsung’s duct and the splenic artery. Corporeo-caudal spleno-pancreatectomy with primary ligation of the splenic artery at its origin was the surgical choice. Macroscopically, the pancreatic body presented an inhomogeneous lesion of 13/8 mm, microscopically corresponding to a dilated and ulcerated main pancreatic duct, containing important hematic infiltrations, fibrin exudation and leukocytes, surrounded by hyalinization of the conjunctive tissue and moderate mixed inflammatory infiltrate. In the pancreatic tail there was an area of 17/13 mm with yellow necrotic content, microscopically corresponding to a dilated ductal system, with focal ulcerations and hemorrhagic areas. Also, there was marked acinar atrophy and massive fibrosis. The conclusion was of chronic pancreatitis, most probably of obstructive cause. The patient’s postoperative course was uneventful.
Discussion !
Diagnosing HP is usually difficult as it has an intermittent character [11]. The imaging diagnosis is made by visualization of bleeding inside the pancreatic duct, either by CT or by angiography [12]. Direct visualization of the fistulous trajectory, by the means
of CT, as seen in our case, is not frequent. The intermittent character of the hemorrhage is the reason why bleeding has been clearly demonstrated by CT scan but not visualized at/by endoscopy or CEUS. The most common cause of HP is a fistula between a splenic artery pseudoaneurysm and the main pancreatic duct. Pseudoaneurysms form when enzyme-rich pancreatic fluid leads to autodigestion and weakening of adjacent artery walls [13]. Pseudocysts or ductal calculi can also lead to HP by eroding the walls of blood vessels. In most cases, rupture of a pseudoaneurysm inside the ductus pancreaticus causes an initially slow, self-limited hemorrhage followed, hours or days later, by massive and life-threatening bleeding. Bleeding is associated with epigastric pain and with the elevation of serum lipase and amylase, thought to be caused by the rapid distension of the pancreatic duct. Blood passing into the duodenum through the ampula of Vater causes hematemesis and melena. The initial self-limited character of the hemorrhage may be due to tamponade of the bleeding vessel by the increased pressure in the pseudocyst [14]. This evolution emphasizes the importance of a correct and quick diagnosis of HP, allowing for the right treatment. If the pseudocyst does not communicate with the ductus pancreaticus, bleeding will cause rapid enlargement of the pseudocyst, pain and a sudden fall in hemoglobin [13]. The initial diagnostic work-up requires upper gastrointestinal endoscopy to rule out other causes of bleeding. Bleeding from the biliary tree or pancreatic duct should be suspected if no other source of bleeding can be identified at digestive endoscopy, particularly if blood is detected in the second part of the duodenum. Bleeding visualized through the ampulla of Vater on duodenoscopy is suggestive, but can require prolonged observation with a side-viewing duodenoscope. CT is a very valuable diagnostic tool, being able to show simultaneous opacification of an aneurysmal artery and pseudocyst or persistence of contrast within a pseudocyst. The characteristic finding of clotted blood in the pancreatic duct, known as the sentinel
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
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Fig. 2 Pancreatic ultrasound examination, transversal sections. a, b Gray scale – three hypoechoic lesions are seen, the biggest one located at the level of pancreatic head, all having inhomogeneous content; c, d contrast-enhanced ultrasound, arterial time – the pancreatic lesions presented no enhancement after contrast-agent injection, nor did the pancreatic duct. The CEUS aspect was refuting active bleeding at the time of examination.
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clot, may seldom be demonstrated on unenhanced sequences [15]. Also, CT can show pancreatic calcifications due to an underlying chronic pancreatitis. Ultrasound also has an important contribution, due to its good availability and non-invasiveness. By visualizing pancreatic pseudocysts or aneurysms of the peripancreatic arteries it can raise the suspicion of a chronic pancreatitis with HP as the cause of the gastrointestinal bleeding. Contrast-enhanced ultrasound may be useful in assessing the presence or absence of active bleeding Ultimately, angiography is the diagnostic reference standard, but there is a clear tendency for it to be replaced by CT angiography. Because direct visualization of the hemorrhage by endoscopy and imaging techniques is not always possible due to the intermittent nature of the bleeding, diagnosis needs correlation of medical history, clinical examination, and biological and imaging signs. Our patient had no medical history of chronic pancreatitis, nor complaints of chronic abdominal pains. He has not been biologically evaluated for chronic pancreatitis since there was no clinical suspicion; a suspicion was raised by the CT and ultrasound appearance, due to the presence of t pancreatic lesions suspicious for pseudocysts. The diagnosis of chronic pancreatitis in our case has been histologically established. HP is a life-threating condition. Several papers report a mortality of 90 % in untreated and 12.5 % in treated patients [14, 16]. Because of potentially catastrophic complications of hemorrhage, a pseudoaneurysm should be treated whether or not it has caused bleeding [14]. Due to both its diagnostic value in determining the exact site of bleeding and therapeutic value in stopping bleeding, radiological procedures are the first choice of treatment. Surgery is reserved as a second line therapy, after the failure of interventional radiology procedures. Patients with hemosuccus pancreaticus are often not eligible for surgery due to associated pathological conditions or therapies (such as anticoagulants). The main radiological technique used in the treatment of haemosuccus pancreaticus is selective transcatheter embolization. The mediums we can choose for/to perform the embolization can be either temporary or permanent obstructive materials such as steel spirals or cyanoacrylate adhesives. An alternative to transcatheter embolization can be stent insertion. The main advantage of stent insertion is that the stent does not cause obstruction of the vessel and does not cause ischemia of the corresponding tissue. Supportive treatment alone is not recommended as the mortality exceeds 90 %. Once the hemodynamic situation of the patient is stable, interventional radiology will be the first therapeutic option with positive outcome in 79 − 100 % of cases [17, 18]. Surgical treatment is indicated for uncontrolled hemorrhage, persistent shock and re-bleeding after initial embolization. Most surgical procedures have a good outcome with a chance of re-bleeding of 0 − 5 % [10].
Conclusion !
HP is a rare pathology and represents a diagnostic and therapeutic challenge. The most common cause of HP is chronic pancreatitis leading to pseudoaneurysm formation. Ultrasonography can be used to detect signs of chronic pancreatitis. CEUS can be a helpful tool in the diagnosis of active bleeding, and, due to its non-invasive nature, allows repeated examinations and can be used for the follow-up. CT may demonstrate extravasation of contrast media in the pseudocyst or in the pancreatic duct, and
Caraiani C et al. Chronic Pancreatitis with … Z Gastroenterol 2014; 52: 1263–1267
sometimes may reveal the source of bleeding. Also, it is an excellent modality for demonstrating an underlying pancreatic pathology. Overall, for a correct diagnosis, a high index of suspicion is required in patients with pancreatitis and gastrointestinal bleeding. Interventional radiology is the treatment of choice for these patients, with surgery as second line option. Supportive treatment alone does not represent a therapeutic option.
Abbreviations HP CEUS CT
haemosuccus pancreaticus contrast-enhanced ultrasound computed-tomography
Affiliations 1
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Department of Radiology and Computed Tomography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania Department of Ultrasonography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, Cluj-Napoca, Romania Department of Gastroenterology, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania Department of Surgery, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, ClujNapoca, Romania Department of Pathology, “Octavian Fodor” Institute of Gastroenterology and Hepatology, Cluj-Napoca, Romania Department of Ultrasonography, “Octavian Fodor” Institute of Gastroenterology and Hepatology, “Iuliu Hatieganu” University of Medicine and Pharmacy, Cluj-Napoca, Romania
Acknowledgements !
Dr. Cosmin Caraiani is a fellow of POSDRU grant no.159/1.5/S/ 138 776 “Model colaborativ institutional pentru translatarea cercetarii stiintifice biomedicale in practica clinica − TRANSCENT”
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