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Gastric cirsoid aneurysm: Uncommon cause of death from upper GI bleed
Human Pathology: Case Reports 10 (2017) 89–91
Contents lists available at ScienceDirect
Human Pathology: Case Reports journal homepage: http://www.humanpathologycasereports.com
Case Report
Gastric cirsoid aneurysm: Uncommon cause of death from upper GI bleed Tatiana Bihun, BA a,⁎, James Ribe, JD, MD b a b
Saba University School of Medicine, C/O R3 Education Inc., 27 Jackson Road, Suite 301, Devens, MA 01434, United States Los Angeles County Department of Medical Examiner –Coroner, 1104 N Mission Road, Los Angeles, CA 90033, United States
a r t i c l e
i n f o
Article history: Received 19 April 2017 Received in revised form 2 July 2017 Accepted 14 July 2017 Available online xxxx Keywords: Gastric cirsoid aneurysm Dieulafoy lesion Arterial malformation Gastrointestinal bleed
a b s t r a c t Gastric cirsoid aneurysm is an arterial malformation found in the submucosa of the stomach. It is a rare, but potentially life-threatening cause of gastrointestinal bleed. We present a case of a 48 year old male who presented to the ER unconscious, unresponsive, pale, and tachycardic. Patient expired and an autopsy was performed. Upon examination blood was found in the GI tract. During examination an arterial malformation was found in the body of the stomach. Histological samples were taken and the findings were consistent with gastric cirsoid aneurysm. Diagnosis can be made through endoscopy, angiography, or red cell scanning. Current treatment is hemostasis achieved by either thermal, regional injection or mechanical therapies. Multiple therapies are found to be more successful than monotherapy. Gastric cirsoid aneurysms are thought to make up b 5% of upper GI bleeds, however clinicians should be mindful when working up a differential diagnosis. © 2017 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Dr. Paul Dieulafoy (1839–1911) was a French physician who first described patients with hematemesis due to a bleeding gastric vessel with no evidence of ulceration. Dieulafoy lesion was used to describe this finding. Moving away from this type of nomenclature, Dieulafoy lesions have been reclassified as gastric cirsoid aneurysms. No definitive cause is known, but it is characterized as an abnormally large tortuous submucosal artery that protrudes through a 2–5 mm opening, that may rupture spontaneously. Cirsoid Aneurysms can be found anywhere along the GI tract, but 70% are found in the stomach. They can be found in all age groups and both genders, but are twice as common in men [1]. Patients will most commonly present with melena, hematemesis, hematochezia, anemia, weakness, fatigue, dizziness, and dyspnea on exertion [1,2,3]. Hemorrhage may also be intermittent if a thrombus is formed and later becomes dislodged [8]. Cirsoid aneurysms are often difficult to recognize due to vague symptomatology and a negative history of NSAIDs, peptic ulcers, or alcohol abuse [4,8].
2. Case report A 48 year old male presented to the ER unconscious and unresponsive from a construction site. Clinical exam exhibited no central pulse, pallor, tachycardia of 153 bpm, no ecchymosis, abrasions or lacerations ⁎ Corresponding author at: 892 Stanley Street, West Islip, NY 11795, United States. E-mail addresses: [email protected], [email protected] (T. Bihun).
to the chest or abdomen, deep laceration to left leg and blood pressure of 142/62 mm Hg. FAST ultrasound was performed and was negative. A left sided thoracotomy was performed, no pericardial effusions or blood was seen in the left thorax. A right sided chest tube was placed but no blood drained. Patient was given 5 units of packed red blood cells. Despite life saving measures patient expired shortly after arrival. The case was reported to the coroner's office for autopsy. Upon examination there was no free blood in the cavity, but blood was found within the GI tract as well as the stomach (Fig. 1). Over 350 cm3 of blood were collected from the stomach after it was removed (Fig. 2). Upon dissection, an arterial malformation was seen in the body of the stomach (Fig. 3). Samples of the arterial malformation were taken for histology. A tortuous artery could be seen imbedded in the submucosa (Fig. 4). Diagnosis of gastric cirsoid aneurysm was made.
3. Discussion Gastric cirsoid aneurysms can be diagnosed through endoscopy, angiography, and red cell scanning. Most commonly the diagnosis is made through endoscopy. Proper diagnosis is imperative. Recognition and treatment reduces mortality from 80% to b 10% [3]. Confirmation is made using the following criteria: arterial or micropulsatile streaming from mucosal defect b 3 mm or through normal surrounding mucosa, protruding vessel with or without bleeding, and clot with narrow point of attachment to mucosal defect or to normal appearing mucosa [1]. In our patient, the laceration to the left leg was thought to be the sole proprietor of blood loss, so other sources were overlooked and an
http://dx.doi.org/10.1016/j.ehpc.2017.07.005 2214-3300/© 2017 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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T. Bihun, J. Ribe / Human Pathology: Case Reports 10 (2017) 89–91
Fig. 1. Gross photograph of blood in GI tract. Fig. 3. Arterial malformation in body of stomach.
endoscopy was never ordered. Endoscopy can be problematic, 30% of cases can be misdiagnosed or missed completely if the aneurysm is surrounded by normal mucosa or actively bleeding [2,3]. Capsule endoscopy is another non-invasive alternative, but again it can be missed if not actively bleeding and is limited in the sense that it does not allow for therapeutic intervention [1]. Computed tomography angiography can also be used for visualization if endoscopy is negative. A suggestive, not definitive, diagnosis is when tortuous vessels in the area of the left gastric artery lack early venous return [1]. Rarely, red cell scanning using technetium-99 mm labeled red blood cells can also be used for diagnosis. Current treatment calls for endoscopic hemostasis, and is the treatment of choice for lesions that are easily accessible [7]. Hemostasis is achieved through 3 different therapies: thermal, regional injection, and mechanical. Thermal uses electrocoagulation, heat probe coagulation, and argon plasma coagulation. Regional injection employs local epinephrine injections and sclerotherapy. Mechanical therapy uses banding and clipping. Studies have shown mechanical therapy is the most effective, but combined therapies have been proven to be more successful than monotherapy [1]. The following is criteria that must be met for treatment to be considered successful: no blood spurting or capillary hemorrhage, endoscopic field of view is clear and not clouded by blood, no hematemesis or melena, blood pressure must be in normal range or stable, and pulse rate decreased or in normal rage. Treatment is considered unsuccessful if hematemesis or melena is seen after 48 h, hemoglobin
Fig. 2. Gross photograph of stomach that contained 350 cm3 of blood.
decreases by 2.0 g/L or if there is evidence of hypovolemic shock [5]. Surgical intervention is usually only employed for therapeutic failure. Hemostasis is achieved in 90–95% of cases, therefore surgical ligation is needed in b 5% of cases [6]. However, in the cases of jejunal cirsoid aneurysms, which are similar in clinical and histological presentation, surgical resection has been more successful in the resolution of bleeding [7]. What actually causes the cirsoid aneurysm to bleed is unknown, but it has been theorized that the abnormal vessel bleeds when the thin mucosa is eroded due to mechanical pressure or trauma [1,7]. There are also reports that propose an increase in visceral arterial blood flow secondary to some form of heart disease may predispose those patients with cirsoid aneurysm to rupture [7]. In our case, the individual underwent trauma and autopsy found evidence of heart disease including cardiomegaly as well as a hypertrophied septum and dilated ventricles. Ultimately, gastric cirsoid aneurysm is a rare, but potentially fatal, cause of GI bleed. It should not be overlooked and considered in differential diagnosis when attempting to find the source of an upper GI bleed.
Acknowledgement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Fig. 4. H&E stain of arterial malformation in the submucosa of the stomach.
T. Bihun, J. Ribe / Human Pathology: Case Reports 10 (2017) 89–91
References [1] M. Baxter, E. Aly, Dieulafoy's lesion: current trends in diagnosis and management, Ann. R. Coll. Surg. Engl. 92 (2010) 548–554. [2] H. Jeon, G. Kim, Endoscopic Management of Dieulafoy's Lesion, Clin. Endosc. 48 (2015) 112–120. [3] R. Saleh, A. Lucerna, J. Espinosa, et al., Dieulafoy lesion: the little known sleeping giant of gastrointestinal bleeds, Am. J. Emerg. Med. 34 (2016) 2464. [4] M. Njeru, A. Seifi, Z. Salam, et al., Dieulafoy Lesion: A Rare Cause of Gastrointestinal Bleeding [Letter to the Editor], Southern Medical Association, 2009 336–337.
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[5] P. Gambhire, S. Jain, P. Rathi, et al., Dieulafoy disease of stomach—an uncommon cause of gastrointestinal system bleeding, J. Assoc. Physicians India 62 (2014) 526–528. [6] B. Nandi, P. Hota, R. Ganjoo, Dieulafoy's lesion: an uncommon cause of upper gastrointestinal bleeding, Armed Forces Med. J. India 62 (2005) 284–285. [7] J. Vetto, P. Richman, K. Kariger, et al., Cirsoid aneurysms of the jejunum: an unrecognized cause of massive gastrointestinal bleeding, Arch. Surg. 124 (1989) 1460–1562. [8] L. Finkel, I. Schwartz, Fatal hemorrhage from a gastric cirsoid aneurysm, Hum. Pathol. 16 (1985) 420–422.
Contents lists available at ScienceDirect
Human Pathology: Case Reports journal homepage: http://www.humanpathologycasereports.com
Case Report
Gastric cirsoid aneurysm: Uncommon cause of death from upper GI bleed Tatiana Bihun, BA a,⁎, James Ribe, JD, MD b a b
Saba University School of Medicine, C/O R3 Education Inc., 27 Jackson Road, Suite 301, Devens, MA 01434, United States Los Angeles County Department of Medical Examiner –Coroner, 1104 N Mission Road, Los Angeles, CA 90033, United States
a r t i c l e
i n f o
Article history: Received 19 April 2017 Received in revised form 2 July 2017 Accepted 14 July 2017 Available online xxxx Keywords: Gastric cirsoid aneurysm Dieulafoy lesion Arterial malformation Gastrointestinal bleed
a b s t r a c t Gastric cirsoid aneurysm is an arterial malformation found in the submucosa of the stomach. It is a rare, but potentially life-threatening cause of gastrointestinal bleed. We present a case of a 48 year old male who presented to the ER unconscious, unresponsive, pale, and tachycardic. Patient expired and an autopsy was performed. Upon examination blood was found in the GI tract. During examination an arterial malformation was found in the body of the stomach. Histological samples were taken and the findings were consistent with gastric cirsoid aneurysm. Diagnosis can be made through endoscopy, angiography, or red cell scanning. Current treatment is hemostasis achieved by either thermal, regional injection or mechanical therapies. Multiple therapies are found to be more successful than monotherapy. Gastric cirsoid aneurysms are thought to make up b 5% of upper GI bleeds, however clinicians should be mindful when working up a differential diagnosis. © 2017 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http:// creativecommons.org/licenses/by-nc-nd/4.0/).
1. Introduction Dr. Paul Dieulafoy (1839–1911) was a French physician who first described patients with hematemesis due to a bleeding gastric vessel with no evidence of ulceration. Dieulafoy lesion was used to describe this finding. Moving away from this type of nomenclature, Dieulafoy lesions have been reclassified as gastric cirsoid aneurysms. No definitive cause is known, but it is characterized as an abnormally large tortuous submucosal artery that protrudes through a 2–5 mm opening, that may rupture spontaneously. Cirsoid Aneurysms can be found anywhere along the GI tract, but 70% are found in the stomach. They can be found in all age groups and both genders, but are twice as common in men [1]. Patients will most commonly present with melena, hematemesis, hematochezia, anemia, weakness, fatigue, dizziness, and dyspnea on exertion [1,2,3]. Hemorrhage may also be intermittent if a thrombus is formed and later becomes dislodged [8]. Cirsoid aneurysms are often difficult to recognize due to vague symptomatology and a negative history of NSAIDs, peptic ulcers, or alcohol abuse [4,8].
2. Case report A 48 year old male presented to the ER unconscious and unresponsive from a construction site. Clinical exam exhibited no central pulse, pallor, tachycardia of 153 bpm, no ecchymosis, abrasions or lacerations ⁎ Corresponding author at: 892 Stanley Street, West Islip, NY 11795, United States. E-mail addresses: [email protected], [email protected] (T. Bihun).
to the chest or abdomen, deep laceration to left leg and blood pressure of 142/62 mm Hg. FAST ultrasound was performed and was negative. A left sided thoracotomy was performed, no pericardial effusions or blood was seen in the left thorax. A right sided chest tube was placed but no blood drained. Patient was given 5 units of packed red blood cells. Despite life saving measures patient expired shortly after arrival. The case was reported to the coroner's office for autopsy. Upon examination there was no free blood in the cavity, but blood was found within the GI tract as well as the stomach (Fig. 1). Over 350 cm3 of blood were collected from the stomach after it was removed (Fig. 2). Upon dissection, an arterial malformation was seen in the body of the stomach (Fig. 3). Samples of the arterial malformation were taken for histology. A tortuous artery could be seen imbedded in the submucosa (Fig. 4). Diagnosis of gastric cirsoid aneurysm was made.
3. Discussion Gastric cirsoid aneurysms can be diagnosed through endoscopy, angiography, and red cell scanning. Most commonly the diagnosis is made through endoscopy. Proper diagnosis is imperative. Recognition and treatment reduces mortality from 80% to b 10% [3]. Confirmation is made using the following criteria: arterial or micropulsatile streaming from mucosal defect b 3 mm or through normal surrounding mucosa, protruding vessel with or without bleeding, and clot with narrow point of attachment to mucosal defect or to normal appearing mucosa [1]. In our patient, the laceration to the left leg was thought to be the sole proprietor of blood loss, so other sources were overlooked and an
http://dx.doi.org/10.1016/j.ehpc.2017.07.005 2214-3300/© 2017 Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
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T. Bihun, J. Ribe / Human Pathology: Case Reports 10 (2017) 89–91
Fig. 1. Gross photograph of blood in GI tract. Fig. 3. Arterial malformation in body of stomach.
endoscopy was never ordered. Endoscopy can be problematic, 30% of cases can be misdiagnosed or missed completely if the aneurysm is surrounded by normal mucosa or actively bleeding [2,3]. Capsule endoscopy is another non-invasive alternative, but again it can be missed if not actively bleeding and is limited in the sense that it does not allow for therapeutic intervention [1]. Computed tomography angiography can also be used for visualization if endoscopy is negative. A suggestive, not definitive, diagnosis is when tortuous vessels in the area of the left gastric artery lack early venous return [1]. Rarely, red cell scanning using technetium-99 mm labeled red blood cells can also be used for diagnosis. Current treatment calls for endoscopic hemostasis, and is the treatment of choice for lesions that are easily accessible [7]. Hemostasis is achieved through 3 different therapies: thermal, regional injection, and mechanical. Thermal uses electrocoagulation, heat probe coagulation, and argon plasma coagulation. Regional injection employs local epinephrine injections and sclerotherapy. Mechanical therapy uses banding and clipping. Studies have shown mechanical therapy is the most effective, but combined therapies have been proven to be more successful than monotherapy [1]. The following is criteria that must be met for treatment to be considered successful: no blood spurting or capillary hemorrhage, endoscopic field of view is clear and not clouded by blood, no hematemesis or melena, blood pressure must be in normal range or stable, and pulse rate decreased or in normal rage. Treatment is considered unsuccessful if hematemesis or melena is seen after 48 h, hemoglobin
Fig. 2. Gross photograph of stomach that contained 350 cm3 of blood.
decreases by 2.0 g/L or if there is evidence of hypovolemic shock [5]. Surgical intervention is usually only employed for therapeutic failure. Hemostasis is achieved in 90–95% of cases, therefore surgical ligation is needed in b 5% of cases [6]. However, in the cases of jejunal cirsoid aneurysms, which are similar in clinical and histological presentation, surgical resection has been more successful in the resolution of bleeding [7]. What actually causes the cirsoid aneurysm to bleed is unknown, but it has been theorized that the abnormal vessel bleeds when the thin mucosa is eroded due to mechanical pressure or trauma [1,7]. There are also reports that propose an increase in visceral arterial blood flow secondary to some form of heart disease may predispose those patients with cirsoid aneurysm to rupture [7]. In our case, the individual underwent trauma and autopsy found evidence of heart disease including cardiomegaly as well as a hypertrophied septum and dilated ventricles. Ultimately, gastric cirsoid aneurysm is a rare, but potentially fatal, cause of GI bleed. It should not be overlooked and considered in differential diagnosis when attempting to find the source of an upper GI bleed.
Acknowledgement This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.
Fig. 4. H&E stain of arterial malformation in the submucosa of the stomach.
T. Bihun, J. Ribe / Human Pathology: Case Reports 10 (2017) 89–91
References [1] M. Baxter, E. Aly, Dieulafoy's lesion: current trends in diagnosis and management, Ann. R. Coll. Surg. Engl. 92 (2010) 548–554. [2] H. Jeon, G. Kim, Endoscopic Management of Dieulafoy's Lesion, Clin. Endosc. 48 (2015) 112–120. [3] R. Saleh, A. Lucerna, J. Espinosa, et al., Dieulafoy lesion: the little known sleeping giant of gastrointestinal bleeds, Am. J. Emerg. Med. 34 (2016) 2464. [4] M. Njeru, A. Seifi, Z. Salam, et al., Dieulafoy Lesion: A Rare Cause of Gastrointestinal Bleeding [Letter to the Editor], Southern Medical Association, 2009 336–337.
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[5] P. Gambhire, S. Jain, P. Rathi, et al., Dieulafoy disease of stomach—an uncommon cause of gastrointestinal system bleeding, J. Assoc. Physicians India 62 (2014) 526–528. [6] B. Nandi, P. Hota, R. Ganjoo, Dieulafoy's lesion: an uncommon cause of upper gastrointestinal bleeding, Armed Forces Med. J. India 62 (2005) 284–285. [7] J. Vetto, P. Richman, K. Kariger, et al., Cirsoid aneurysms of the jejunum: an unrecognized cause of massive gastrointestinal bleeding, Arch. Surg. 124 (1989) 1460–1562. [8] L. Finkel, I. Schwartz, Fatal hemorrhage from a gastric cirsoid aneurysm, Hum. Pathol. 16 (1985) 420–422.