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Endovascular therapy for limb salvage in a case of critical lower limb ischemia resulting from fibromuscular dysplasia
Endovascular therapy for limb salvage in a case of critical lower limb ischemia resulting from fibromuscular dysplasia Osamu Iida, MD, Shinsuke Nanto, MD, PhD, Masaaki Uematsu, MD, PhD, Takakazu Morozumi, MD, Hirokuni Akahori, MD, and Seiki Nagata, MD, PhD, Amagasaki, Hyogo, Japan Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that mostly affects the renal and internal carotid arteries, rarely complicating lower limbs. We report a case of FMD complicating critical limb ischemia due to the obstruction of below the knee arteries, diagnosed and treated with the support of skin perfusion pressure (SPP) measurement. Initial angiogram revealed the anterior tibial artery (ATA) was subtotally occluded showing a “strings of beads” pattern, which is typical of the arteries affected by FMD. SPP guided endovascular therapy was successful for ATA lesions. Intractable rest pain subsequently disappeared and the ulcer was completely healed without amputation. ( J Vasc Surg 2007;46:803-7.)
Fibromuscular dysplasia (FMD) is a nonatherosclerotic and noninflammatory systemic vascular disease of unknown origin that frequently involves renal arteries, and less frequently, the carotid, vertebral, subclavian, mesenteric, coronary, and iliac arteries.1-3 Symptoms such as headache, abdominal angina, and limb ischemia are caused by the occlusion, stenosis, aneurysm, rupture, and dissection of these arteries due to fibrous proliferation and smooth muscle cell dysplasia.4-9 In general, either surgical bypass grafting10-12 or endovascular therapy13,14 are currently chosen for the treatment of critical limb ischemia (CLI) due to advanced atherosclerosis. Although surgical bypass using vein grafts claims good long-term patency and clinical durability,15,16 an adequate vein is often difficult to find and the subsequent complication rate is higher than that of endovascular therapy.17 Currently, endovascular therapy has become widely applied in patients with CLI. In fact, surgical bypass and balloon angioplasty have similar outcomes in terms of amputation and survival rate in a long-term follow-up of 5.5 years.18 On the other hand, FMD has the distinct pathology from atherosclerosis, and CLI in FMD is rare.1 To our knowledge, there has been no report that describes CLI resulting from FMD of the tibial arteries. We report here our experience of successful endovascular therapy in a case of critical lower limb ischemia resulting from FMD of the tibial arteries. Skin perfurion pressure measurement was useful in evaluating the lesion below the knee.
From the Cardiovascular Division, Kansai Rosai Hospital. Competition of interest: none. Reprint requests: Osamu Iida, MD, Cardiovascular Division, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, Hyogo 660-8511, Japan (e-mail: [email protected]). 0741-5214/$32.00 Copyright © 2007 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2007.05.037
CASE REPORT A 29-year-old female was referred to our hospital complaining of intractable pain at rest and nonhealing ulcer in the left foot. She had a history of cholelithiasis at the age of 25 years. She suffered from subarachnoid hemorrhage at 28 years. The diagnosis of FMD was made by the biopsy of the superficial temporal artery at that time. She had no atherosclerotic risk factors such as hypertension, hyperlipidemia, smoking, and family history. HbA1c was 5.2% and fasting blood sugar was 82 mg/dl, indicating no evidence of diabetes mellitus. She had no history of other collagen disease, Burger disease, or Bechet disease, which were also excluded by physical and laboratory examinations. White blood cell count was 5500/mm3. Eosinophil cell count was not elevated, suggesting the acute toe syndrome was unlikely. C-reactive protein was slightly elevated to 0.7, probably due to the nonhealing ulcer. Although sedimentation rate was 47 mm/hour, and antinuclear antibody titer was 1:40, all other serologic tests (rheumatoid factor, p-ANCA, c-ANCA, DNA antibody, and circulating immune complex) were negative. Echocardiograms were all normal, and no history of paroxysmal atrial fibrillation had been documented. Her legs had revealed nonhealing ulcer of Rutherford classification 5 (Fig 1, a) for the past 3 months. Ankle brachial index was normal (left, 1.16; right, 1.12). However, pulsed-wave Doppler recordings showed decreased velocity in the dorsalis pedis, and the posterior tibial arteries of the left foot, indicating significant obstruction in the arteries below the knee. The index skin perfusion pressure (SPP) in the dorsal region was 27 mm Hg, and that in the plantar region was 19 mm Hg, both of which were not sufficient for the ulcer to be healed (Fig 1, b and c). Digital subtraction angiogram showed the anterior tibial artery was subtotally occluded, and the posterior tibial artery was totally occluded. The lesions revealed a “strings of beads” pattern, which is typical of the arteries affected by FMD (Fig 3, b). The arteries above the knee showed normal contour without plaques (Fig 2, a). Systemic arteries, such as the carotid, the vertebral, the subclavian, and the renal arteries, which are commonly involved in FMD, were angiographically normal, except for the superior mes-
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Fig 1. Conditions of ulcer and measurements of skin perfusion pressure (SPP) before and after the endovascular therapy. Nonhealing ulcer with intractable rest pain presented in the left foot before the procedure (a). SPP stood at 27 mm Hg in the dorsal region (b) and 19 mm Hg in the plantar region (c). These indicated the flow was not sufficient for the ulcer to be healed. The ulcer was completely healed 2 weeks after the endovascular therapy (d). Amputation was unnecessary. SPP significantly improved to 56 mm Hg in the dorsal region (e) and to 55 mm Hg in the plantar region (f), which were sufficient (⬎45 mm Hg) for the ulcer to be healed.
enteric artery, which also revealed a “strings of beads” pattern (Fig 2, b). Hence, we suspect the nonhealing ulcer accompanying intractable rest pain due to the occlusion of the anterior tibial artery was caused by FMD. Based on these angiograms, surgical bypass was initially considered, but was abandoned because the patient feared possible
complications and morbidities. We, therefore, offered her a low invasive approach using an endovascular technique. Before procedure, 3000 unit heparin was intra-arterially given and balloon angioplasty was performed from the left femoral artery using a 5F sheath (Terumo) with antegrade approach. A 0.014 inch guidewire (Syn CHRO-14S, Boston Scientific, Natick, Mass) was pre-
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Fig 2. CT angiogram of the aorta-iliac-femoro-popliteal arteries (a), the angiogram (b) and the pathology of the mesenteric artery (c and d). The aortoiliac, femoral, and popliteal arteries (above the knee) were all normal by CT angiogram without any embolic sources (a). Angiogram of the superior mesenteric artery revealed “string of beads” pattern, which is typical to FMD (b). In the mesenteric arteries of the small intestine, walls were irregularly thickened without any inflammatory changes. Accumulation of subendothelial mesenchymal cells was noted in the fibrous connective tissue. This fibrous connective tissue was sometimes loose, and at times, myxoid change was observed (c) (hematoxylin-eosin stain, 20⫻). Elastic lamina, highlighted by Verhoeff-van Gieson stain (d) (20⫻), was occasionally dissociated.
ceded to a 2⫻100 mm balloon catheter (Savvy, Cordis, Warren, NJ) into the anterior tibial artery (ATA), which was responsible for the nonhealing ulcer. We gradually advanced the balloon catheter little by little (approximately 1 to 2 cm at a time) by repeating inflation and deflation and subsequently dilated the balloon at 12 atm for 3 minutes (Fig 3, c and d). The subtotal occlusive lesion in
the ATA was successfully recanalized from its origin to the pedal arch (Fig 3, e). After the endovascular therapy, the rest pain disappeared within a day. The ulcer was completely healed within the next 2 weeks, saving her form amputation (Fig 1, d). SPP significantly improved to 56 mm Hg in the dorsal region and 55 mm Hg in the
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Fig 3. Angiographic findings of the tibial arteries before and after the endovascular therapy. Angiogram of the contralateral tibial arteries in the right leg also demonstrated “string of beads” (a). Baseline angiogram of the left tibial arteries revealed a subtotal occlusion in the distal anterior tibial artery (ATA) and a total occlusion in tibioperoneal artery with collaterals (b). These lesions showed “strings of beads” pattern, which is typical of fibromuscular dysplasia. After balloon angioplasty (c and d), sufficient blood flow was restored from the ATA to the dorsalis pedis artery (e). plantar region, both of which were sufficient (⬎45 mm Hg) for the ulcer to be healed (Fig 1, e and f). Follow-up duplex scan 12 months later showed normal flow velocities in the ATA. The patient suffered from the rupture of the jejunum afterwards, and surgical repair was mandatory. Specimen of the arteries of the jejunum was obtained and demonstrated the typical pathology compatible with FMD (Fig 2, c and d).
DISCUSSION Here we report our successful experience of endovascular therapy in a young female case of critical lower limb ischemia resulting from FMD of the tibial arteries, suffering from nonhealing foot ulcer with intractable rest pain. In this case of FMD, the arteries below the knee was severely
obstructed, which is rarely seen in FMD. The ulcer and pain were resistant to medical treatment, and initial SPP was not sufficient for the ulcer to be healed. The patient was successfully treated with endovascular therapy with the support of SPP measurements. Intractable rest pain immediately disappeared following the angioplasty, and the ulcer was healed within the next 2 weeks. No complication occurred, and the amputation of the limb was unnecessary. To our knowledge, this is the first report on the successful endovascular treatment of the lesions of FMD involving below the knee arteries with the support of skin perfusion pressure measurement. FMD is a rare multifocal vasculopathy of unknown etiology, typically involving the medium to large arteries
JOURNAL OF VASCULAR SURGERY Volume 46, Number 4
that branch from the aorta, predominantly affecting young women.1 The renal arteries are the most commonly involved (60% to 70%), followed by the carotid arteries (20% to 30%), the visceral arteries (9%), and the arteries of the extremities are rarely involved (5%).1 Among the arteries of the lower limbs, the iliac artery is the most common part of the arteries involved.7 Earlier studies have demonstrated that both a high grade focal stenosis and a “strings of beads-like” lesion due to FMD can be easily dilated by balloon angioplasty, with very low incidence of complications.19 To date, however, surgical bypass with venous grafts is the most durable revascularization technique in patients who have below the knee lesions of CLI.18 Due to the long stenotic lesion in the ATA in the patient, surgical bypass was initially considered. In this case, however, angioplasty was preferred by the patient. Recently, endovascular therapy has been widely applied to patients with CLI. In fact, Bypass Versus Angioplasty in Severe Ischemia of the Leg (BASIL) trial demonstrated comparable rates of amputation and survival in patients underwent endovascular therapy to those underwent surgery for 5 years, although the conclusions drawn from BASIL trial cannot be extrapolated to this rare case of FMD. SPP is a novel index of tissue microcirculation that utilizes laser Doppler flowmetry. It was originally introduced to predict the prognosis of patients with critical limb ischemia. If SPP is grater than 30 mm Hg, 80% of patients will eventually be healed.20 In this case report, SPP was improved from 19 mm Hg to 55 mm Hg after endovascular therapy; the ulcer was completely healed 2 weeks later. Thus, SPP measurements were useful in the management of the patient. The diagnosis of FMD is usually established by the combination of typical angiographical and pathological changes. We could not obtain histology samples from the tibial artery in this case because amputation was unnecessary. Nonetheless, FMD was pathologically diagnosed previously in the specimen from the temporal artery, and also in the specimen from the mesenteric arteries. Vasculitis of other causes was unlikely from the clinical picture. In addition, the typical angiographic pattern of “strings of beads” strongly supports the diagnosis. Although conventional balloon angioplasty without stenting appeared successful in this patient, close follow-up care is prudent for possible restenosis. REFERENCES 1. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med 2004;350: 1862-71. 2. Stanly JC, Wakefield TW. Arterial fibrodysplasia. In: Rutherford RB, editor. Vascular surgery. 5th ed. Philadelphia (PA): Saunders; 2000. p. 387-408.
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3. Luscher TF, Lie JT, Stanson AW, Houser OW Hollier LH, Sheps SG. Arterial fibromuscular dysplasia. Mayo Clin Proc 1987;62:931-52. 4. Topaz O, Warner M, Lanter P, Soffer A, Burns C, DiSciascio G, et al. Isolated significant left main coronary artery stenosis: angiographic, hemodynamic, and clinical findings in 16 patients. Am Heart J 1991; 122:1308-14. 5. Myers SI, Clagett GP, Valentine RJ, Hansen ME, Anand A, Chervu A. Chronic intestinal ischemia caused by intravenous cocaine use: report of two cases and review of the literature. J Vasc Surg 1996;23:724-9. 6. Edwards JM, Antonius JI, Porter JM. Critical hand ischemia caused by forearm fibromuscular dysplasia. J Vasc Surg 1985;2:459-63. 7. Sauer L, Reilly LM, Goldstone J, Ehrenfeld WK, Hutton JE, Stoney RJ. Clinical spectrum of symptomatic external iliac fibromuscular dysplasia. J Vasc Surg 1990;12:488-96. 8. Reilly LM, Cunningham CG, Maggisano R, Ehrenfeld WK, Stoney RJ. The role of arterial reconstruction in spontaneous renal artery dissection. J Vasc Surg 1991;14:4687. 9. 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 dysplasia: a case report and review of the literature on iliac artery dissections secondary to fibromuscular dysplasia. J Vasc Surg 2004;40:1032-6. 10. Berceli SA, Chan AK, Pomposelli FB, Gibbons GW, Campbell DR, Akbari CM, et al. Efficacy of dorsal pedal artery bypass in limb salvage for ischemic heel ulcers. J Vasc Surg 1999;30:499-508. 11. Pomposelli FB, Kansal N, Hamdan AD, Belfield A, Sheahan M, Campbell DR, et al. decade of experience with dorsalis pedis artery bypass: analysis of outcome in more than 1000 cases. J Vasc Surg 2003;37: 307-15. 12. Hughes K, Domenig CM, Hamdan AD, Schermerhorn M, Aulivola B, Blattman S, et al. Bypass to plantar and tarsal arteries: an acceptable approach to limb salvage. J Vasc Surg 2004;40:1149-57. 13. Dorros G, Jaff MR, Dorros AM, Mathiak LM, He T. Tibioperoneal (outflow lesion) angioplasty can be used as primary treatment in 235 patients with critical limb ischemia: five-year follow-up. Circulation 2001;104:2057-62. 14. Feiring AJ, Wesolowski AA, Lade S. Primary stent-supported angioplasty for treatment of below-knee critical limb ischemia and severe claudication: early and one-year outcomes. J Am Coll Cardiol 2004;44: 2307-14. 15. Nicoloff AD, Taylor LM, McLafferty RB, Moneta GL, Porter JM. Patient recovery after infrainguinal bypass grafting for limb salvage. J Vasc Surg 1998;27:256-63. 16. Gentile AT, Lee RW, Moneta GL, Taylor LM, Edwards JM, Porter JM. Results of bypass to the popliteal and tibial arteries with alternative sources of autogenous vein. J Vasc Surg 1996;23:272-79. 17. Goshima KR, Mills JL Sr, Hughes JD. A new look at outcomes after infrainguinal bypass surgery: traditional reporting standards systematically underestimate the expenditure of effort required to attain limb salvage. J Vasc Surg 2004;39:330-5. 18. Adam DJ, Beard JD, Cleveland T, Bell J, Bradbury AW, Forbes JF, et al. Bypass versus angioplasty in severe ischemia of the leg (BASIL): multicenter, randomized controlled trial. Lancet 2005;366:1925-34. 19. Yoshida T, Ohashi I, Suzuki S, Iwai T. Fibromuscular disease of the brachial artery with digital emboli treated effectively by transluminal angioplasty. Cardiovasc Intervent Radiol 1994;17:99-101. 20. Castronuovo JJ Jr, Adera HM, Smiell JM, Price RM. Skin perfusion pressure measurement is valuable in the diagnosis of critical limb ischemia. J Vasc Surg. 1997;26:629-37.
Submitted Feb 21, 2007; accepted May 14, 2007.
Fibromuscular dysplasia (FMD) is a nonatherosclerotic and noninflammatory systemic vascular disease of unknown origin that frequently involves renal arteries, and less frequently, the carotid, vertebral, subclavian, mesenteric, coronary, and iliac arteries.1-3 Symptoms such as headache, abdominal angina, and limb ischemia are caused by the occlusion, stenosis, aneurysm, rupture, and dissection of these arteries due to fibrous proliferation and smooth muscle cell dysplasia.4-9 In general, either surgical bypass grafting10-12 or endovascular therapy13,14 are currently chosen for the treatment of critical limb ischemia (CLI) due to advanced atherosclerosis. Although surgical bypass using vein grafts claims good long-term patency and clinical durability,15,16 an adequate vein is often difficult to find and the subsequent complication rate is higher than that of endovascular therapy.17 Currently, endovascular therapy has become widely applied in patients with CLI. In fact, surgical bypass and balloon angioplasty have similar outcomes in terms of amputation and survival rate in a long-term follow-up of 5.5 years.18 On the other hand, FMD has the distinct pathology from atherosclerosis, and CLI in FMD is rare.1 To our knowledge, there has been no report that describes CLI resulting from FMD of the tibial arteries. We report here our experience of successful endovascular therapy in a case of critical lower limb ischemia resulting from FMD of the tibial arteries. Skin perfurion pressure measurement was useful in evaluating the lesion below the knee.
From the Cardiovascular Division, Kansai Rosai Hospital. Competition of interest: none. Reprint requests: Osamu Iida, MD, Cardiovascular Division, Kansai Rosai Hospital, 3-1-69 Inabaso, Amagasaki, Hyogo 660-8511, Japan (e-mail: [email protected]). 0741-5214/$32.00 Copyright © 2007 by The Society for Vascular Surgery. doi:10.1016/j.jvs.2007.05.037
CASE REPORT A 29-year-old female was referred to our hospital complaining of intractable pain at rest and nonhealing ulcer in the left foot. She had a history of cholelithiasis at the age of 25 years. She suffered from subarachnoid hemorrhage at 28 years. The diagnosis of FMD was made by the biopsy of the superficial temporal artery at that time. She had no atherosclerotic risk factors such as hypertension, hyperlipidemia, smoking, and family history. HbA1c was 5.2% and fasting blood sugar was 82 mg/dl, indicating no evidence of diabetes mellitus. She had no history of other collagen disease, Burger disease, or Bechet disease, which were also excluded by physical and laboratory examinations. White blood cell count was 5500/mm3. Eosinophil cell count was not elevated, suggesting the acute toe syndrome was unlikely. C-reactive protein was slightly elevated to 0.7, probably due to the nonhealing ulcer. Although sedimentation rate was 47 mm/hour, and antinuclear antibody titer was 1:40, all other serologic tests (rheumatoid factor, p-ANCA, c-ANCA, DNA antibody, and circulating immune complex) were negative. Echocardiograms were all normal, and no history of paroxysmal atrial fibrillation had been documented. Her legs had revealed nonhealing ulcer of Rutherford classification 5 (Fig 1, a) for the past 3 months. Ankle brachial index was normal (left, 1.16; right, 1.12). However, pulsed-wave Doppler recordings showed decreased velocity in the dorsalis pedis, and the posterior tibial arteries of the left foot, indicating significant obstruction in the arteries below the knee. The index skin perfusion pressure (SPP) in the dorsal region was 27 mm Hg, and that in the plantar region was 19 mm Hg, both of which were not sufficient for the ulcer to be healed (Fig 1, b and c). Digital subtraction angiogram showed the anterior tibial artery was subtotally occluded, and the posterior tibial artery was totally occluded. The lesions revealed a “strings of beads” pattern, which is typical of the arteries affected by FMD (Fig 3, b). The arteries above the knee showed normal contour without plaques (Fig 2, a). Systemic arteries, such as the carotid, the vertebral, the subclavian, and the renal arteries, which are commonly involved in FMD, were angiographically normal, except for the superior mes-
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804 Iida et al
JOURNAL OF VASCULAR SURGERY October 2007
Fig 1. Conditions of ulcer and measurements of skin perfusion pressure (SPP) before and after the endovascular therapy. Nonhealing ulcer with intractable rest pain presented in the left foot before the procedure (a). SPP stood at 27 mm Hg in the dorsal region (b) and 19 mm Hg in the plantar region (c). These indicated the flow was not sufficient for the ulcer to be healed. The ulcer was completely healed 2 weeks after the endovascular therapy (d). Amputation was unnecessary. SPP significantly improved to 56 mm Hg in the dorsal region (e) and to 55 mm Hg in the plantar region (f), which were sufficient (⬎45 mm Hg) for the ulcer to be healed.
enteric artery, which also revealed a “strings of beads” pattern (Fig 2, b). Hence, we suspect the nonhealing ulcer accompanying intractable rest pain due to the occlusion of the anterior tibial artery was caused by FMD. Based on these angiograms, surgical bypass was initially considered, but was abandoned because the patient feared possible
complications and morbidities. We, therefore, offered her a low invasive approach using an endovascular technique. Before procedure, 3000 unit heparin was intra-arterially given and balloon angioplasty was performed from the left femoral artery using a 5F sheath (Terumo) with antegrade approach. A 0.014 inch guidewire (Syn CHRO-14S, Boston Scientific, Natick, Mass) was pre-
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Iida et al 805
Fig 2. CT angiogram of the aorta-iliac-femoro-popliteal arteries (a), the angiogram (b) and the pathology of the mesenteric artery (c and d). The aortoiliac, femoral, and popliteal arteries (above the knee) were all normal by CT angiogram without any embolic sources (a). Angiogram of the superior mesenteric artery revealed “string of beads” pattern, which is typical to FMD (b). In the mesenteric arteries of the small intestine, walls were irregularly thickened without any inflammatory changes. Accumulation of subendothelial mesenchymal cells was noted in the fibrous connective tissue. This fibrous connective tissue was sometimes loose, and at times, myxoid change was observed (c) (hematoxylin-eosin stain, 20⫻). Elastic lamina, highlighted by Verhoeff-van Gieson stain (d) (20⫻), was occasionally dissociated.
ceded to a 2⫻100 mm balloon catheter (Savvy, Cordis, Warren, NJ) into the anterior tibial artery (ATA), which was responsible for the nonhealing ulcer. We gradually advanced the balloon catheter little by little (approximately 1 to 2 cm at a time) by repeating inflation and deflation and subsequently dilated the balloon at 12 atm for 3 minutes (Fig 3, c and d). The subtotal occlusive lesion in
the ATA was successfully recanalized from its origin to the pedal arch (Fig 3, e). After the endovascular therapy, the rest pain disappeared within a day. The ulcer was completely healed within the next 2 weeks, saving her form amputation (Fig 1, d). SPP significantly improved to 56 mm Hg in the dorsal region and 55 mm Hg in the
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JOURNAL OF VASCULAR SURGERY October 2007
Fig 3. Angiographic findings of the tibial arteries before and after the endovascular therapy. Angiogram of the contralateral tibial arteries in the right leg also demonstrated “string of beads” (a). Baseline angiogram of the left tibial arteries revealed a subtotal occlusion in the distal anterior tibial artery (ATA) and a total occlusion in tibioperoneal artery with collaterals (b). These lesions showed “strings of beads” pattern, which is typical of fibromuscular dysplasia. After balloon angioplasty (c and d), sufficient blood flow was restored from the ATA to the dorsalis pedis artery (e). plantar region, both of which were sufficient (⬎45 mm Hg) for the ulcer to be healed (Fig 1, e and f). Follow-up duplex scan 12 months later showed normal flow velocities in the ATA. The patient suffered from the rupture of the jejunum afterwards, and surgical repair was mandatory. Specimen of the arteries of the jejunum was obtained and demonstrated the typical pathology compatible with FMD (Fig 2, c and d).
DISCUSSION Here we report our successful experience of endovascular therapy in a young female case of critical lower limb ischemia resulting from FMD of the tibial arteries, suffering from nonhealing foot ulcer with intractable rest pain. In this case of FMD, the arteries below the knee was severely
obstructed, which is rarely seen in FMD. The ulcer and pain were resistant to medical treatment, and initial SPP was not sufficient for the ulcer to be healed. The patient was successfully treated with endovascular therapy with the support of SPP measurements. Intractable rest pain immediately disappeared following the angioplasty, and the ulcer was healed within the next 2 weeks. No complication occurred, and the amputation of the limb was unnecessary. To our knowledge, this is the first report on the successful endovascular treatment of the lesions of FMD involving below the knee arteries with the support of skin perfusion pressure measurement. FMD is a rare multifocal vasculopathy of unknown etiology, typically involving the medium to large arteries
JOURNAL OF VASCULAR SURGERY Volume 46, Number 4
that branch from the aorta, predominantly affecting young women.1 The renal arteries are the most commonly involved (60% to 70%), followed by the carotid arteries (20% to 30%), the visceral arteries (9%), and the arteries of the extremities are rarely involved (5%).1 Among the arteries of the lower limbs, the iliac artery is the most common part of the arteries involved.7 Earlier studies have demonstrated that both a high grade focal stenosis and a “strings of beads-like” lesion due to FMD can be easily dilated by balloon angioplasty, with very low incidence of complications.19 To date, however, surgical bypass with venous grafts is the most durable revascularization technique in patients who have below the knee lesions of CLI.18 Due to the long stenotic lesion in the ATA in the patient, surgical bypass was initially considered. In this case, however, angioplasty was preferred by the patient. Recently, endovascular therapy has been widely applied to patients with CLI. In fact, Bypass Versus Angioplasty in Severe Ischemia of the Leg (BASIL) trial demonstrated comparable rates of amputation and survival in patients underwent endovascular therapy to those underwent surgery for 5 years, although the conclusions drawn from BASIL trial cannot be extrapolated to this rare case of FMD. SPP is a novel index of tissue microcirculation that utilizes laser Doppler flowmetry. It was originally introduced to predict the prognosis of patients with critical limb ischemia. If SPP is grater than 30 mm Hg, 80% of patients will eventually be healed.20 In this case report, SPP was improved from 19 mm Hg to 55 mm Hg after endovascular therapy; the ulcer was completely healed 2 weeks later. Thus, SPP measurements were useful in the management of the patient. The diagnosis of FMD is usually established by the combination of typical angiographical and pathological changes. We could not obtain histology samples from the tibial artery in this case because amputation was unnecessary. Nonetheless, FMD was pathologically diagnosed previously in the specimen from the temporal artery, and also in the specimen from the mesenteric arteries. Vasculitis of other causes was unlikely from the clinical picture. In addition, the typical angiographic pattern of “strings of beads” strongly supports the diagnosis. Although conventional balloon angioplasty without stenting appeared successful in this patient, close follow-up care is prudent for possible restenosis. REFERENCES 1. Slovut DP, Olin JW. Fibromuscular dysplasia. N Engl J Med 2004;350: 1862-71. 2. Stanly JC, Wakefield TW. Arterial fibrodysplasia. In: Rutherford RB, editor. Vascular surgery. 5th ed. Philadelphia (PA): Saunders; 2000. p. 387-408.
Iida et al 807
3. Luscher TF, Lie JT, Stanson AW, Houser OW Hollier LH, Sheps SG. Arterial fibromuscular dysplasia. Mayo Clin Proc 1987;62:931-52. 4. Topaz O, Warner M, Lanter P, Soffer A, Burns C, DiSciascio G, et al. Isolated significant left main coronary artery stenosis: angiographic, hemodynamic, and clinical findings in 16 patients. Am Heart J 1991; 122:1308-14. 5. Myers SI, Clagett GP, Valentine RJ, Hansen ME, Anand A, Chervu A. Chronic intestinal ischemia caused by intravenous cocaine use: report of two cases and review of the literature. J Vasc Surg 1996;23:724-9. 6. Edwards JM, Antonius JI, Porter JM. Critical hand ischemia caused by forearm fibromuscular dysplasia. J Vasc Surg 1985;2:459-63. 7. Sauer L, Reilly LM, Goldstone J, Ehrenfeld WK, Hutton JE, Stoney RJ. Clinical spectrum of symptomatic external iliac fibromuscular dysplasia. J Vasc Surg 1990;12:488-96. 8. Reilly LM, Cunningham CG, Maggisano R, Ehrenfeld WK, Stoney RJ. The role of arterial reconstruction in spontaneous renal artery dissection. J Vasc Surg 1991;14:4687. 9. 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 dysplasia: a case report and review of the literature on iliac artery dissections secondary to fibromuscular dysplasia. J Vasc Surg 2004;40:1032-6. 10. Berceli SA, Chan AK, Pomposelli FB, Gibbons GW, Campbell DR, Akbari CM, et al. Efficacy of dorsal pedal artery bypass in limb salvage for ischemic heel ulcers. J Vasc Surg 1999;30:499-508. 11. Pomposelli FB, Kansal N, Hamdan AD, Belfield A, Sheahan M, Campbell DR, et al. decade of experience with dorsalis pedis artery bypass: analysis of outcome in more than 1000 cases. J Vasc Surg 2003;37: 307-15. 12. Hughes K, Domenig CM, Hamdan AD, Schermerhorn M, Aulivola B, Blattman S, et al. Bypass to plantar and tarsal arteries: an acceptable approach to limb salvage. J Vasc Surg 2004;40:1149-57. 13. Dorros G, Jaff MR, Dorros AM, Mathiak LM, He T. Tibioperoneal (outflow lesion) angioplasty can be used as primary treatment in 235 patients with critical limb ischemia: five-year follow-up. Circulation 2001;104:2057-62. 14. Feiring AJ, Wesolowski AA, Lade S. Primary stent-supported angioplasty for treatment of below-knee critical limb ischemia and severe claudication: early and one-year outcomes. J Am Coll Cardiol 2004;44: 2307-14. 15. Nicoloff AD, Taylor LM, McLafferty RB, Moneta GL, Porter JM. Patient recovery after infrainguinal bypass grafting for limb salvage. J Vasc Surg 1998;27:256-63. 16. Gentile AT, Lee RW, Moneta GL, Taylor LM, Edwards JM, Porter JM. Results of bypass to the popliteal and tibial arteries with alternative sources of autogenous vein. J Vasc Surg 1996;23:272-79. 17. Goshima KR, Mills JL Sr, Hughes JD. A new look at outcomes after infrainguinal bypass surgery: traditional reporting standards systematically underestimate the expenditure of effort required to attain limb salvage. J Vasc Surg 2004;39:330-5. 18. Adam DJ, Beard JD, Cleveland T, Bell J, Bradbury AW, Forbes JF, et al. Bypass versus angioplasty in severe ischemia of the leg (BASIL): multicenter, randomized controlled trial. Lancet 2005;366:1925-34. 19. Yoshida T, Ohashi I, Suzuki S, Iwai T. Fibromuscular disease of the brachial artery with digital emboli treated effectively by transluminal angioplasty. Cardiovasc Intervent Radiol 1994;17:99-101. 20. Castronuovo JJ Jr, Adera HM, Smiell JM, Price RM. Skin perfusion pressure measurement is valuable in the diagnosis of critical limb ischemia. J Vasc Surg. 1997;26:629-37.
Submitted Feb 21, 2007; accepted May 14, 2007.