Annals of Vascular Surgery
Volume 24, Issue 7 , Pages 952.e13-952.e16, October 2010

Spontaneous Dissection of the Iliac Artery in a Patient With Fibromuscular Dysplasia

Department of Surgery, Kurume University School of Medicine, Kurume, Japan

published online 05 July 2010.

Article Outline

A 49-year-old man was referred to our hospital at 10 days after the onset of sudden intermittent claudication of the right lower limb and with right lower abdominal pain. Diagnosis by computed tomography scan and pelvic angiography was dissection from the common iliac artery to peripheral external iliac artery with thrombosed false lumen. Replacement of the common and external iliac artery was performed using a 10-mm Dacron prosthesis. A pathological examination of the right common iliac artery revealed an arterial disorder caused by fibromuscular dysplasia.

 

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Introduction 

Spontaneous dissection of the peripheral arteries without involvement of the aorta is uncommon and can be associated with pregnancy and hormonal intake, or can be seen in younger men with history of hypertension.1 Other rare conditions such as fibromuscular dysplasia (FMD),2 the Marfan syndrome,3 cystic medial necrosis4 have been associated with spontaneous dissection. FMD (disease) was described by Leadbetter and Burkland5 in 1938 and has long been considered specific to the renal arteries. Several extrarenal sites, including the abdominal, cervicocephalic, iliac, femoral, and popliteal arteries, have been reported since 1964.6, 7, 8, 9, 10, 11 FMD involves, less frequently, the iliac arteries. Affected arteries tend to be occlusive primarily, and secondary events include aneurysm, dissection, and arteriovenous fistula. FMD involving the iliac arteries are associated with lower limb ischemia from dissection is extremely rare.2, 12, 13, 14, 15 We report a rare patient, to our knowledge, with previously undiagnosed FMD who required surgery because of spontaneous dissection of the iliac artery (SDI).

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Case Reports 

A 49-year-old man without history of trauma and complicate atherosclerotic risk factors was referred to our hospital at 10 days after the onset of sudden intermittent claudication of the right lower limb and with right lower abdominal pain. The patient had no history of arterial hypertension and smoking and other risk factors for atherosclerosis, nor was he an athlete. There had been no previous surgical interventions. On admission, his physical examination showed a blood pressure of 110/70 mm Hg on both arms and regular pulse rate of 70 beats per minute. Routine laboratory tests, including blood chemistry, liver function test, C-reactive protein, and bleeding time, were normal. All pulses of the right leg were weaker than those of the left leg and a low-frequency bruit was noticed at the site of right iliac artery. The ankle-brachial index was a significant lower value on the right leg (0.83) than that on the left leg (1.06). A contrast-enhanced computed tomography scan of the pelvis showed an intimal flap involving the right common iliac artery (CIA) (Fig. 1A) and extending into the external iliac artery (Fig. 1B), which was consistent with iliac dissection. We performed angiography above the suprarenal abdominal aorta. The patient had no vascular abnormalities such as stenosis in the abdominal aorta and the renal arteries. Pelvic angiography (Fig. 2) showed a pseudoaneurysm in the bifurcation site of the right internal iliac artery and external iliac artery, and long segment stenosis of right external iliac artery. Diagnosis of computed tomography scan and pelvic angiography was dissection from the CIA to peripheral external iliac artery with thrombosed false lumen. The true lumen of the right external iliac artery was almost completely compressed by the false lumen that was thrombosed at the distal portion. The patient underwent planed operation because of younger age and stable situation of right limb ischemia. Replacement of the common and external iliac artery was performed using a 10-mm Dacron prosthesis through a right pararectal retroperitoneal approach (a right lumbotomy incision). The entry site of the CIA and thrombosed false lumen in the external iliac artery could be found (Fig. 3A). A pathological examination of the right CIA revealed an arterial disorder caused by FMD (Fig. 3B). The postoperative recovery was uneventful and the patient was discharged 10 days after the operation; at present he is doing well.

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  • Fig. 1 

    Contrast-enhanced computed tomography scan on admission. A Shows an intimal flap involving the right common iliac artery. B Shows thrombosed false lumen extending into the external iliac artery.

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  • Fig. 2 

    Digital subtraction angiogram. Dissection associated with thrombosis of the right external iliac artery. Digital subtraction angiogram of the pelvis showed entry site like a pseudoaneurysm in the common iliac artery of the bifurcation site, and long segment stenosis of the right external iliac artery caused by compression of thrombosed false lumen.

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  • Fig. 3 

    A Intraoperative findings. The proximal right external iliac artery with dissection was cut off. True lumen (left side: black arrow) and false lumen (right side: white arrow) of the external iliac artery are indicated. B Pathological findings. Fibromuscular dysplasia of media associated with dissection.

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Discussion 

SDI is very rare. Traumatic dissections from blunt high-energy injuries are well understood, as well as those in connection with collagen diseases.16, 17 SDI has been reported in connection with FMD,2, 12, 13, 14, 15 pregnancy,1 and alpha-1-antitrypsin deficiency.18 As another cause, Cook et al. reported acute dissection of the external iliac artery during competition in highly trained endurance athletes.19 Our patient suffered from a dissection of the iliac artery, but he had no history of arterial hypertension and smoking and other risk factors for atherosclerosis and high-energy trauma, nor was he an athlete. Finally, the etiology of our case has led to the diagnosis of FMD because of a pathological examination.

FMD is a non-atherosclerotic, non-inflammatory vascular disease and, though a variety of genetic, mechanical, and hormonal factors have been proposed, the etiology of FMD remains uncertain. Although FMD most commonly affects the renal artery and internal carotid arteries, any artery throughout the body can be affected.20 Most FMD cases are considered to be asymptomatic. However, an ischemic condition can arise in a distal area of an arterial disorder, depending on the region. The cause of renovascular hypertension is well known, and is demonstrated by angiography as a string-of-beads, which is a result of stenosis in the artery. Furthermore, secondary aneurysm, dissection, and rupture can develop.

Most patients with stenotic lesions secondary to iliac artery FMD without dissections have no symptoms, whereas most patients with dissection had symptoms associated with acute or chronic ischemia. Our patient had right lower abdominal pain initially, and thereafter he presented with intermittent claudication. Right lower abdominal pain may be explained as having caused by the onset of dissection in the iliac artery.

Eleven cases of iliac arterial dissections with FMD have been previously reported (Table I). Burri et al.12 in 1983 first reported a patient with dissection of the iliac artery secondary to FMD. Since then 12 cases, including the present case, with dissection of the iliac arteries associated with FMD have been reported2, 12, 13, 14, 15 (Table I). Twelve cases were encountered in seven men and five women whose ages ranged from 21 to 56 years (mean: 42.5 years). Eleven of all 12 cases had the pain from lower abdomen to the inguinal region or claudication of the limbs or hips, and one patient presented with shock because of rupture. The site of dissection was in the external iliac artery in seven patients, in the common and external iliac artery in four patients, and in the aortoiliofemoral region in one patient. Three patients had evidences of ruptures, which were two common iliac cases and one external iliac case. Those results may indicate that risk for rupture is greater in the patient with dissection of CIA than in the patient with dissection of the external iliac artery. The dissection was on the right side in six patients, on the left side in five patients, and on both sides in one patient. Regarding surgical intervention, anatomic bypass or interposition of the diseased artery with an autogenously vein or prosthetic graft was performed in nine patients involving two bilateral bypasses, thromboendarterectomy in one patient, extra anatomical bypass in one patient, and conservative therapy in one patient. It was generally appropriate judgment that the surgical intervention such as anatomical bypass was commonly performed, because dissection of the iliac artery associated with FMD was caused in relative younger patients, who presented with lower abdominal pain and lower limb ischemia.

Table I. Previous reports and present case according to patients with iliac artery dissection associated with fibromuscular dysplasia
AuthorAgeSexPresentationSite of dissectionTreatmentOutcome
Burri et al.45FLeft inguinal painLeft external iliac arteryIliofemoral bypassAlive
Sauer et al.56FHip and thigh claudicationRight common iliac arteryConservative therapyAlive
Patel et al.39MLeft inguinal painLeft external iliac arteryIliofemoral bypassAlive
Thevenet et al.45FLower limb ischemiaRight external iliac arteryAortobifemoral bypassAlive
51MIntermittent claudicationBil. aortoiliofemoral arteryBil. Iliofemoral bypassAlive
47FAbdominal painRight external iliac arteryIliac bypassAlive
29FIliac fossa painLeft external iliac arteryInterposition with veinAlive
53MIntermittent claudicationRight external iliac arteryIliofemoral bypassAlive
Luck et al.45MIntermittent claudicationRight external iliac arteryThromboendarterectomyAlive
Honjo et al.2130MShockLeft common iliac arteryIliofemoral bypassDead
Yoshioka et al.2221MAbdominal painLeft common iliac arteryAxillobifemoral bypassDead
Present case49MLower abdominal pain Intermittent claudicationRight common iliac arteryReplacement of common and external iliac arteryAlive

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Conclusions 

We experienced a rare patient with spontaneous acute dissection of the iliac artery associated with FMD. FMD may be considered the cause of SDI in a patient with symptoms who is of younger age, without history of trauma and complicate atherosclerotic risk factors, and surgical repair is mandatory.

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References 

  1. Nolte JE, Rutherford RB, Nawaz S, Rosenberger A, Speers WC, Krupski WC. Arterial dissections associated with pregnancy. J Vasc Surg. 1995;21:515–520
  2. Lück I, Hanschke D, Geissler C, Gruss JD. Spontaneous dissection of the external iliac artery due to fibromuscular dysplasia. Vasa. 2002;31:115–121
  3. Barker SG, Burnand KG. Retrograde iliac artery dissection in Marfan's syndrome: a case report. J Cardiovasc Surg. 1989;30:953–954
  4. Sogaro F, Toffon A, Galeazzi E, Ganassin L. Spontaneous limited dissection of the external iliac artery: case report after 14 year follow-up. Vasa. 1994;23:370–372
  5. Leadbetter WF, Burkland CE. Hypertension in unilateral renal disease. J Urol. 1938;39:611
  6. Palubinskas AJ, Perloff D, Newton TH. Fibromuscular hyperplasia in extrarenal arteries. Radiology. 1964;82:451–455
  7. Palubinskas AJ, Perloff D, Newton TH. Fibromuscular hyperplasia: an arterial dysplasia of increasing clinical importance. Am J Roentgenol. 1966;98:907–913
  8. Twigg HL, Palmisano PJ. Fibromuscular hyperplasia of the iliac artery. Am J Roentgenol. 1965;95:418–423
  9. Wylie EJ, Binkley FM, Palubinskas AJ. Extrarenal fibromuscular hyperplasia. Am J Surg. 1966;112:149–154
  10. Thevenet A, Mary H. Dysplasies et dissections des art ères iliaques externes. In: Actualités Chirurgicales. Paris: Masson; 1977;p. 17–19
  11. Iwai T, Konno S, Hiejima K, et al. Fibromuscular dysplasia in the extremities. J Cardiovasc Surg. 1985;26:495–501
  12. Burri B, Fontolliet C, Ruegsegger CH, Mosimann R. External iliac artery dissection due to fibromuscular dysplasia. Vasa. 1983;12:76–78
  13. Sauer L, Reilly LM, Goldstone J, Ehrenfeld WK, Hutton JE, Stoney RJ. Clinical spectrum of symptomatic external iliac fibromuscular dysplasia. J Vasc Surg. 1990;12:488–496
  14. Patel KS, Wolfe JH, Mathias C. Left external iliac artery dissection and bilateral renal artery aneurysms secondary to fibromuscular dysplasia: a case report. Neth J Surg. 1990;42:118–120
  15. Thevenet A, Latil JL, Albat B. Fibromuscular disease of the external iliac artery. Ann Vasc Surg. 1992;6:199–204
  16. Lyden SP, Sristava S, Waldman D, Green RM. Common iliac artery dissection after blunt trauma: a case report of endovascular repair and literature review. J Trauma. 2001;50:339–342
  17. Siavelis HA, Mansour M. Aortoiliac dissection after blunt abdominal trauma: case report. J Trauma. 1997;43:862–864
  18. Cattan S, Mariette X, Labrousse F, et al. Iliac artery dissection in alpha-1-antitrypsin deficiency. Lancet. 1994;343:1371–1372
  19. Cook PS, Erdoes L, Selzer P, et al. Dissection of the external artery in highly trained athletes. J Vasc Surg. 1995;22:173–177
  20. Declemy S, Kreitmann P, Popoff G, et al. Spontaneous dissecting aneurysm of the common iliac artery. Ann Vasc Surg. 1991;5:549–551
  21. Honjo O, Yamada Y, Kuroko Y, Kushida Y, Une D, Hioki K. Spontaneous dissection and rupture of common iliac artery in a patient with fibromuscular displasia: A case report and review of the literature on iliac artery dissections secondary to fibromuscular dysplasia. J Vasc Surg. 2004;40:1032–1036
  22. Yoshioka Y, Arichi N, Tokugawa S, Kishikawa H, Nishimura K, Ishikawa Y. Spontaneous rupture of common iliac artery associated with fibromuscular dysplasia presenting with colic pain suggestive of ureternal stone. Acta Urol Jpn. 2007;53:721–724

PII: S0890-5096(10)00145-7

doi:10.1016/j.avsg.2010.02.047

Annals of Vascular Surgery
Volume 24, Issue 7 , Pages 952.e13-952.e16, October 2010

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