Article Vascular and Endovascular Surgery 2014, Vol. 48(7-8) 445-451 ª The Author(s) 2014 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/1538574414551206 ves.sagepub.com
Renal Infarction Complicating Fibromuscular Dysplasia M. Gavalas, MD1, R. Meisner, MD1, N. Labropoulos, PhD1, A. Gasparis, MD1, and A. Tassiopoulos, MD1
Abstract Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries. We present 3 cases of renal infarction complicating renal artery FMD in 42-, 43-, and 46-year-old females and provide a comprehensive review of the literature on this topic. In our patients, oral anticoagulation therapy was used to treat all cases of infarction, and percutaneous angioplasty was used nonemergently in one case to treat refractory hypertension. All patients remained stable at 1-year follow-up. This is consistent with outcomes in previously published reports where conservative medical management was comparable to surgical and interventional therapies. Demographic differences may also exist in patients with renal infarction and FMD. A higher prevalence of males and a younger age at presentation have been found in these patients when compared to the general population with FMD. Keywords vascular medicine, renal artery, fibromuscular dysplasia, hypertension
Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries with the former being implicated in 60% to 75% of cases.1 Presentation of symptoms varies depending on the degree of stenosis, vascular beds involved, type of FMD, and its sequelae such as renal artery aneurysm, dissection, or thrombotic phenomena. Renovascular hypertension secondary to renal artery FMD is well documented and accounts for approximately 10% of all cases of renal artery stenosis.2 A less frequently reported complication of renal artery FMD is renal infarction. A review of the literature showed that the first documented case of renal infarction with renal artery FMD was by Kincaid et al in 1968.3 Since then, numerous case studies have been published on this topic, which account for our current understanding of this rare but serious pathology. In this report, we present 3 cases of renal artery FMD complicated by renal infarction treated conservatively with medical therapy, and review the literature on this rare complication.
Case Reports Patient 1
she was found to have hypertension and costovertebral angle tenderness. Routine laboratory work showed moderate leukocytosis and small hematuria. Nephrolithiasis was suspected at that time, and a noncontrast abdominal computed tomography (CT) was performed, which demonstrated a small nephrolithiasis of the right renal pelvis. However, the left and symptomatic side was without stones. It was assumed that the patient had passed a stone from the left, and the patient was discharged with oral analgesic medication and a urine strainer. Two days later, the patient presented to the emergency department with the same symptoms complicated by moderate hematuria. Duplex ultrasound showed elevated velocities consistent with renal artery stenosis in the mid to distal segment suggestive of FMD. A CT angiogram demonstrated a large area of infarction and a patent left renal artery with focal areas of dilation and stenosis and a small saccular aneurysm (Figure 1). A selective angiogram of the left renal artery confirmed the results. Balloon dilation and stenting were not performed. The next day, a hypercoagulability work-up was performed, and a lactate dehydrogenase (LDH) level was ordered. Protein S, protein C, antithrombin III, and factor V Leiden values were all within 1
A 42-year-old female with past medical history of hypertension controlled with monotherapy presented to the emergency department with a 36-hour history of sharp left-sided flank pain, low grade fever, and nausea. The patient had a 10 packyear history of smoking cigarettes. On physical examination,
Department of Vascular Surgery, Stony Brook University Medical Center, Stony Brook, NY, USA Corresponding Author: M. Gavalas, Department of Vascular Surgery, Stony Brook University Medical Center, HSC Level 19, Rm 090, Stony Brook, NY 11720, USA. Email: [email protected]
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Figure 1. Patient 1: CT coronal view of left kidney with moderate area of infarction to the renal parenchyma (A). Digital subtraction angiogram with stenotic disease of left mid renal artery with subsequent dilation of renal artery (B). The CT angiogram of bilateral renal arteries (C). Left renal artery disease with focal stenosis and dilation (C and D) and small renal artery aneurysm. Left accessory renal artery affected with focal stenosis apparent (C). Reconstituted view of dedicated CT angiogram of renal arteries (D). Demonstrating high-grade stenosis, pointed out above and small renal artery aneurysm, also with arrow above (D). CT indicates computed tomography.
normal limits. Lactate dehydrogenase was elevated at 521 IU/L (normal range ¼ 100-260 IU/L). The patient was treated with anticoagulation therapy. She remained stable and was discharged on oral anticoagulation for 6 months. During followup at 1, 6, 12, and 23 months, there was complete resolution of symptoms.
Patient 2 A 43-year-old female with past medical history of hypertension controlled with 3 medications presented with acute onset of right-sided flank pain, nausea, and vomiting. She had a 17 pack-year history of smoking cigarettes. On physical
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examination, the patient was noted to be hypertensive. Routine laboratory work showed normal cell blood count and electrolyte levels, hematuria, and a markedly elevated LDH (908 IU/L). Abdominal CT with contrast was performed, which demonstrated a moderate right-sided renal infarct. Duplex ultrasound showed elevated velocities consistent with renal artery stenosis. Diagnosis of bilateral FMD was later confirmed by selective angiography, which demonstrated bilateral FMD with a ‘‘string of beads’’ pattern. Angiography showed 75% stenosis and a poststenotic aneurysmal dilation of 9.2 mm of the right renal artery and 60% stenosis of the left renal artery. The patient was placed on anticoagulation therapy and antiplatelet therapy. This patient underwent elective percutaneous transluminal angioplasty on the right and left renal arteries at 6 months and again at 9 months for treatment of her hypertension with normotension seen at 12- and 24-month follow-up.
Patient 3 A 46-year-old female nonsmoker with past medical history of untreated hypertension presented for evaluation of her condition. Her medical history was also significant for migraines and preeclampsia. Her blood pressure in the 2 weeks prior to admission ranged between 140 and 160 mm Hg systolic and 90 and 100 mm Hg diastolic. She had no history of motor or sensory transient ischemic attacks, amaurosis fugax, or strokes. On physical examination, carotid bruits were detected bilaterally, and her blood pressure was markedly elevated. Laboratory work for pheochromocytoma, which included evaluation of normetanephrine, metanephrine, and vanillylmandelic acid levels, was within normal limits. Aldosterone and plasma renin levels were 34.1 ng/dL and 2.2 ng/mL/h, respectively. Lactate dehydrogenase was elevated, peaking at 604 IU/L 15 hours after admission. The patient developed right-sided flank pain, and an abdominal CT showed a right kidney infarction. The patient was started on dual antiplatelet therapy. After 2 weeks, a duplex ultrasound was performed to assess renal parenchymal flow and identify any abnormalities with the renal arteries, at which time bilateral renal artery stenosis was detected. Digital subtraction angiogram confirmed bilateral FMD. Duplex ultrasound of the patient’s carotid arteries suggested bilateral internal carotid artery (ICA) FMD as well. The patient was continued on antiplatelet therapy, and her blood pressure was controlled on 2 antihypertensive medications. She remained stable at 6- and 26-month follow-up.
Methods Published reports of renal infarction secondary to FMD were found through an extensive MEDLINE search for English-language articles using the key words ‘‘renal artery fibromuscular dysplasia’’ and ‘‘renal infarction.’’ A total of 49 articles were identified using this method. Additionally, related articles were found through a bibliography review of relevant articles and were included in the initial evaluation. Articles were retained if the patients presented with signs and
symptoms of acute renal infarction, and the involved artery was identified on the ipsilateral side of infarction. Articles were excluded if treatment and outcomes were not reported, if patients presented with symptoms attributable to nonrenal organ dysfunction and infarction was incidentally discovered on imaging, and if cases were presented retrospectively without describing presentation, treatment, and follow-up. Additionally, a patient with a rare case of congenital FMD involving multiple organs who died at the age of 14 months from multisystem involvement was excluded from the analysis.4 In total, 15 cases of renal infarction secondary to renal artery FMD were included in our analysis and are presented in Table 1.
Discussion Complications of renal artery FMD include renovascular hypertension, aneurysm, dissection, thrombosis, arterial occlusion, and renal infarction. Infarction is a rare complication of FMD and its true incidence has yet to be determined. Kincaid et al first reported on renal infarction secondary to FMD and found that 13 (10%) of the 125 patients with renal artery FMD presented with renal infarction. Of the 13 cases, 4 were found to have arterial dissection, while 8 displayed severe renal artery stenosis.3 More recently, the largest registry of 447 patients with FMD found the incidence of renal infarction to be 0.9%.20 Clearly, renal infarction is a rare manifestation of this disease. Five subtypes of FMD have been identified with classification being based on the affected layer of the arterial wall. Medial fibroplasia (75%-80%), perimedial fibroplasia (10%-15%), intimal fibroplasia (
Renal Infarction Complicating Fibromuscular Dysplasia M. Gavalas, MD1, R. Meisner, MD1, N. Labropoulos, PhD1, A. Gasparis, MD1, and A. Tassiopoulos, MD1
Abstract Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries. We present 3 cases of renal infarction complicating renal artery FMD in 42-, 43-, and 46-year-old females and provide a comprehensive review of the literature on this topic. In our patients, oral anticoagulation therapy was used to treat all cases of infarction, and percutaneous angioplasty was used nonemergently in one case to treat refractory hypertension. All patients remained stable at 1-year follow-up. This is consistent with outcomes in previously published reports where conservative medical management was comparable to surgical and interventional therapies. Demographic differences may also exist in patients with renal infarction and FMD. A higher prevalence of males and a younger age at presentation have been found in these patients when compared to the general population with FMD. Keywords vascular medicine, renal artery, fibromuscular dysplasia, hypertension
Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory vascular disease that most commonly affects the renal and extracranial carotid arteries with the former being implicated in 60% to 75% of cases.1 Presentation of symptoms varies depending on the degree of stenosis, vascular beds involved, type of FMD, and its sequelae such as renal artery aneurysm, dissection, or thrombotic phenomena. Renovascular hypertension secondary to renal artery FMD is well documented and accounts for approximately 10% of all cases of renal artery stenosis.2 A less frequently reported complication of renal artery FMD is renal infarction. A review of the literature showed that the first documented case of renal infarction with renal artery FMD was by Kincaid et al in 1968.3 Since then, numerous case studies have been published on this topic, which account for our current understanding of this rare but serious pathology. In this report, we present 3 cases of renal artery FMD complicated by renal infarction treated conservatively with medical therapy, and review the literature on this rare complication.
Case Reports Patient 1
she was found to have hypertension and costovertebral angle tenderness. Routine laboratory work showed moderate leukocytosis and small hematuria. Nephrolithiasis was suspected at that time, and a noncontrast abdominal computed tomography (CT) was performed, which demonstrated a small nephrolithiasis of the right renal pelvis. However, the left and symptomatic side was without stones. It was assumed that the patient had passed a stone from the left, and the patient was discharged with oral analgesic medication and a urine strainer. Two days later, the patient presented to the emergency department with the same symptoms complicated by moderate hematuria. Duplex ultrasound showed elevated velocities consistent with renal artery stenosis in the mid to distal segment suggestive of FMD. A CT angiogram demonstrated a large area of infarction and a patent left renal artery with focal areas of dilation and stenosis and a small saccular aneurysm (Figure 1). A selective angiogram of the left renal artery confirmed the results. Balloon dilation and stenting were not performed. The next day, a hypercoagulability work-up was performed, and a lactate dehydrogenase (LDH) level was ordered. Protein S, protein C, antithrombin III, and factor V Leiden values were all within 1
A 42-year-old female with past medical history of hypertension controlled with monotherapy presented to the emergency department with a 36-hour history of sharp left-sided flank pain, low grade fever, and nausea. The patient had a 10 packyear history of smoking cigarettes. On physical examination,
Department of Vascular Surgery, Stony Brook University Medical Center, Stony Brook, NY, USA Corresponding Author: M. Gavalas, Department of Vascular Surgery, Stony Brook University Medical Center, HSC Level 19, Rm 090, Stony Brook, NY 11720, USA. Email: [email protected]
Downloaded from ves.sagepub.com at University of Otago Library on March 14, 2015
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Figure 1. Patient 1: CT coronal view of left kidney with moderate area of infarction to the renal parenchyma (A). Digital subtraction angiogram with stenotic disease of left mid renal artery with subsequent dilation of renal artery (B). The CT angiogram of bilateral renal arteries (C). Left renal artery disease with focal stenosis and dilation (C and D) and small renal artery aneurysm. Left accessory renal artery affected with focal stenosis apparent (C). Reconstituted view of dedicated CT angiogram of renal arteries (D). Demonstrating high-grade stenosis, pointed out above and small renal artery aneurysm, also with arrow above (D). CT indicates computed tomography.
normal limits. Lactate dehydrogenase was elevated at 521 IU/L (normal range ¼ 100-260 IU/L). The patient was treated with anticoagulation therapy. She remained stable and was discharged on oral anticoagulation for 6 months. During followup at 1, 6, 12, and 23 months, there was complete resolution of symptoms.
Patient 2 A 43-year-old female with past medical history of hypertension controlled with 3 medications presented with acute onset of right-sided flank pain, nausea, and vomiting. She had a 17 pack-year history of smoking cigarettes. On physical
Downloaded from ves.sagepub.com at University of Otago Library on March 14, 2015
Gavalas et al
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examination, the patient was noted to be hypertensive. Routine laboratory work showed normal cell blood count and electrolyte levels, hematuria, and a markedly elevated LDH (908 IU/L). Abdominal CT with contrast was performed, which demonstrated a moderate right-sided renal infarct. Duplex ultrasound showed elevated velocities consistent with renal artery stenosis. Diagnosis of bilateral FMD was later confirmed by selective angiography, which demonstrated bilateral FMD with a ‘‘string of beads’’ pattern. Angiography showed 75% stenosis and a poststenotic aneurysmal dilation of 9.2 mm of the right renal artery and 60% stenosis of the left renal artery. The patient was placed on anticoagulation therapy and antiplatelet therapy. This patient underwent elective percutaneous transluminal angioplasty on the right and left renal arteries at 6 months and again at 9 months for treatment of her hypertension with normotension seen at 12- and 24-month follow-up.
Patient 3 A 46-year-old female nonsmoker with past medical history of untreated hypertension presented for evaluation of her condition. Her medical history was also significant for migraines and preeclampsia. Her blood pressure in the 2 weeks prior to admission ranged between 140 and 160 mm Hg systolic and 90 and 100 mm Hg diastolic. She had no history of motor or sensory transient ischemic attacks, amaurosis fugax, or strokes. On physical examination, carotid bruits were detected bilaterally, and her blood pressure was markedly elevated. Laboratory work for pheochromocytoma, which included evaluation of normetanephrine, metanephrine, and vanillylmandelic acid levels, was within normal limits. Aldosterone and plasma renin levels were 34.1 ng/dL and 2.2 ng/mL/h, respectively. Lactate dehydrogenase was elevated, peaking at 604 IU/L 15 hours after admission. The patient developed right-sided flank pain, and an abdominal CT showed a right kidney infarction. The patient was started on dual antiplatelet therapy. After 2 weeks, a duplex ultrasound was performed to assess renal parenchymal flow and identify any abnormalities with the renal arteries, at which time bilateral renal artery stenosis was detected. Digital subtraction angiogram confirmed bilateral FMD. Duplex ultrasound of the patient’s carotid arteries suggested bilateral internal carotid artery (ICA) FMD as well. The patient was continued on antiplatelet therapy, and her blood pressure was controlled on 2 antihypertensive medications. She remained stable at 6- and 26-month follow-up.
Methods Published reports of renal infarction secondary to FMD were found through an extensive MEDLINE search for English-language articles using the key words ‘‘renal artery fibromuscular dysplasia’’ and ‘‘renal infarction.’’ A total of 49 articles were identified using this method. Additionally, related articles were found through a bibliography review of relevant articles and were included in the initial evaluation. Articles were retained if the patients presented with signs and
symptoms of acute renal infarction, and the involved artery was identified on the ipsilateral side of infarction. Articles were excluded if treatment and outcomes were not reported, if patients presented with symptoms attributable to nonrenal organ dysfunction and infarction was incidentally discovered on imaging, and if cases were presented retrospectively without describing presentation, treatment, and follow-up. Additionally, a patient with a rare case of congenital FMD involving multiple organs who died at the age of 14 months from multisystem involvement was excluded from the analysis.4 In total, 15 cases of renal infarction secondary to renal artery FMD were included in our analysis and are presented in Table 1.
Discussion Complications of renal artery FMD include renovascular hypertension, aneurysm, dissection, thrombosis, arterial occlusion, and renal infarction. Infarction is a rare complication of FMD and its true incidence has yet to be determined. Kincaid et al first reported on renal infarction secondary to FMD and found that 13 (10%) of the 125 patients with renal artery FMD presented with renal infarction. Of the 13 cases, 4 were found to have arterial dissection, while 8 displayed severe renal artery stenosis.3 More recently, the largest registry of 447 patients with FMD found the incidence of renal infarction to be 0.9%.20 Clearly, renal infarction is a rare manifestation of this disease. Five subtypes of FMD have been identified with classification being based on the affected layer of the arterial wall. Medial fibroplasia (75%-80%), perimedial fibroplasia (10%-15%), intimal fibroplasia (
