Ann Vasc Dis Vol. 7, No. 2; 2014; pp 169–172 ©2014 Annals of Vascular Diseases
Online Month May 16, 2014 doi:10.3400/avd.cr.13-00113
Case Report
Vasospastic Limb Ischemia Presenting Acute and Chronic Limb Ischemia Junji Kaneyama, MD,1 Osami Kawarada, MD,1,2 Shingo Sakamoto, MD,1 Koichiro Harada, MD, PhD,1 Masaharu Ishihara, MD, PhD,1,2 Satoshi Yasuda, MD, PhD,1,2 and Hisao Ogawa, MD, PhD1,3
Vasospastic limb ischemia might have been underappreciated compared to vasospasm in other territories such as heart and brain. However, an increasing awareness of this vascular disorder can be translated to an improved patients’ care. Herein, we report a case of vasospasm presenting acute and chronic limb ischemia in four extremities. Keywords: vasospasm, limb ischemia, recurrence
Introduction Vasospasm in the extremities is uncommon compared to vasospasm in other territories such as heart and brain.1,2) However, an increasing knowledge about vasospastic limb ischemia is crucial in appropriate diagnosis and treatment for vascular specialists. Herein, we report a case of vasospasm presenting both acute and chronic limb ischemia in four extremities.
Case Report A 28-year-old male (height 182 cm, body weight 80 kg) with a current smoking history was referred to our hospital for the diagnosis and treatment of developing ischemic symptoms in upper and lower limbs. Two 1Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan 2Department of Advanced Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan 3Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
Received: November 25, 2013; Accepted: March 15, 2014 Corresponding author: Osami Kawarada, MD. Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan Tel: +81-6-6833-5012, Fax: +81-6-6872-7486 E-mail: [email protected]
Annals of Vascular Diseases Vol. 7, No. 2 (2014)
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weeks before, the patient had experienced bilateral hand pain at rest. One week before, the patient’s symptom had included life-style limiting claudication and calf pain at rest despite smoking cessation. On his arrival, peripheral pulsations in radial arteries, dorsalis pedis arteries, and posterior tibial arteries were absent. The patient’s blood pressure was 121/60 mmHg and pulse rate was 82 beats per minute with regular rhythm. Laboratory data including lipid and diabetic profile was normal except for increase in serum creatinine phosphokinase (3961 U/L). Chest X-ray and electrocardiography also revealed no abnormality. Diagnostic imaging including duplex ultrasonography and enhanced computed tomography (CT) demonstrated the artery disruptions in upper and lower extremities (Fig. 1A). We initiated medical treatment including intravenous administration of alprostadil as well as oral administration of cilostazol and beraprost immediately after hospitalization. His symptom dramatically improved with significant recovery of pulsation in extremity arteries, and imaging examinations also demonstrated excellent recovery of disruptive lesions (Fig. 1B). The patient uneventfully discharged under medical management 7 days later. A rheumatologic and serological examination proved negative, and neither relationship between the patient’s symptom and the cold stimulus suggestive of Raynaud’s disease nor occupational history inducing vibration syndrome and hammer syndrome 169
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(A)
(B)
was rehospitalized for the treatment of “acute” limb ischemia due to recurrent vasospasm. Oral administration of calcium channel blocker and isosorbide dinitrate-containing patch were added to the baseline treatment. His symptoms dramatically improved with full recovery of four limb arteries’ pulsation immediately after medical treatment. Enhanced CT also detected successful recovery of the disruptive arteries (Fig. 2B). Although serum creatinine phosphokinase increased up to 40820 U/L and reperfusion injury following the relief of vasospasm was observed in bilateral calves (Fig. 3), neither of myonephropathic metabolic syndrome, compartment syndrome nor amputation was observed. He was uneventfully discharged 16 days later, and no recurrence of vascular event has been observed under medical treatment and smoking cessation during the 12-month follow-up period.
Discussion
Fig. 1
Enhanced computed tomography (CT) angiogram in the left upper extremity. (A) Diagnostic enhanced CT angiogram showing segmental disruptions in the brachial (large arrow), radial and ulnar arteries (small arrows). (B) After the initiation of medical treatment, complete recovery of those disruptive lesions was observed.
was present. Also, Buerger’s disease was excluded because of failure to meet diagnostic criteria.3) No history of administration of any drugs inducing vasospasm was evident. Thus, differential diagnosis confirmed spontaneous vasospastic limb ischemia. However, the patient suddenly suffered from serious resting pain in bilateral upper and lower extremities 7 days later after the first discharge. He had discontinued oral medications without physician’s permission after the discharge, and his peripheral arteries were again pale and pulseless, and enhanced CT angiography demonstrated recurrent disruptive lesions in the four extremity arteries (Fig. 2A). He 170
17_CR_113_Kawarada.indd 170 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
To the best of our knowledge, this is the first to report recurrent limb ischemia consisting of not only chronic limb ischemia but also acute limb ischemia due to spontaneous vasospasm in four extremities. Vasospasm can occur by the constriction of arterial smooth muscle in the territory of muscular artery. Drug-induced vasospatic limb ischemia by ergotamine, methysergide, cocaine and lysergic acid diethylamide is well known.4–6) However, there are only a few cases regarding spontaneous vasospasm in the lower limb artery in which acute onset of femoropopliteal or peroneal artery occlusion was observed.1,2) Unfortunately, the reason for this rare phenomenon remains unclear. Of great interest, our case presented both “acute” and “chronic” limb ischemia. The diagnosis of vasospastic limb ischemia could be challenging in the setting of clinical practice. The process of differential diagnosis ruled out other vascular diseases based on the following reasons: (1) lack of atherosclerotic risk factors except for current smoking history; (2) no evidence of vasculitis; (3) no suggestion of Raynaud’s disease; (4) no occupational exposure; (5) no history of drug use to induce vasospasm; and (6) failure to meet diagnostic criteria for Buerger’s disease. Thus, we believed that this patient had vasospastic limb ischemia. As of today, no established remedy exists despite vasospastic limb ischemia could potentially results Annals of Vascular Diseases Vol. 7, No. 2 (2014)
2014/06/13 15:30
Vasospastic Limb Ischemia
(A)
Fig. 2
(B)
Enhanced computed tomography (CT) angiogram in the bilateral lower extremity. (A) Diagnostic enhanced CT angiogram showing tight narrowings in the bilateral femoropopliteal segments (large arrows) and disruptions in the bilateral anterior tibial arteries (small arrows). Also, the proximal segment in the superficial femoral artery seems to be spastic. (B) After the initiation of medical treatment in the 2nd hospitalization, complete recovery of those disruptive lesions was observed though crural arteries were superimposed upon the veins.
(A)
Fig. 3
(B)
Reperfusion injury following the relief of vasospasm in the lower limb arteries. Please note swollen and reddish calves (arrows). (A) right, (B) left.
in catastrophic clinical scenarios. Smoking is well known to be associated with coronary vasospasm.7) In this case as well, smoking might have served as a trigger of limb arteries’ spasm and smoking cessation could be of help for prevention of recurrence of limb arteries’ spasm. Also, calcium channel blocker can be essential in the medical treatment of coronary vasospasm.8,9) In this case as well, besides vascular dilators, calcium channel blocker might have worked well in terms of prevention of recurrent vasospastic Annals of Vascular Diseases Vol. 7, No. 2 (2014)
17_CR_113_Kawarada.indd 171 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
limb ischemia. Thus, the combination of smoking cessation and medical therapy could be the mainstream in the management of vasospasm although further investigation needs to be undertaken regarding optimal medical treatment for vasospastic limb ischemia. With the possibility of vasospasm in our mind, the appreciation of clinical and angiographical findings can definitely reduce the chance of misdiagnosis and unnecessary invasive intervention, and can be 171
2014/06/13 15:30
Kaneyama J, et al.
translated to an improved patient’s care. We emphasize the need for a more awareness of vasospastic limb ischemia in the field of vascular medicine.
Acknowledgements The present work was supported in part by the grants-in-aid [23591026] from the Scientific Research and the grants-in-aid [H24-Junkanki-009] from the Japanese Ministry of Health, Labour and Welfare, Tokyo, Japan.
3) 4)
5)
6)
Disclosure Statement All authors have no conflict of interest.
References 1) Bory M, Mattei M, Egre A, et al. Acute ischemic syndrome and apparently spontaneous spasms of the lower leg arteries. Coeur Med Interne 1979; 18: 607-11. (in French) 2) Winckiewicz M, Stanisic MG, Szajkowski R, et al. Acute lower limb ischemia in a young woman with
172
17_CR_113_Kawarada.indd 172 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
7) 8)
9)
arterial hypoplasia: a case report. Angiology 2007; 58: 494-7. Olin JW. Thromboangiitis obliterans (Buerger’s disease). N Engl J Med 2000; 343: 864-9. Dorne HL, Satin R. Methysergide-induced lower extremity arterial insufficiency. Can Assoc Radiol J 1986; 37: 210-2. Silverman DG, Kosten TR, Jatlow PI, et al. Decreased digital flow persists after the abatement of cocaine-induced hemodynamic stimulation. Anesth Analg 1997; 84: 46-50. Raval MV, Gaba RC, Brown K, et al. Percutaneous transluminal angioplasty in the treatment of extensive LSD-induced lower extremity vasospasm refractory to pharmacologic therapy. J Vasc Interv Radiol 2008; 19: 1227-30. Sugiishi M, Takatsu F. Cigarette smoking is a major risk factor for coronary spasm. Circulation 1993; 87: 76-9. Nishigaki K, Inoue Y, Yamanouchi Y, et al. Prognostic effects of calcium channel blockers in patients with vasospastic angina—a meta-analysis. Circ J 2010; 74: 1943-50. JCS Joint Working Group. Guidelines for diagnosis and treatment of patients with vasospastic angina (coronary spastic angina) (JCS 2008): digest version. Circ J 2010; 74: 1745-62.
Annals of Vascular Diseases Vol. 7, No. 2 (2014)
2014/06/13 15:31
Online Month May 16, 2014 doi:10.3400/avd.cr.13-00113
Case Report
Vasospastic Limb Ischemia Presenting Acute and Chronic Limb Ischemia Junji Kaneyama, MD,1 Osami Kawarada, MD,1,2 Shingo Sakamoto, MD,1 Koichiro Harada, MD, PhD,1 Masaharu Ishihara, MD, PhD,1,2 Satoshi Yasuda, MD, PhD,1,2 and Hisao Ogawa, MD, PhD1,3
Vasospastic limb ischemia might have been underappreciated compared to vasospasm in other territories such as heart and brain. However, an increasing awareness of this vascular disorder can be translated to an improved patients’ care. Herein, we report a case of vasospasm presenting acute and chronic limb ischemia in four extremities. Keywords: vasospasm, limb ischemia, recurrence
Introduction Vasospasm in the extremities is uncommon compared to vasospasm in other territories such as heart and brain.1,2) However, an increasing knowledge about vasospastic limb ischemia is crucial in appropriate diagnosis and treatment for vascular specialists. Herein, we report a case of vasospasm presenting both acute and chronic limb ischemia in four extremities.
Case Report A 28-year-old male (height 182 cm, body weight 80 kg) with a current smoking history was referred to our hospital for the diagnosis and treatment of developing ischemic symptoms in upper and lower limbs. Two 1Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, Suita, Osaka, Japan 2Department of Advanced Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan 3Department of Cardiovascular Medicine, Graduate School of Medical Sciences, Kumamoto University, Kumamoto, Kumamoto, Japan
Received: November 25, 2013; Accepted: March 15, 2014 Corresponding author: Osami Kawarada, MD. Department of Cardiovascular Medicine, National Cerebral and Cardiovascular Center, 5-7-1 Fujishiro-dai, Suita, Osaka 565-8565, Japan Tel: +81-6-6833-5012, Fax: +81-6-6872-7486 E-mail: [email protected]
Annals of Vascular Diseases Vol. 7, No. 2 (2014)
17_CR_113_Kawarada.indd 169 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
weeks before, the patient had experienced bilateral hand pain at rest. One week before, the patient’s symptom had included life-style limiting claudication and calf pain at rest despite smoking cessation. On his arrival, peripheral pulsations in radial arteries, dorsalis pedis arteries, and posterior tibial arteries were absent. The patient’s blood pressure was 121/60 mmHg and pulse rate was 82 beats per minute with regular rhythm. Laboratory data including lipid and diabetic profile was normal except for increase in serum creatinine phosphokinase (3961 U/L). Chest X-ray and electrocardiography also revealed no abnormality. Diagnostic imaging including duplex ultrasonography and enhanced computed tomography (CT) demonstrated the artery disruptions in upper and lower extremities (Fig. 1A). We initiated medical treatment including intravenous administration of alprostadil as well as oral administration of cilostazol and beraprost immediately after hospitalization. His symptom dramatically improved with significant recovery of pulsation in extremity arteries, and imaging examinations also demonstrated excellent recovery of disruptive lesions (Fig. 1B). The patient uneventfully discharged under medical management 7 days later. A rheumatologic and serological examination proved negative, and neither relationship between the patient’s symptom and the cold stimulus suggestive of Raynaud’s disease nor occupational history inducing vibration syndrome and hammer syndrome 169
2014/06/13 15:30
Kaneyama J, et al.
(A)
(B)
was rehospitalized for the treatment of “acute” limb ischemia due to recurrent vasospasm. Oral administration of calcium channel blocker and isosorbide dinitrate-containing patch were added to the baseline treatment. His symptoms dramatically improved with full recovery of four limb arteries’ pulsation immediately after medical treatment. Enhanced CT also detected successful recovery of the disruptive arteries (Fig. 2B). Although serum creatinine phosphokinase increased up to 40820 U/L and reperfusion injury following the relief of vasospasm was observed in bilateral calves (Fig. 3), neither of myonephropathic metabolic syndrome, compartment syndrome nor amputation was observed. He was uneventfully discharged 16 days later, and no recurrence of vascular event has been observed under medical treatment and smoking cessation during the 12-month follow-up period.
Discussion
Fig. 1
Enhanced computed tomography (CT) angiogram in the left upper extremity. (A) Diagnostic enhanced CT angiogram showing segmental disruptions in the brachial (large arrow), radial and ulnar arteries (small arrows). (B) After the initiation of medical treatment, complete recovery of those disruptive lesions was observed.
was present. Also, Buerger’s disease was excluded because of failure to meet diagnostic criteria.3) No history of administration of any drugs inducing vasospasm was evident. Thus, differential diagnosis confirmed spontaneous vasospastic limb ischemia. However, the patient suddenly suffered from serious resting pain in bilateral upper and lower extremities 7 days later after the first discharge. He had discontinued oral medications without physician’s permission after the discharge, and his peripheral arteries were again pale and pulseless, and enhanced CT angiography demonstrated recurrent disruptive lesions in the four extremity arteries (Fig. 2A). He 170
17_CR_113_Kawarada.indd 170 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
To the best of our knowledge, this is the first to report recurrent limb ischemia consisting of not only chronic limb ischemia but also acute limb ischemia due to spontaneous vasospasm in four extremities. Vasospasm can occur by the constriction of arterial smooth muscle in the territory of muscular artery. Drug-induced vasospatic limb ischemia by ergotamine, methysergide, cocaine and lysergic acid diethylamide is well known.4–6) However, there are only a few cases regarding spontaneous vasospasm in the lower limb artery in which acute onset of femoropopliteal or peroneal artery occlusion was observed.1,2) Unfortunately, the reason for this rare phenomenon remains unclear. Of great interest, our case presented both “acute” and “chronic” limb ischemia. The diagnosis of vasospastic limb ischemia could be challenging in the setting of clinical practice. The process of differential diagnosis ruled out other vascular diseases based on the following reasons: (1) lack of atherosclerotic risk factors except for current smoking history; (2) no evidence of vasculitis; (3) no suggestion of Raynaud’s disease; (4) no occupational exposure; (5) no history of drug use to induce vasospasm; and (6) failure to meet diagnostic criteria for Buerger’s disease. Thus, we believed that this patient had vasospastic limb ischemia. As of today, no established remedy exists despite vasospastic limb ischemia could potentially results Annals of Vascular Diseases Vol. 7, No. 2 (2014)
2014/06/13 15:30
Vasospastic Limb Ischemia
(A)
Fig. 2
(B)
Enhanced computed tomography (CT) angiogram in the bilateral lower extremity. (A) Diagnostic enhanced CT angiogram showing tight narrowings in the bilateral femoropopliteal segments (large arrows) and disruptions in the bilateral anterior tibial arteries (small arrows). Also, the proximal segment in the superficial femoral artery seems to be spastic. (B) After the initiation of medical treatment in the 2nd hospitalization, complete recovery of those disruptive lesions was observed though crural arteries were superimposed upon the veins.
(A)
Fig. 3
(B)
Reperfusion injury following the relief of vasospasm in the lower limb arteries. Please note swollen and reddish calves (arrows). (A) right, (B) left.
in catastrophic clinical scenarios. Smoking is well known to be associated with coronary vasospasm.7) In this case as well, smoking might have served as a trigger of limb arteries’ spasm and smoking cessation could be of help for prevention of recurrence of limb arteries’ spasm. Also, calcium channel blocker can be essential in the medical treatment of coronary vasospasm.8,9) In this case as well, besides vascular dilators, calcium channel blocker might have worked well in terms of prevention of recurrent vasospastic Annals of Vascular Diseases Vol. 7, No. 2 (2014)
17_CR_113_Kawarada.indd 171 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
limb ischemia. Thus, the combination of smoking cessation and medical therapy could be the mainstream in the management of vasospasm although further investigation needs to be undertaken regarding optimal medical treatment for vasospastic limb ischemia. With the possibility of vasospasm in our mind, the appreciation of clinical and angiographical findings can definitely reduce the chance of misdiagnosis and unnecessary invasive intervention, and can be 171
2014/06/13 15:30
Kaneyama J, et al.
translated to an improved patient’s care. We emphasize the need for a more awareness of vasospastic limb ischemia in the field of vascular medicine.
Acknowledgements The present work was supported in part by the grants-in-aid [23591026] from the Scientific Research and the grants-in-aid [H24-Junkanki-009] from the Japanese Ministry of Health, Labour and Welfare, Tokyo, Japan.
3) 4)
5)
6)
Disclosure Statement All authors have no conflict of interest.
References 1) Bory M, Mattei M, Egre A, et al. Acute ischemic syndrome and apparently spontaneous spasms of the lower leg arteries. Coeur Med Interne 1979; 18: 607-11. (in French) 2) Winckiewicz M, Stanisic MG, Szajkowski R, et al. Acute lower limb ischemia in a young woman with
172
17_CR_113_Kawarada.indd 172 プロセスシアンプロセスマゼンタプロセスイエロープロセスブラック
7) 8)
9)
arterial hypoplasia: a case report. Angiology 2007; 58: 494-7. Olin JW. Thromboangiitis obliterans (Buerger’s disease). N Engl J Med 2000; 343: 864-9. Dorne HL, Satin R. Methysergide-induced lower extremity arterial insufficiency. Can Assoc Radiol J 1986; 37: 210-2. Silverman DG, Kosten TR, Jatlow PI, et al. Decreased digital flow persists after the abatement of cocaine-induced hemodynamic stimulation. Anesth Analg 1997; 84: 46-50. Raval MV, Gaba RC, Brown K, et al. Percutaneous transluminal angioplasty in the treatment of extensive LSD-induced lower extremity vasospasm refractory to pharmacologic therapy. J Vasc Interv Radiol 2008; 19: 1227-30. Sugiishi M, Takatsu F. Cigarette smoking is a major risk factor for coronary spasm. Circulation 1993; 87: 76-9. Nishigaki K, Inoue Y, Yamanouchi Y, et al. Prognostic effects of calcium channel blockers in patients with vasospastic angina—a meta-analysis. Circ J 2010; 74: 1943-50. JCS Joint Working Group. Guidelines for diagnosis and treatment of patients with vasospastic angina (coronary spastic angina) (JCS 2008): digest version. Circ J 2010; 74: 1745-62.
Annals of Vascular Diseases Vol. 7, No. 2 (2014)
2014/06/13 15:31
