Successful surgical treatment of a patient with multiple visceral artery aneurysms due to fibromuscular dysplasia

Successful surgical treatment of a patient with multiple visceral artery aneurysms due to fibromuscular dysplasia

Cardiovascular Surgery, Vol. 10, No. 2, pp. 157–160, 2002  2002 Published by Elsevier Science Ltd on behalf of The International Society for Cardiova...

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Cardiovascular Surgery, Vol. 10, No. 2, pp. 157–160, 2002  2002 Published by Elsevier Science Ltd on behalf of The International Society for Cardiovascular Surgery 0967-2109/02 $22.00

PII: S0967-2109(01)00111-9

www.elsevier.com/locate/cardiosur

CASE REPORT Successful surgical treatment of a patient with multiple visceral artery aneurysms due to fibromuscular dysplasia Atsuo Kojima, Shunya Shindo, Kenji Kubota, Keiji Iyori, Tadao Ishimoto, Masahiro Kobayashi and Yusuke Tada Second Department of Surgery, Yamanashi Medical University, 1110 Shimokato, Tamaho-cho, Nakakoma-gun, Yamanashi, 409-3898, Japan Multiple visceral artery aneurysms due to fibromuscular dysplasia are rare. A 43-yr-old man with a pulsatile abdominal mass detected by ultrasonography had multiple visceral artery aneurysms diagnosed by angiography. This included a huge superior mesenteric artery aneurysm. Aneurysm resection and arterial reconstruction was performed successfully. Pathologic examination revealed fibromuscular dysplasia of the medial fibroplasia type.  2002 Published by Elsevier Science Ltd on behalf of The International Society for Cardiovascular Surgery Keywords: fibromuscular dysplasia, visceral artery aneurysm

Introduction Visceral artery aneurysms are relatively uncommon [1]. Usually, they are diagnosed after rupture. Recent advances in diagnostic techniques, including the routine use of ultrasonography, have made it possible to detect these aneurysms in an asymptomatic stage. A patient with multiple visceral artery aneurysms was successfully treated in our department. The etiology of the aneurysms was fibromuscular dysplasia (FMD) of the medial fibroplasia type.

Case report A pulsatile abdominal mass was detected incidentally by ultrasonography in a 43-yr-old man. Contrast enhanced computed tomography revealed a 6-cm diameter superior mesenteric artery aneurysm. Angiography demonstrated visceral artery aneurysms of the superior mesenteric artery, common hepatic artery, proper hepatic artery, splenic artery, jejunal

Correspondence to: Atsuo Kojima, M.D. Tel.: +81-55-273-9682; Fax: +81-55-273-6767; e-mail: [email protected]

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artery, and bilateral internal iliac arteries (Figure 1). The left femoral artery also contained a fusiform aneurysm [Figure 2(A)]. The common hepatic artery was occluded just distal to the aneurysm. The hepatic circulation was maintained via the pancreaticoduodenal artery. The superior mesenteric artery was occluded distal to the aneurysm. The intestinal blood flow was maintained via the arcade of the jejunal artery. The patient had no risk factors for atherosclerosis. At operation, the superior mesenteric artery aneurysm was resected. The distal superior mesenteric artery near the orifice of the ileocolic artery was bypassed with one leg of a pantaloon vein graft made from autogenous saphenous vein. The middle colic artery was ligated because it originated from the aneurysm. The other leg of the pantaloon vein graft was anastomosed sequentially to the conjoined jejunal arteries and the inferior pancreatico-duodenal artery [Figure 2(B)]. The common hepatic artery aneurysm was resected. The splenic artery aneurysm was excluded by ligation. It was reconstructed from the celiac axis with a vein graft. Angioplasty of the proper hepatic artery aneurysm was performed with lateral sutures. The internal iliac artery aneurysms were untouched because they were small. The left femoral 157

Multiple visceral aneurysms A. Kojima et al.

Figure 1 Preoperative angiography showing multiple visceral artery aneurysms. (A) The superior mesenteric artery and common hepatic artery were occluded just distal to the aneurysms. The bilateral internal iliac artery and left femoral artery also had aneurysms. (B) Selective angiography of the superior mesenteric artery showed the jejunal artery aneurysm. (No. 1: the superior mesenteric artery aneurysm, No.2: the jejunal artery aneurysm, No. 3: the common hepatic artery aneurysm, No. 4: the splenic artery aneurysm, No. 6: the internal iliac artery aneurysm, No. 7: the femoral artery aneurysm)

Figure 2 Schematic view of the preoperative angiography and the operation. (A) Each aneurysm was identified preoperatively and confirmed during surgery (No. 5: aneurysm of the proper hepatic artery, which is not well visualized in the angiography in Figure 1 was found during surgery). (B) One leg of the pantaloon vein graft was anastomosed to the distal superior mesenteric artery (No. 1). The other leg was anastomosed sequentially to the conjoined jejunal artery and pancreatico-duodenal artery (No. 2). The middle colic artery was ligated. The common hepatic artery was resected (No. 3). The splenic artery was bypassed (No. 4). The proper hepatic artery was sutured laterally (No. 5). The left femoral artery was reconstructed with a prosthesis (No. 7). The internal iliac artery aneurysms were left untouched

artery aneurysm was resected and reconstructed with a prosthesis. After these reconstructions, the small intestine appeared viable. The marginal arteries and proper hepatic artery contained strong pulsations. The pul158

sation in the marginal artery of colon was visible. Therefore, reconstruction of the middle colic artery was not performed. Pathologic examination of the visceral artery aneurysms and femoral artery aneurysm revealed few CARDIOVASCULAR SURGERY

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Multiple visceral aneurysms A. Kojima et al.

smooth muscle cells in the media. The aneurysm walls consisted of fibromuscular tissue enriched with mucin, collagen fibers and elastin fibers. Adjacent areas of the arterial wall contained excessive numbers of irregularly oriented smooth muscle cells in the media, contrasting with the regular arrangement of smooth muscle cells in the intima (Figure 3). In some areas, there was a localized infiltration of lymphocytes, macrophages, and polynuclear large cells. These areas stained positive with HHF-35, indicating an overgrowth of smooth muscle cells. This was indicative of healing after destruction of the arterial wall, not arteritis. There were no atherosclerotic lesions in the aneurysms. Because there was diffuse destruction of the media, making a pathologic diagnosis was difficult. Based on the examination of the adjacent arterial segment, a diagnosis of FMD of the medial fibroplasia type was made. The postoperative complications included temporary hepatic dysfunction and persistent diarrhea. The transaminase level went down to the normal range within one week, but persistent diarrhea continued over one month. The patient was discharged without symptoms after on the 41st postoperative day.

Discussion Visceral artery aneurysms are an uncommon but important disease [1]. McNamara and Griska have reported an incidence of 1 in 12,000 in an autopsy series [2]. In a clinical review, Stanley and Whitehouse have reported an incidence of 0.1% among macroaneurysms [3]. This disease appears to be more common than previously claimed [4]. They are usually asymptomatic until rupture, although nonspecific symptoms have been reported. Routine noninvasive abdominal screening can be used to detect asymptomatic visceral artery aneurysms. Although the critical size likely to rupture has not been well defined in the visceral aneurysms, Carr et al. has recommended the operative treatment for splenic artery aneurysm greater than 2.5 cm [5]. Pregnancy may increase the rupture rate of splenic artery aneurysms [6]. Once ruptured, the mortality has been reported to be 25% for splenic artery aneurysms, 35% for hepatic artery aneurysms, and 50% for pancreaticoduodenal artery aneurysms [7]. Therefore, operative treatment is recommended for visceral artery aneurysms to prevent rupture, even if they are asymptomatic. The etiologies of multiple visceral artery aneur-

Figure 3 Pathologic examination showing changes in the media (i: intima, m: media, a: adventitia). (A) Massive destruction of the media was observed in the aneurysm wall. (B) Medial fibrodysplasia was observed in the arterial wall in the adjacent segments (Elastica van Gieson stain, original magnification ×40)

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Multiple visceral aneurysms A. Kojima et al.

ysms include atherosclerosis, dysplasia of the media, arteritis, infection, anomalies of the connective tissue, and congenital defects of the arterial wall structure [8]. FMD usually affects the renal arteries, causing renovascular hypertension in young patients. FMD causes arterial stenosis due to hyperplasia of the media or intima. However, if the elastic fibers are destroyed by the degeneration of the media, an aneurysm can form. Multiple visceral aneurysms caused by FMD are rare. Since the first report of FMD of the renal artery by Leadbetter and Burkland in 1938 [9], this disease has been classified as intimal fibroplasia, medial hyperplasia, medial fibroplasia, and perimedial dysplasia [10]. Medial dysplasia, by far the most common type of FMD, causes stenosis or aneurysm. It is characterized by the loss of medial smooth muscle cells and fragmentation of the internal and external elastic lamina. Although the etiology remains obscure, some investigators have suggested a hormonal component because of the predominance in young women [11]. Most often, the renal arteries are affected, which results in renovascular hypertension. The second most commonly affected vessels are the extracranial internal carotid arteries. Involvement of the subclavian, intestinal, iliac, and coronary arteries, as well as the aorta has been reported. Although Luscher et al. have reported a 26% incidence of multivessel involvement [12], there are few reports of multiple visceral artery aneurysms due to FMD. Jones et al. have reported the rupture of multiple hepatic artery aneurysms in a 58-yr-old woman with FMD [13]. den Butter et al. have reported a patient with heterochronic rupture of mesenteric artery and middle colic artery aneurysms [14]. Lee et al. have treated a patient with rupture of superior gluteal artery and gastroduodenal artery aneurysms [15]. In our case, the multiple visceral artery aneurysms were treated prior to rupture. Aneurysm resection without reconstruction is performed when multiple collateral channels exist which can preserve the visceral circulation. In emergencies, transcatheter coil embolization has been used for aneurysm exclusion. However, when the collateral circulation is insufficient, vascular reconstruction is mandatory. In our case, both the celiac axis and superior mesenteric artery were involved. Therefore, arterial reconstruction was performed with a vein graft. A pantaloon vein graft is useful when multiple reconstructions of the arterial branches are necessary [16]. At present, a few methods about the evaluation of perfusion viability those like doppler ultrasound,

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fluorescein and thermal imaging are reported [17], but there is no definitive method to confirm adequate blood flow to maintain normal intestinal function. Thus, we confirmed the presence of pulsations in the marginal artery by visual inspection and palpation. The prolonged postoperative diarrhea eventually resolved.

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