American Journal of Emergency Medicine xxx (2015) xxx–xxx
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm☆,☆☆,★,★★ Intraabdominal bleeding from a ruptured artery aneurysm in a young adult is rare. This report describes a patient who presented to the emergency department with hemorrhagic shock caused by a ruptured aneurysm in an omental branch of the right gastroepiploic artery. The gastroepiploic artery aneurysm was successfully treated with a superselective transcatheter arterial embolization. In addition, the angiography of the superior mesenteric artery revealed multifocal stenosis, which has a typical “string-of-beads“ appearance, characteristic of fibromuscular dysplasia (FMD) in the jejunal artery. Fibromuscular dysplasia is a rare disease that can cause stenosis, occlusion, aneurysm, or dissection in a medium-sized artery and is more common in women than men. The natural history, causative genes, and pathogenesis of FMD have not been fully elucidated; however, delayed diagnosis of FMD has been reported. In a young female patient with intraabdominal bleeding, clinicians should consider the possibility of a ruptured aneurysm due to FMD. Examination with early-phase angiography and endovascular treatment may reduce the need for laparotomy for hemostasis. Awareness of FMD among emergency physicians could potentially shorten diagnostic delays and increase the chances of effective examination and treatment of this condition. Intraabdominal bleeding from a ruptured artery aneurysm in a young adult is rare. Herein, we describe a patient who presented to the emergency department (ED) of the hospital with sudden-onset intraabdominal hemorrhage from a right gastroepiploic artery aneurysm with a string-ofbeads appearance characteristic of fibromuscular dysplasia (FMD). A 20-year-old woman was transferred to the ED of the hospital owing to worsening upper abdominal pain. She was healthy and without any abdominal symptoms or trauma history until the day of admission. She experienced onset of abdominal pain 12 hours before transfer to our hospital. Upon admission to the ED, she was disoriented and had a respiratory rate of 25 breaths per minute, blood pressure of 72/45 mm Hg, heart rate of 148 beats per minute, a blood lactate level of 6.8 mmol/L, base excess of − 7.5 mmol/L, and a hemoglobin level of 10.0 g/dL. She had upper abdominal pain and displayed Blumberg sign. Intraabdominal fluid collection was identified on ultrasonography. After rapid stabilization of her blood pressure using 1-L crystalloid infusion, abdominal contrast-enhanced computed tomography (CT) was carried out. It revealed intraperitoneal bleeding in the omental bursa ☆ Informed consent: Written informed consent for the publication of their individual details was obtained from the patient whose case is reported here. ☆☆ Funding: There was no funding. ★ Conflict of interest: none. ★★ Presentation at meeting: none.
and mesentery of the transverse colon; however, neither extravasation nor aneurysm was identified using a 320-row multidetector CT with a slice thickness of 0.5 mm (Fig. 1). Initial fluid therapy consisting of an additional 1.5-L crystalloid infusion and a packed red cell transfusion of 560 mL reversed the patient’s hypotension and tachycardia. Subsequent to emergency treatment, the patient received conservative therapy in the intensive care unit. On day 3 after admission, she was discharged from the intensive care unit to the general ward of the hospital because she had no symptoms of recurrent bleeding. On day 6, to identify the bleeding source, an abdominal contrast-enhanced CT scan was repeated. A small (3 mm) aneurysm in an omental branch of the right gastroepiploic artery was suspected, as seen on maximum intensity projection (MIP) images (Fig. 2). Thus, elective angiography was indicated and performed on day 8. Radiographic selective catheter angiography of the superior mesenteric artery revealed a string-of-beads appearance (a typical sign of FMD) in the jejunal artery (Fig. 3). Subsequent angiography of the celiac artery revealed a right gastroepiploic artery aneurysm, which was successfully treated by superselective transcatheter arterial embolization using 17% N-butyl cyanoacrylate via a triple coaxial system (Fig. 4). The patient was discharged on day 15 and did not experience relapse or complications in the 8-month follow-up period. This patient had both acute abdominal pain and hemorrhagic shock upon arrival at the ED. She had a ruptured gastroepiploic artery aneurysm and multifocal stenosis, which has a typical “string-of-beads” appearance characteristic of FMD [1,2]. Fibromuscular dysplasia is a nonatherosclerotic, non-inflammatory disease that can develop stenosis, occlusion, aneurysms, or dissection of a medium-sized artery [2,1]. The estimated prevalence of FMD is 1 in 200,000 or higher [3,4]. Genetic factors likely contribute to the susceptibility to FMD, but causative genes have not been identified [1–3]. An average 4- to 9-year delay in diagnosing FMD from initial symptom onset [5] may correlate with the condition’s low prevalence and unresolved disease mechanism. Fibromuscular dysplasia is more common in women than in men by a ratio of 9:1 (average age at diagnosis, 52 years) [4,3]. Estrogen and smoking have been suggested as potential factors that lead to the development of FMD [3]. Thus, the present case of a young woman is typical for FMD, although she had no smoking history. Recent advances in angiographic examination and endovascular treatments have decreased the availability of pathology specimens [3]. In 2012, a European Consensus panel proposed 3 angiographic classifications, including unifocal (solitary stenosis b 1 cm in length), tubular (stenosis ≥1 cm in length), and multifocal (with a “string-of-beads” appearance), as our case was classified [1]. Differential diagnosis for multifocal FMD includes exposure to vasoconstrictor agents or aortic
0735-6757/© 2015 Elsevier Inc. All rights reserved.
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
2
M. Yamada et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
Fig. 1. Abdominal contrast-enhanced CT on admission. Intraperitoneal bleeding in omental brusa and mesentery of transverse colon. A, Axial image. B, Coronal image.
involvement, the latter including mid-aortic syndrome characterized by segmental narrowing of the proximal abdominal aorta [1], which was not identified in our case. The most common sites of vascular abnormalities in FMD are the renal area (80%-89%) or cervicocephalic arteries (26%-74%); celiac and mesenteric arteries are less common sites (9%-26%) [6,4]. Thus, the vascular abnormality in the gastroepiploic artery (aneurysm) or the superior mesenteric artery (showing a string-of-beads appearance) in this case is uncommon. Because FMD often affects multiple vascular sites
Fig. 2. Abdominal contrast-enhanced CT on day 6. A small aneurysm in an omental branch of the right gastroepiploic artery with the use of the MIP technique. The arrow indicates the aneurysm.
Fig. 3. Angiography of the superior mesenteric artery. A, Angiography of the superior mesenteric artery. B, A string-of-beads appearance (a typical sign of FMD) in the jejunal artery (enlarged image of panel A).
(2 or more sites, 16%-35%), susceptible vascular sites, such as the renal area and cervicocephalic arteries, should be screened in cases of suspected FMD. In this case, magnetic resonance angiography of the head and neck identified no vascular abnormalities. The patient experienced hemorrhagic shock due to a gastroepiploic artery aneurysm, which has been reported in 3.5% of all cases of visceral artery aneurysms [7]. The size of the aneurysm in this patient was 3 mm, which was considerably smaller than the average size of a ruptured aneurysm in the visceral arteries (12-37 mm) [8]. Thus, it appears possible that
Fig. 4. Angiography of celiac artery. Angiography of celiac artery showing a right gastroepiploic artery aneurysm, which was successfully treated by superselective transcatheter arterial embolization. The arrow indicates the aneurysm.
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
M. Yamada et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
even small FMD aneurysms can rupture. Because the aneurysm was not detected in the initial enhanced CT scan performed on admission, even with the use of the MIP technique, identifying such a small aneurysm immediately after recovery from shock was not likely feasible. However, an aneurysm identified by the second thin-slice CT scan with MIP under hemodynamically stable conditions highlighted the effectiveness of such an examination. Subsequent catheter angiography confirmed the presence of the aneurysm, which was successfully treated using an endovascular approach involving a minimally invasive procedure. In a young female patient who presents with intraabdominal bleeding, the possibility of a ruptured aneurysm of FMD should be considered. Thin-slice CT using the MIP technique was effective for identifying a small aneurysm; early-phase angiography for examination and endovascular treatment may reduce the need for laparotomy for hemostasis. The awareness of FMD among emergency physicians would potentially shorten diagnostic delay and increase the chances of effective examination and treatment of this condition. Mami Yamada MD Senshu Trauma and Critical Care Center, 2 -23 Rinku Orai Kita Osaka 598-8577, Japan Taka-aki Nakada MD PhD Senshu Trauma and Critical Care Center 2 -23 Rinku Orai Kita, Osaka 598-8577, Japan Chiba University Graduate School of Medicine 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan Corresponding author at: Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan. Tel.: +81 72 469 3111 fax: +81 72 469 7929 E-mail address: [email protected]
3
Koji Idoguchi MD Senshu Trauma and Critical Care Center, 2 -23 Rinku Orai Kita Osaka 598-8577, Japan Tetsuya Matsuoka MD PhD Chiba University Graduate School of Medicine 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan
http://dx.doi.org/10.1016/j.ajem.2015.06.054 References [1] Persu A, Giavarini A, Touze E, Januszewicz A, Sapoval M, Azizi M, et al. European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens 2014;32:1367–78. [2] Olin JW, Gornik HL, Bacharach JM, Biller J, Fine LJ, Gray BH, et al. Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation 2014;129:1048–78. [3] O'Connor SC, Gornik HL. Recent developments in the understanding and management of fibromuscular dysplasia. J Am Heart Assoc 2014;3:e001259. [4] Olin JW, Froehlich J, Gu X, Bacharach JM, Eagle K, Gray BH, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation 2012;125:3182–90. [5] Olin JW. Is fibromuscular dysplasia a single disease? Circulation 2012;126: 2925–7. [6] Luscher TF, Keller HM, Imhof HG, Greminger P, Kuhlmann U, Largiader F, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic outcome. Results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron 1986(44 Suppl. 1):109–14. [7] Faler B, Mukherjee D. Hemorrhagic shock secondary to rupture of a right gastroepiploic artery aneurysm: case report and brief review of splanchnic artery aneurysms. Int J Angiol 2007;16:24–6. [8] Shukla AJ, Eid R, Fish L, Avgerinos E, Marone L, Makaroun M, et al. Contemporary outcomes of intact and ruptured visceral artery aneurysms. J Vasc Surg 2015. http:// dx.doi.org/10.1016/j.jvs.2015.01.005 [Epub ahead of print].
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
Contents lists available at ScienceDirect
American Journal of Emergency Medicine journal homepage: www.elsevier.com/locate/ajem
Case Report
Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm☆,☆☆,★,★★ Intraabdominal bleeding from a ruptured artery aneurysm in a young adult is rare. This report describes a patient who presented to the emergency department with hemorrhagic shock caused by a ruptured aneurysm in an omental branch of the right gastroepiploic artery. The gastroepiploic artery aneurysm was successfully treated with a superselective transcatheter arterial embolization. In addition, the angiography of the superior mesenteric artery revealed multifocal stenosis, which has a typical “string-of-beads“ appearance, characteristic of fibromuscular dysplasia (FMD) in the jejunal artery. Fibromuscular dysplasia is a rare disease that can cause stenosis, occlusion, aneurysm, or dissection in a medium-sized artery and is more common in women than men. The natural history, causative genes, and pathogenesis of FMD have not been fully elucidated; however, delayed diagnosis of FMD has been reported. In a young female patient with intraabdominal bleeding, clinicians should consider the possibility of a ruptured aneurysm due to FMD. Examination with early-phase angiography and endovascular treatment may reduce the need for laparotomy for hemostasis. Awareness of FMD among emergency physicians could potentially shorten diagnostic delays and increase the chances of effective examination and treatment of this condition. Intraabdominal bleeding from a ruptured artery aneurysm in a young adult is rare. Herein, we describe a patient who presented to the emergency department (ED) of the hospital with sudden-onset intraabdominal hemorrhage from a right gastroepiploic artery aneurysm with a string-ofbeads appearance characteristic of fibromuscular dysplasia (FMD). A 20-year-old woman was transferred to the ED of the hospital owing to worsening upper abdominal pain. She was healthy and without any abdominal symptoms or trauma history until the day of admission. She experienced onset of abdominal pain 12 hours before transfer to our hospital. Upon admission to the ED, she was disoriented and had a respiratory rate of 25 breaths per minute, blood pressure of 72/45 mm Hg, heart rate of 148 beats per minute, a blood lactate level of 6.8 mmol/L, base excess of − 7.5 mmol/L, and a hemoglobin level of 10.0 g/dL. She had upper abdominal pain and displayed Blumberg sign. Intraabdominal fluid collection was identified on ultrasonography. After rapid stabilization of her blood pressure using 1-L crystalloid infusion, abdominal contrast-enhanced computed tomography (CT) was carried out. It revealed intraperitoneal bleeding in the omental bursa ☆ Informed consent: Written informed consent for the publication of their individual details was obtained from the patient whose case is reported here. ☆☆ Funding: There was no funding. ★ Conflict of interest: none. ★★ Presentation at meeting: none.
and mesentery of the transverse colon; however, neither extravasation nor aneurysm was identified using a 320-row multidetector CT with a slice thickness of 0.5 mm (Fig. 1). Initial fluid therapy consisting of an additional 1.5-L crystalloid infusion and a packed red cell transfusion of 560 mL reversed the patient’s hypotension and tachycardia. Subsequent to emergency treatment, the patient received conservative therapy in the intensive care unit. On day 3 after admission, she was discharged from the intensive care unit to the general ward of the hospital because she had no symptoms of recurrent bleeding. On day 6, to identify the bleeding source, an abdominal contrast-enhanced CT scan was repeated. A small (3 mm) aneurysm in an omental branch of the right gastroepiploic artery was suspected, as seen on maximum intensity projection (MIP) images (Fig. 2). Thus, elective angiography was indicated and performed on day 8. Radiographic selective catheter angiography of the superior mesenteric artery revealed a string-of-beads appearance (a typical sign of FMD) in the jejunal artery (Fig. 3). Subsequent angiography of the celiac artery revealed a right gastroepiploic artery aneurysm, which was successfully treated by superselective transcatheter arterial embolization using 17% N-butyl cyanoacrylate via a triple coaxial system (Fig. 4). The patient was discharged on day 15 and did not experience relapse or complications in the 8-month follow-up period. This patient had both acute abdominal pain and hemorrhagic shock upon arrival at the ED. She had a ruptured gastroepiploic artery aneurysm and multifocal stenosis, which has a typical “string-of-beads” appearance characteristic of FMD [1,2]. Fibromuscular dysplasia is a nonatherosclerotic, non-inflammatory disease that can develop stenosis, occlusion, aneurysms, or dissection of a medium-sized artery [2,1]. The estimated prevalence of FMD is 1 in 200,000 or higher [3,4]. Genetic factors likely contribute to the susceptibility to FMD, but causative genes have not been identified [1–3]. An average 4- to 9-year delay in diagnosing FMD from initial symptom onset [5] may correlate with the condition’s low prevalence and unresolved disease mechanism. Fibromuscular dysplasia is more common in women than in men by a ratio of 9:1 (average age at diagnosis, 52 years) [4,3]. Estrogen and smoking have been suggested as potential factors that lead to the development of FMD [3]. Thus, the present case of a young woman is typical for FMD, although she had no smoking history. Recent advances in angiographic examination and endovascular treatments have decreased the availability of pathology specimens [3]. In 2012, a European Consensus panel proposed 3 angiographic classifications, including unifocal (solitary stenosis b 1 cm in length), tubular (stenosis ≥1 cm in length), and multifocal (with a “string-of-beads” appearance), as our case was classified [1]. Differential diagnosis for multifocal FMD includes exposure to vasoconstrictor agents or aortic
0735-6757/© 2015 Elsevier Inc. All rights reserved.
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
2
M. Yamada et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
Fig. 1. Abdominal contrast-enhanced CT on admission. Intraperitoneal bleeding in omental brusa and mesentery of transverse colon. A, Axial image. B, Coronal image.
involvement, the latter including mid-aortic syndrome characterized by segmental narrowing of the proximal abdominal aorta [1], which was not identified in our case. The most common sites of vascular abnormalities in FMD are the renal area (80%-89%) or cervicocephalic arteries (26%-74%); celiac and mesenteric arteries are less common sites (9%-26%) [6,4]. Thus, the vascular abnormality in the gastroepiploic artery (aneurysm) or the superior mesenteric artery (showing a string-of-beads appearance) in this case is uncommon. Because FMD often affects multiple vascular sites
Fig. 2. Abdominal contrast-enhanced CT on day 6. A small aneurysm in an omental branch of the right gastroepiploic artery with the use of the MIP technique. The arrow indicates the aneurysm.
Fig. 3. Angiography of the superior mesenteric artery. A, Angiography of the superior mesenteric artery. B, A string-of-beads appearance (a typical sign of FMD) in the jejunal artery (enlarged image of panel A).
(2 or more sites, 16%-35%), susceptible vascular sites, such as the renal area and cervicocephalic arteries, should be screened in cases of suspected FMD. In this case, magnetic resonance angiography of the head and neck identified no vascular abnormalities. The patient experienced hemorrhagic shock due to a gastroepiploic artery aneurysm, which has been reported in 3.5% of all cases of visceral artery aneurysms [7]. The size of the aneurysm in this patient was 3 mm, which was considerably smaller than the average size of a ruptured aneurysm in the visceral arteries (12-37 mm) [8]. Thus, it appears possible that
Fig. 4. Angiography of celiac artery. Angiography of celiac artery showing a right gastroepiploic artery aneurysm, which was successfully treated by superselective transcatheter arterial embolization. The arrow indicates the aneurysm.
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
M. Yamada et al. / American Journal of Emergency Medicine xxx (2015) xxx–xxx
even small FMD aneurysms can rupture. Because the aneurysm was not detected in the initial enhanced CT scan performed on admission, even with the use of the MIP technique, identifying such a small aneurysm immediately after recovery from shock was not likely feasible. However, an aneurysm identified by the second thin-slice CT scan with MIP under hemodynamically stable conditions highlighted the effectiveness of such an examination. Subsequent catheter angiography confirmed the presence of the aneurysm, which was successfully treated using an endovascular approach involving a minimally invasive procedure. In a young female patient who presents with intraabdominal bleeding, the possibility of a ruptured aneurysm of FMD should be considered. Thin-slice CT using the MIP technique was effective for identifying a small aneurysm; early-phase angiography for examination and endovascular treatment may reduce the need for laparotomy for hemostasis. The awareness of FMD among emergency physicians would potentially shorten diagnostic delay and increase the chances of effective examination and treatment of this condition. Mami Yamada MD Senshu Trauma and Critical Care Center, 2 -23 Rinku Orai Kita Osaka 598-8577, Japan Taka-aki Nakada MD PhD Senshu Trauma and Critical Care Center 2 -23 Rinku Orai Kita, Osaka 598-8577, Japan Chiba University Graduate School of Medicine 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan Corresponding author at: Senshu Trauma and Critical Care Center 2-23 Rinku Orai Kita, Osaka 598-8577, Japan. Tel.: +81 72 469 3111 fax: +81 72 469 7929 E-mail address: [email protected]
3
Koji Idoguchi MD Senshu Trauma and Critical Care Center, 2 -23 Rinku Orai Kita Osaka 598-8577, Japan Tetsuya Matsuoka MD PhD Chiba University Graduate School of Medicine 1-8-1 Inohana, Chuo, Chiba 260-8677, Japan
http://dx.doi.org/10.1016/j.ajem.2015.06.054 References [1] Persu A, Giavarini A, Touze E, Januszewicz A, Sapoval M, Azizi M, et al. European consensus on the diagnosis and management of fibromuscular dysplasia. J Hypertens 2014;32:1367–78. [2] Olin JW, Gornik HL, Bacharach JM, Biller J, Fine LJ, Gray BH, et al. Fibromuscular dysplasia: state of the science and critical unanswered questions: a scientific statement from the American Heart Association. Circulation 2014;129:1048–78. [3] O'Connor SC, Gornik HL. Recent developments in the understanding and management of fibromuscular dysplasia. J Am Heart Assoc 2014;3:e001259. [4] Olin JW, Froehlich J, Gu X, Bacharach JM, Eagle K, Gray BH, et al. The United States Registry for Fibromuscular Dysplasia: results in the first 447 patients. Circulation 2012;125:3182–90. [5] Olin JW. Is fibromuscular dysplasia a single disease? Circulation 2012;126: 2925–7. [6] Luscher TF, Keller HM, Imhof HG, Greminger P, Kuhlmann U, Largiader F, et al. Fibromuscular hyperplasia: extension of the disease and therapeutic outcome. Results of the University Hospital Zurich Cooperative Study on Fibromuscular Hyperplasia. Nephron 1986(44 Suppl. 1):109–14. [7] Faler B, Mukherjee D. Hemorrhagic shock secondary to rupture of a right gastroepiploic artery aneurysm: case report and brief review of splanchnic artery aneurysms. Int J Angiol 2007;16:24–6. [8] Shukla AJ, Eid R, Fish L, Avgerinos E, Marone L, Makaroun M, et al. Contemporary outcomes of intact and ruptured visceral artery aneurysms. J Vasc Surg 2015. http:// dx.doi.org/10.1016/j.jvs.2015.01.005 [Epub ahead of print].
Please cite this article as: Yamada M, et al, Fibromuscular dysplasia presenting as hemorrhagic shock due to spontaneous rupture of a right gastroepiploic artery aneurysm, Am J Emerg Med (2015), http://dx.doi.org/10.1016/j.ajem.2015.06.054
