Ruptured Aneurysm of the Splenic Artery Associated with Fibromuscular Dysplasia € u,1 Danyel A.F. van den Heuvel,2 Jan-Williem Leeuwis,3 and C¸ agdas¸ Unl€ Jean-Paul P.M. de Vries,1 Nieuwegein, The Netherlands
Rupture of a splenic artery aneurysm is a rare event associated with high mortality rates. Aneurysm of the splenic artery associated with fibromuscular dysplasia (FMD) is even rarer, with only 3 previously reported cases. We report a patient with previously undiagnosed FMD who required emergent intervention because of a ruptured splenic aneurysm. In discussion, we present an overview of literature on splenic aneurysms and FMD, together with pathology and treatment.
Splenic artery aneurysms (SAAs) are an uncommon vascular pathology.1 In large autopsy studies and angiography series, the incidence of SAAs has been estimated between 0.01% and 0.78%.2,3 Rupture of a SAA is a rare event associated with high mortality rates.4 Aneurysm of the splenic artery associated with fibromuscular dysplasia (FMD) is even rarer, with only 3 previously reported cases.5e7 We report a patient with previously undiagnosed FMD who required emergent intervention because of a ruptured splenic aneurysm.
CASE REPORT A 60-year-old woman was presented at our emergency department with acute abdominal pain, which radiated to the back. At presentation, the patient was hypotensive (90/50 mm Hg), with a palpable and tender mass in the left upper quadrant of the abdomen. Complete blood count 1 Department of Vascular Surgery, St. Antonius Hospital, Nieuwegein, The Netherlands. 2 Department of Radiology, St. Antonius Hospital, Nieuwegein, The Netherlands. 3 Department of Pathology, St. Antonius Hospital, Nieuwegein, The Netherlands. € u, Department of Vascular Surgery, Correspondence to: C¸ agdas Unl€ St.Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands; E-mail: [email protected]
Ann Vasc Surg 2014; 28: 1799.e15–1799.e18 http://dx.doi.org/10.1016/j.avsg.2014.05.016 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: April 9, 2014; manuscript accepted: May 20, 2014; published online: June 11, 2014.
showed leukocytosis of 10.8 cells/dL3 and hemoglobin of 7.1 g/dL. A permissive hypotension protocol was started (fluid administration to keep the mean arterial pressure around 70 mm Hg). Contrast-enhanced computed tomography (CT) showed rupture of an aneurysm of the splenic artery with a large perisplenic and peripancreatic hematoma and hemoperitoneum. The celiac trunk was chronically occluded, with extensive collaterals originating from the superior mesenteric artery (SMA) perfusing the celiac axis. Both the left renal artery and the external iliac arteries showed signs of FMD, with the typical ‘string of beads’ appearance (Fig. 1). Despite the occlusion of the celiac trunk, an endovascular approach was regarded as the best treatment. Selective catheterization of the SMA confirmed the occlusion of the celiac trunk and collateral perfusion of the splenic artery. Despite the use of a 2.4F microcatheter (Progreat; Terumo, Tokyo, Japan) supported by a 4F cobra diagnostic catheter (Cordis; Johnson & Johnson, NJ) and a 6F introduction sheath (Destination; Terumo, Tokyo, Japan), it was not possible to navigate through the extensive collaterals and reach the distal embolization site (i.e. distal of the aneurysm) in the splenic artery which was close to the splenic hilum. Thus, a definite endovascular treatment was not possible because of the persistent backbleeding from the extensive anastomoses between the left gastric, transverse pancreatic and gastroepiploic arteries, and the splenic artery. Proximal embolization (i.e. proximal of the aneurysm) of the splenic artery with coils was performed as a step-up approach in an attempt to reduce blood loss during surgery (Fig. 2). Through a midline laparotomy, 1,000 mL of blood was evacuated from the peritoneal cavity, and a splenectomy was performed with ligation of multiple arterial collaterals. Postoperative, the patient was monitored 24 hr at the intensive care unit. After 4 days, the patient could be
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Fig. 1. (A) Contrast-enhanced computed tomography demonstrating the ruptured splenic artery aneurysm; note the large perisplenic hematoma and hemoperitoneum with blood surrounding the liver (arrow). (B) Large peripancreatic and perisplenic hematoma; note
the presence of vast collateralization due to an occlusion of the celiac trunk and again the hemoperitoneum with blood surrounding the liver. (C) Fibromuscular dysplasia of the left renal artery (arrow).
Fig. 2. (A) Selective angiogram of the SMA; enlargement of the arteries of the pancreatico-duodenal arcade and collateral perfusion of the SA (GDA: arrowhead; LGA: short arrow; SA: long arrow). (B) Selective angiogram of the SA; microcatheter in the SA supported by a diagnostic catheter in the proximal collateral vessel and a guiding sheath in the SMA. Note the tortuosity of the
collateral artery. The arrow points to the aneurysm in the SA. (C) Selective angiogram from the proximal collateral after embolization of the proximal SA (LGA: short arrow; TPA: arrowhead; distal SA: long arrow). GDA, gastroduodenal artery; LGA, left gastric artery; SA, splenic artery; TPA, transverse pancreatic artery.
discharged without any complications and without the need for blood transfusion. After splenectomy, vaccine prophylaxis was given. Pathologic examination of the splenic artery showed medial disorganization. The internal elastic lamina was partly fragmented, and the media showed thin areas where most of the smooth muscle fibers were replaced with collagen, alternating with medial fibromuscular thickening. These findings are compatible with medial fibroplasia, the most common form of medial FMD (Fig. 3). There were no signs of inflammation or prominent atherosclerotic lesions. The findings were compared with sex- and agematched control splenic arteries which showed a continuous internal elastic lamina and no medial disorganization.
aneurysmal formation.1 Portal hypertension and atherosclerosis are regarded as important factors for SAA formation.4,8 Specific to SAAs is a 4:1 female-to-male predominance. Multiple pregnancies are another factor associated with SAA.4 FMD is a nonatherosclerotic vascular disease of unknown origin that frequently involves small- to medium-sized arteries that most commonly affects the renal and carotid arteries.9 Prevalence of aneurysm formation (19%) is low and most patients present with stenosis.9,10 It occurs frequently in young individuals and women.9 The first histologically proven case of FMD concerned the carotid artery reported by Connett and Lansche in 1965.11 The pathologic FMD classification is based on the depth of the arterial wall layer involved. Intimal FMD (also called intimal fibroplasia) accounts for
Rupture of a splenic artery aneurysm is a rare event associated with high mortality rates. Aneurysm of the splenic artery associated with fibromuscular dysplasia (FMD) is even rarer, with only 3 previously reported cases. We report a patient with previously undiagnosed FMD who required emergent intervention because of a ruptured splenic aneurysm. In discussion, we present an overview of literature on splenic aneurysms and FMD, together with pathology and treatment.
Splenic artery aneurysms (SAAs) are an uncommon vascular pathology.1 In large autopsy studies and angiography series, the incidence of SAAs has been estimated between 0.01% and 0.78%.2,3 Rupture of a SAA is a rare event associated with high mortality rates.4 Aneurysm of the splenic artery associated with fibromuscular dysplasia (FMD) is even rarer, with only 3 previously reported cases.5e7 We report a patient with previously undiagnosed FMD who required emergent intervention because of a ruptured splenic aneurysm.
CASE REPORT A 60-year-old woman was presented at our emergency department with acute abdominal pain, which radiated to the back. At presentation, the patient was hypotensive (90/50 mm Hg), with a palpable and tender mass in the left upper quadrant of the abdomen. Complete blood count 1 Department of Vascular Surgery, St. Antonius Hospital, Nieuwegein, The Netherlands. 2 Department of Radiology, St. Antonius Hospital, Nieuwegein, The Netherlands. 3 Department of Pathology, St. Antonius Hospital, Nieuwegein, The Netherlands. € u, Department of Vascular Surgery, Correspondence to: C¸ agdas Unl€ St.Antonius Hospital, Koekoekslaan 1, 3435 CM Nieuwegein, The Netherlands; E-mail: [email protected]
Ann Vasc Surg 2014; 28: 1799.e15–1799.e18 http://dx.doi.org/10.1016/j.avsg.2014.05.016 Ó 2014 Elsevier Inc. All rights reserved. Manuscript received: April 9, 2014; manuscript accepted: May 20, 2014; published online: June 11, 2014.
showed leukocytosis of 10.8 cells/dL3 and hemoglobin of 7.1 g/dL. A permissive hypotension protocol was started (fluid administration to keep the mean arterial pressure around 70 mm Hg). Contrast-enhanced computed tomography (CT) showed rupture of an aneurysm of the splenic artery with a large perisplenic and peripancreatic hematoma and hemoperitoneum. The celiac trunk was chronically occluded, with extensive collaterals originating from the superior mesenteric artery (SMA) perfusing the celiac axis. Both the left renal artery and the external iliac arteries showed signs of FMD, with the typical ‘string of beads’ appearance (Fig. 1). Despite the occlusion of the celiac trunk, an endovascular approach was regarded as the best treatment. Selective catheterization of the SMA confirmed the occlusion of the celiac trunk and collateral perfusion of the splenic artery. Despite the use of a 2.4F microcatheter (Progreat; Terumo, Tokyo, Japan) supported by a 4F cobra diagnostic catheter (Cordis; Johnson & Johnson, NJ) and a 6F introduction sheath (Destination; Terumo, Tokyo, Japan), it was not possible to navigate through the extensive collaterals and reach the distal embolization site (i.e. distal of the aneurysm) in the splenic artery which was close to the splenic hilum. Thus, a definite endovascular treatment was not possible because of the persistent backbleeding from the extensive anastomoses between the left gastric, transverse pancreatic and gastroepiploic arteries, and the splenic artery. Proximal embolization (i.e. proximal of the aneurysm) of the splenic artery with coils was performed as a step-up approach in an attempt to reduce blood loss during surgery (Fig. 2). Through a midline laparotomy, 1,000 mL of blood was evacuated from the peritoneal cavity, and a splenectomy was performed with ligation of multiple arterial collaterals. Postoperative, the patient was monitored 24 hr at the intensive care unit. After 4 days, the patient could be
1799.e15
1799.e16 Case reports
Annals of Vascular Surgery
Fig. 1. (A) Contrast-enhanced computed tomography demonstrating the ruptured splenic artery aneurysm; note the large perisplenic hematoma and hemoperitoneum with blood surrounding the liver (arrow). (B) Large peripancreatic and perisplenic hematoma; note
the presence of vast collateralization due to an occlusion of the celiac trunk and again the hemoperitoneum with blood surrounding the liver. (C) Fibromuscular dysplasia of the left renal artery (arrow).
Fig. 2. (A) Selective angiogram of the SMA; enlargement of the arteries of the pancreatico-duodenal arcade and collateral perfusion of the SA (GDA: arrowhead; LGA: short arrow; SA: long arrow). (B) Selective angiogram of the SA; microcatheter in the SA supported by a diagnostic catheter in the proximal collateral vessel and a guiding sheath in the SMA. Note the tortuosity of the
collateral artery. The arrow points to the aneurysm in the SA. (C) Selective angiogram from the proximal collateral after embolization of the proximal SA (LGA: short arrow; TPA: arrowhead; distal SA: long arrow). GDA, gastroduodenal artery; LGA, left gastric artery; SA, splenic artery; TPA, transverse pancreatic artery.
discharged without any complications and without the need for blood transfusion. After splenectomy, vaccine prophylaxis was given. Pathologic examination of the splenic artery showed medial disorganization. The internal elastic lamina was partly fragmented, and the media showed thin areas where most of the smooth muscle fibers were replaced with collagen, alternating with medial fibromuscular thickening. These findings are compatible with medial fibroplasia, the most common form of medial FMD (Fig. 3). There were no signs of inflammation or prominent atherosclerotic lesions. The findings were compared with sex- and agematched control splenic arteries which showed a continuous internal elastic lamina and no medial disorganization.
aneurysmal formation.1 Portal hypertension and atherosclerosis are regarded as important factors for SAA formation.4,8 Specific to SAAs is a 4:1 female-to-male predominance. Multiple pregnancies are another factor associated with SAA.4 FMD is a nonatherosclerotic vascular disease of unknown origin that frequently involves small- to medium-sized arteries that most commonly affects the renal and carotid arteries.9 Prevalence of aneurysm formation (19%) is low and most patients present with stenosis.9,10 It occurs frequently in young individuals and women.9 The first histologically proven case of FMD concerned the carotid artery reported by Connett and Lansche in 1965.11 The pathologic FMD classification is based on the depth of the arterial wall layer involved. Intimal FMD (also called intimal fibroplasia) accounts for
