DOCSLIB.ORG

Fibromuscular Dysplasia Complicated by Primary

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Fibromuscular Dysplasia Complicated by Primary Case Report Fibromuscular Dysplasia Complicated by Primary Thrombocythemia Presenting Recurrent Ischemic Cerebrovascular Accidents: A Case Report Kaori ABE, Satoru OHTA, Tadatoshi KOMIYA, Naotaka Sakamoto* and Yoshikuni Mizuno A patient with fibromuscular dysplasia associated with primary thrombocythemia is reported. The patient, a 46-year-old female, had recurrent episodes of ischemic cerebrovascular accidents. Cranial CTscans revealed multiple low density areas in the right frontal and parietal lobes, and cerebral angiograms showed evidence of multiple embolisms. In addition, occlusion of the right internal carotid artery developed. Toour knowledge, this is the first report on fibromuscular dysplasia complicated by essential thrombocythemia. The pathogenesis of the severe thrombotic tendency is discussed. Key words: Fibromuscular dysplasia , Cerebral infarction, Transient ischemic attack, Carotid occlusion Both essential thrombocythemia and fibro- bocytosis was noted at the time. From the summer muscular dysplasia are rare disorders in Japan. They of 1988 on, she had frequent episodes of headache can be considered as possible causes of stroke in for which she required an ergot derivative for 6 younger persons. Wereport a patient with fibro- months. Also, in August 1988, she had an episode muscular dysplasia complicated by essential throm- of tingling sensation and weakness in her left hand. bocythemia. To our knowledge, such an association In January 1989 she felt a transient weakness on the has never been reported. The patient showed left side of her face and dysarthria upon waking up multiple cerebrovascular thrombo-embolicepisodes one morning. OnMarch 8, 1989 she noted weakness and complete occlusion of the right internal carotid in her upper and lower left extremities and was artery. The purpose of this report is to present the admitted to the Department of Radiology at first known case of fibromuscular dysplasia Juntendo University Medical Center the next day. associated with primary thrombocythemia and to A low density area was found in the right parieto- discuss the pathogenesis and the treatment of this occipital area by CT, and angiograms revealed disorder. irregularities in the right internal carotid artery. Her symptoms improved during the next 3 weeks, and she CASE REPORT was discharged with a prescription of 200 mg/day The patient, a 46-year-old female, who was well ticlopidine. On April 4, she again had an episode until the end of December 1987, had a sudden onset of headache and felt weakness in her left arm. On of severe occipital headache, nausea and tinnitus. April 7, weakness appeared in her left leg. She was She was admitted to a hospital where the neuro- re-admitted to the Department of Radiology. On the logical examination and cranial CT scan were 7th hospital day, she noted a visual field defect in reported to have been normal. However, throm- her right eye, and the neurology service was asked From Department of Neurology and *Radiology, Juntendo University School of Medicine, Tokyo Received for publication December 27, 1989; Accepted for publication July 24, 1990 Reprint requests should be addressed to Yoshikuni Mizuno, MD,Department of Neurology, Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113, Japan 548 Jpn J Med Vol 29, No 5 (September, October 1990) Fibromuscular Dysplasia and Thrombocythemia to see the patient. left extremity with pseudo-athetotic movements in Physical examination revealed a well-nourished the left hand. No cerebellar ataxia was present. Deep and well-developed Japanese female in no acute reflexes were slightly exaggerated in her upper left distress. Blood pressure was 130/70 mmHg,and the extremity. No pathological reflexes were noted. pulse rate 70/min and regular. No cervical bruit was Superficial and vibration senses were normal. noted. There wasneither cardiac enlargement nor However, position, movement,stereotactic and two- heart murmur. The lungs were clear. No hepato- point discriminative sensations were diminished in splenomegaly was noted. her left hand. Neurological examination revealed an alert and The laboratory findings are summarized in Table intelligent female. Neither aphasia, apraxia nor 1. A marked increase in thrombocytes with giant agnosia was noted. Her visual acuity was 0.01 on platelets in the peripheral blood was noted (Fig. 1A). the right and 1.2 on the left. She showed a concentric Bleeding time was 1.5 min, PTT 37.9 s and PT 10.0 narrowing of the visual field bilaterally and a central s. The blood thromboxane B2 level was elevated to scotoma in her right eye. The optic fundi showed 78 pg/ml (normal: 14-50), and platelet factor 4 to occlusion of the right central retinal artery. Occular 49 ng/ml (20 >). These values were interpreted as movement,pupils and light reflex were normal. No suggesting the presence of a thrombotic tendency. nystagmus was seen. She showed left facial weakness Protein C, factor VIII, antithrombin III, fibrinogen, and deviation of the tongue to the left without FDP, oyplasmin and plasminogen inhibitor were atrophy. Other cranial nerves were intact. Her gait normal. The CSF findings were unremarkable. was normal. Slight weakness was noted in her upper Echocardiography and ECGwere normal. Ab- Table 1. Laboratory data. (Data on admission) RBC 442 x lO4/mm3 GOT 19 IU/1 (5-35) Hb 14.2 g/dl GPT 17 » (4-30) Ht 41.7% LDH 625 " (125-500) WBC 13800/mm3 ALP 5.5 KAU (1.6-9.9) Meta 1% BUN 1 1 mg/dl (8-17) Stab 6% Creatinine 0.7 » (0.6-1.2) Seg 72% Na 139 mEq/1 Lympho 10.5% K 3.7 " Mono 3% Cl ,104 " Eos 4.5% T cholesterol 168 mg/dl (150-230) Baso 2.5% Triglycerides 89 » (37-150) PLT 824x l03/mm3 Phospholipids 179 » (150-250) Anti-cardiolipin antibody (-) Mar3 Apr19 Apr25 May10 May20 PLT ( x lO3/mm3) 824 601 1072 1034 1268 Giant PLT + + + WBC ( /mm3) 13800 11400 12900 10100 11700 RBC ( x lOVmm3) 442 466 496 473 451 ESR (1 h/2 h) 4/12 5/ 7/18 9/24 CRP - Mar14 Mar23 Apr20 May16 Platelet 40% 16% 28% 12% aggregation* *ADP 2.3/uM (N: 40-70%) Jpn J Med Vol 29, No 5 (September, October 1990) 549 Abe et al Fig. 1. Giant platelets in the peripheral blood (A), and increase in megakaryocytes in the bone marrow (B). dominal CT scan and ultrasound examination Alow density area with scattered high density spots showedslight splenomegaly. Bone marrow examina- was seen in the right parieto-occipital area on March tion revealed mild generalized hyperplasia (Fig. IB), 9. Asimilar low density area was noted in the frontal including megakaryocytic hyperplasja. Neither lobe on April 10. Cerebral angiograms on March fibrosis nor leukemic cell infiltration was noted. The 9 (Fig. 3A) showed a narrowing in the right internal differential count of bone marrow cells was normal. carotid artery 2 cm above the bifurcation. The Chromosomeanalysis of a bone marrow aspirate appearance of the narrowing suggested "tubular wasnormal. Her neurophile alkaline phosphatase stenosis." The left internal carotid artery showed score was 224 (240 ±60). multiple segmental narrowing and dilatation. Direct The serial cranial CT scans are shown in Fig. 2. right carotid angiograms on the same day (Fig. 4A, Fig. 2. Cranial CT scans on March 9. A low density area with scattered high density spots is seen in the right parieto-occipital area (A), and a low density area in the frontal lobe on April 10 (B). 550 Jpn J Med Vol 29, No 5 (September, October 1990) Fibromuscular Dysplasia and Thrombocythemia B) showed a filling defect along the wall of the right vical spine and extending into the intracranial internal carotid artery in its cavernous portion portion. The entire vascular lumen showed marked extending into the horizontal portion of the right irregular narrowing. Therenal arteries werenormal middle cerebral artery. The terminal branches of the on the aortogram. The right carotid angiogram per- right middle cerebral artery showed multiple area of formed 10 days later (Fig. 6A) revealed no filling obstruction and narrowing, suggesting shower defect in the portion where the previous angiograms embolisms. The right vertebral angiograms on the had shown a marked filling defect. The digital sub- same day, March 9, (Fig. 5) showed segmental traction angiograms (DSA) on April 7 (Fig. 6B) narrowing originating at the level of the first cer- revealed a complete occlusion of the right internal carotid artery just above the bifurcation. Collateral circulations to the right internal carotid area were well visualized through the right ophthalmic artery and the right middle meningeal artery. She was treated with intravenous infusion of glycerol, and her weakness almost disappeared. The central scotomaand deep sensory loss of the left hand did not improve. She was then treated with a 160 mg dose of aspirin every other day and 0.75 mg carbocon daily, and was discharged on the May 24. Platelet count was 60 x lOVmm3,platelet factor 4 27 ng/ml and thromboxane B2 17 pg/ml upon discharge. For the past 6 months, since her discharge from our hospital, she has been doing well. DISCUSSION Fig. 3. The digital subtraction angiogram of cerebral We diagnosed this patient as having vessels on March 10. A narrowing (arrow) is seen in the fibromuscular dysplasia complicated by essential cervical portion of the right internal carotid artery (A), and thrombocythemia. The combination of these two multiple segmental stenoses and dilatations (arrow) in the cervical portion of the left internal carotid artery (B). disorders is rare. Essential thrombocythemia is Fig. 4. The right carotid angiogram obtained on March 10. An irregular filling defect is seen from the cavernous portion of the internal carotid to the horizontal portion of the middle cerebral artery. Multiple occlusions are seen in the pre-central and the anterior parietal arteries (arrows), and stenoses in the posterior parietal and the angular arteries (arrowheads).
Load more

Recommended publications
  • Arterial Manifestations in Young People
    Arterial Manifestations in Young People Ann Marie Kupinski, PhD RVT RDMS FSVU North Country Vascular Diagnostics, Inc, & Albany Medical College, Albany, NY Objectives Describe arterial pathology encountered in young people Discuss criteria used to diagnose nonatherosclerotic disease entities Present cases which illustrate ultrasound findings of arterial disease in young people Arterial Disease in Young People • Approx. 90% of PAD and extracranial arterial disease is due to atherosclerosis • Nonatherosclerotic diseases can include: • Inflammatory diseases • Non-inflammatory diseases (FMD) • Congenital abnormalities • Acquired diseases • Injuries Testing Options • Ultrasound – Useful with large vessel disease – Giant Cell, Takayasu’s, Radiation arteritis – Injury/Trauma • Physiologic testing (PVR, PPG, pressures) – Useful with small vessel disease Buerger’s Disease (Thromboangiitis obliterans) Vasospastic Disease Fibromuscular dysplasia FMD Noninflammatory Nonatherosclerotic Young individuals (mean onset 48 yrs) Women (3:1) Affects small to medium- sized arteries Intima, media or adventitia FMD Distribution Renal 60-75% Cerebrovascular 25-30% Visceral 9% Extremity Arteries 5% Has also been observed in coronary arteries, pulmonary arteries and the aorta 28% of patients have at least two vascular beds involved Intimal fibroplasia Smooth focal stenosis with a concentric band Long smooth tubular stenosis Poloskey, et al. Circulation 2012;125 Medial fibroplasia Alternating areas of thinned media and thickened fibromuscular
    [Show full text]
  • Renal Artery Fibromuscular Dysplasia Update
    COVER STORY Renal Artery Fibromuscular Dysplasia Update Diagnosis and management of this serious but underdiagnosed disease. BY JOE F. LAU, MD, PHD; ROBERT A. LOOKSTEIN, MD; AND JEFFREY W. OLIN, DO ibromuscular dysplasia (FMD) is a noninflam- prevalence than previously thought. Cragg and associ- matory, nonatherosclerotic arterial disease that ates identified FMD in the renal arteries in 3.8% of 1,862 predominantly affects the renal and carotid arter- potential renal donors who underwent renal angiography.4 ies but has also been identified in almost every Neymark and colleagues found on renal angiography Farterial bed.1,2 FMD may cause arterial stenosis, occlu- that 6.6% (47/716) of potential renal donors had FMD.5 sion, aneurysm, and/or dissection, but many patients are If these studies are accurate and applied to the general likely asymptomatic and may remain undiagnosed. FMD population, and we were to assume an estimated preva- occurs most commonly in women who are between the lence of at least 5% within the United States population ages of 20 and 60, but it can be present at any age and of women over the age of 18, then approximately 5 to 8 occurs in approximately 10% of men.2,3 The prevalence million women may have FMD. of FMD in the general population is unknown; however, Renal artery FMD occurs in about 70% of patients with several lines of evidence suggest that FMD has a higher FMD and is often bilateral. The most common mani- A B Figure 1. A 26-year-old woman with a 6-week history of severe hypertension.
    [Show full text]
  • Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions a Scientific Statement from the American Heart Association
    AHA Scientific Statement Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions A Scientific Statement From the American Heart Association Jeffrey W. Olin, DO, FAHA, Co-Chair; Heather L. Gornik, MD, MHS, FAHA, Co-Chair; J. Michael Bacharach, MD, MPH; Jose Biller, MD, FAHA; Lawrence J. Fine, MD, PhD, FAHA; Bruce H. Gray, DO; William A. Gray, MD; Rishi Gupta, MD; Naomi M. Hamburg, MD, FAHA; Barry T. Katzen, MD, FAHA; Robert A. Lookstein, MD; Alan B. Lumsden, MD; Jane W. Newburger, MD, MPH, FAHA; Tatjana Rundek, MD, PhD; C. John Sperati, MD, MHS; James C. Stanley, MD; on behalf of the American Heart Association Council on Peripheral Vascular Disease, Council on Clinical Cardiology, Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Epidemiology and Prevention, Council on Functional Genomics and Translational Biology, Council for High Blood Pressure Research, Council on the Kidney in Cardiovascular Disease, and Stroke Council ibromuscular dysplasia (FMD) is nonatherosclerotic, pathway. A delay in diagnosis can lead to impaired quality of Fnoninflammatory vascular disease that may result in arte- life and poor outcomes such as poorly controlled hypertension rial stenosis, occlusion, aneurysm, or dissection.1–3 The cause and its sequelae, TIA, stroke, dissection, or aneurysm rupture. of FMD and its prevalence in the general population are not It should also be noted that FMD may be discovered inciden- known.4 FMD has been reported in virtually every arterial bed tally while imaging is performed for other reasons or when but most commonly affects the renal and extracranial carotid a bruit is heard in the neck or abdomen in an asymptomatic and vertebral arteries (in ≈65% of cases).5 The clinical mani- patient without the classic risk factors for atherosclerosis.
    [Show full text]
  • Uncommon Vascular Disorders
    Unusual Vascular Disorders John R. Bartholomew, MD, FACC, MSVM Professor of Medicine – Cleveland Clinic Lerner College of Medicine Section Head – Vascular Medicine Department of Cardiovascular Medicine - Cleveland Clinic Disclosure Statement: I have no financial disclosures related to this lecture Unusual Vascular Disorders These disorders may mimic other more commonly seen diseases. They are important for formulating a differential diagnosis and managing your cardiovascular patient. Non-Atherosclerotic Arterial Vascular Disorders • Fibromuscular Dysplasia • Popliteal Artery Entrapment Syndrome • Cystic Adventitial Disease • External Iliac Artery Endofibrosis • Thromboangiitis obliterans • Segmental Arterial Mediolysis • Uncommon Arteriopathies When should you think of these disorders? - Younger patients - Patients with no traditional risk factors for atherosclerosis Case Report • A 44 year old female presents to your office complaining of a whooshing noise in her ears. She has a history of hypertension but otherwise is in good health. You suspect she might have……. a). Fibromuscular dysplasia b). Meniere's disease c). An acoustic neuroma d). A glomus tumor e). A stroke Fibromuscular Dysplasia or FMD • Affects small to medium sized vessels • Nonatherosclerotic, noninflammatory vascular disease • Affects young to middle aged women • Results in arterial stenosis, occlusion, aneurysm formation or dissection Renal Cerebrovascular Visceral Extremities Hypertension Asymptomatic or Abdominal pain, Intermittent headache, pulsatile weight loss,
    [Show full text]
  • Prevalence of Intracranial Aneurysm in Women with Fibromuscular Dysplasia a Report from the US Registry for Fibromuscular Dysplasia
    Research JAMA Neurology | Original Investigation Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia A Report From the US Registry for Fibromuscular Dysplasia Henry D. Lather, BS; Heather L. Gornik, MD, MHS; Jeffrey W. Olin, DO; Xiaokui Gu, MA; Steven T. Heidt, BA; Esther S. H. Kim, MD, MPH; Daniella Kadian-Dodov, MD; Aditya Sharma, MBBS; Bruce Gray, DO; Michael R. Jaff, DO; Yung-Wei Chi, DO; Pamela Mace, RN; Eva Kline-Rogers, MS, RN, NP; James B. Froehlich, MD, MPH IMPORTANCE The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. OBJECTIVE To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. DESIGN, SETTING, AND PARTICIPANTS This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. MAIN OUTCOMES AND MEASURES Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. RESULTS Of 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years).
    [Show full text]
  • Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia
    Published online: 2019-05-23 THIEME Case Report | Relato de Caso 149 Endovascular Treatment of Ruptured Vertebral Artery Dissecting Aneurysm in Fibromuscular Dysplasia Tratamento endovascular de aneurisma dissecante roto de artéria vertebral na displasia fibromuscular Luana Antunes Maranha Gatto1 DiegodoMonteRodriguesSeabra1 Jennyfer Paulla Galdino Chaves1 Gelson Luis Koppe1 Zeferino Demartini Jr1 1 Department of Neurosurgery and Interventional Neuroradiology, Address for correspondence Luana Antunes Maranha Gatto, MD, Hospital Universitário Cajuru, Curitiba, PA, Brazil Departamento de Neurocirurgia e de Neurorradiologia Intervencionista, Hospital Universitário Cajuru, Rua São José, 300, Curitiba, PA, 80050350, Arq Bras Neurocir 2019;38:149–152. Brazil (e-mail: [email protected]). Abstract Background Fibromuscular dysplasia (FMD) affects predominantly the cervical and Keywords renal arteries and may cause the classical angiographic pattern of string-of-beads. The diagnosis is increasing with the advances of imaging techniques. ► fibromuscular Case Report A 37-year-old man presenting with subarachnoid hemorrhage due to a dysplasia dissecting aneurysm of the vertebral artery was treated by angioplasty with stent, with ► dissecting aneurysm good outcome. All of the cervical and renal arteries were diseased and showed ► endovascular dysplasia and/or ectasias. procedure Conclusions There are no guidelines or protocols to treat patients with FMD. ► carotid stenosis ► angioplasty ► postoperative complications Resumo Introdução A displasia fibromuscular (DFM) afeta predominantemente as artérias Palavras-chave cervicais e renais e pode causar o padrão angiográfico clássico de cordão de contas. O diagnóstico tem aumentado com os avanços das técnicas de imagem. ► displasia Relato de Caso Homem de 37 anos, apresentando hemorragia subaracnoidea por fi bromuscular aneurisma dissecante da artéria vertebral, foi tratado por angioplastia com stent, com ► aneurisma dissecante bom resultado.
    [Show full text]
  • First International Consensus on the Diagnosis and Management Of
    VMJ0010.1177/1358863X18821816Vascular MedicineGornik, Persu et al. 821816research-article2019 Consensus Document Vascular Medicine 1 –26 First International Consensus on the diagnosis © The Author(s) 2019 Article reuse guidelines: and management of fibromuscular dysplasia sagepub.com/journals-permissions https://doi.org/10.1177/1358863X18821816DOI: 10.1177/1358863X18821816 journals.sagepub.com/home/vmj Heather L Gornik (Co-Chair)1*, Alexandre Persu (Co-Chair)2*, David Adlam3,4, Lucas S Aparicio5, Michel Azizi6,7,8, Marion Boulanger9, Rosa Maria Bruno10, Peter de Leeuw11, Natalia Fendrikova-Mahlay1, James Froehlich12, Santhi K Ganesh12, Bruce H Gray13, Cathlin Jamison14, Andrzej Januszewicz15, Xavier Jeunemaitre16,17, Daniella Kadian-Dodov18, Esther SH Kim19, Jason C Kovacic18, Pamela Mace20, Alberto Morganti21, Aditya Sharma22, Andrew M Southerland23, Emmanuel Touzé9, Patricia van der Niepen24, Jiguang Wang25, Ido Weinberg26, Scott Wilson27,28, Jeffrey W Olin18**, and Pierre-Francois Plouin6,7,8**, on behalf of the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM) 1 Division of Cardiovascular Medicine, Department of Cardiovascular 19Division of Cardiovascular Medicine, Vanderbilt University Medical Medicine, University Hospitals Cleveland Medical Center and UH Center, Nashville, TN, USA Harrington Heart and Vascular Institute, Cleveland, OH, USA 20Fibromuscular Dysplasia Society of America (FMDSA), North Olmsted, 2Division of Cardiology, Department of
    [Show full text]
  • Three Phases of the Fibromuscular Dysplasia Experience
    Central JSM Atherosclerosis Bringing Excellence in Open Access Case Report *Corresponding author Sherry M. Bumpus, School of Nursing, Eastern Michigan University, 340 Everett L. Marshall Building, Ypsilanti, MI, Stigma, Fear, and Acceptance: USA, Tel: 734-487-2279; Email: Submitted: 27 September 2016 Three Phases of the Accepted: 15 January 2017 Published: 16 January 2017 Fibromuscular Dysplasia Copyright © 2017 Bumpus et al. Experience OPEN ACCESS Sherry M. Bumpus1,2*, Rachel Krallman2, Steven Heidt2, and Eva Keywords Kline-Rogers2 • Fibromuscular dysplasia • Mental health 1 School of Nursing, Eastern Michigan University, USA • Quality of life 2 Department of Internal Medicine-Cardiology, University of Michigan Health System, • Vascular diseases USA Abstract Fibromuscular Dysplasia (FMD) is an arteriopathy that can affect any vascular territory, though most often affects the renal and carotid arterial beds. Symptoms are consistent with the vascular bed affected and are often attributed to other conditions, leading to delays in diagnosis. We present a case of a middle-aged woman who presented to the emergency department (ED) with altered mental status and confusion, with an ED discharge diagnosis of altered mental state secondary to depression and alcohol abuse. During follow-up testing, she was diagnosed with hypertensive encephalopathy and, subsequently, FMD. The true prevalence of FMD is unknown, although current estimates vary from 3-4%. Like other “uncommon” disorders, appropriate diagnoses are often delayed or missed altogether. This delay to diagnosis, in combination with non-specific symptoms and providers’ unfamiliarity with FMD, leads to frustration for many patients. A recent FMD qualitative publication revealed patients’ concerns about physical symptoms along with greater frequency of anxiety and depression.
    [Show full text]
  • 10 Tips Doctors Should Know About Fibromuscular Dysplasia (FMD)
    O M U S B R C U I L F A R • D A Y C S I P R L E A M S A I A F S O O Y C T I E FMDSA • FIBROMUSCULAR DYSPLASIA SOCIETY OF AMERICA • 10 Tips Doctors Should Know About Fibromuscular Dysplasia (FMD) 1. FMD is nonatherosclerotic and noninflammatory disease which can affect arteries in every vascular bed, but it most commonly involves renal arteries and internal carotid and/or vertebral arteries. FMD can present as stenosis, aneurysm, dissection and/or tortuosity. 2. FMD is a diagnosis made by imaging. Good quality CT angiography, MR angiography, duplex ultrasound, or their combination is an important part of the evaluation. Catheter-based angiography remains the gold standard in diagnosing FMD, but it is usually reserved for selected cases. One time, head to pelvis imaging (with CT or MR Angiography) is recommended to determine which arteries are affected and to check for aneurysms and dissections of arteries. 3. Hypertension and headache are two of the most prevalent manifestations in FMD patients. FMD patients who do not have carotid, vertebral or intracranial involvement can still have headaches. FMD patients can have essential (primary) hypertension not caused by renal FMD (in other words, non-renovascular hypertension). Pulsatile tinnitus, a “swooshing” noise in the ears timed to the heart beat, is a common symptom among patients with carotid and vertebral artery FMD. Another common sign of FMD is a bruit heard with a stethoscope over an artery on physical examination. 4. Most patients with FMD are managed conservatively with medical therapy and surveillance.
    [Show full text]
  • Progressive Cerebral Vasculopathy and Recurrent Strokes Due to Intracranial fibromuscular Dysplasia T ⁎ Nicole B
    Interdisciplinary Neurosurgery 15 (2019) 19–21 Contents lists available at ScienceDirect Interdisciplinary Neurosurgery journal homepage: www.elsevier.com/locate/inat Neuroanatomical Studies Progressive cerebral vasculopathy and recurrent strokes due to intracranial fibromuscular dysplasia T ⁎ Nicole B. Sur, MDa, , Sakir H. Gultekin, MDb, Amer M. Malik, MDa, Sebastian Koch, MDa a University of Miami, Department of Neurology, 1120 NW 14th St, 13th Floor, Miami, FL 33136, United States of America b University of Miami, Department of Pathology, Holtz Children's Hospital, 1611 NW 12th Ave, R#2044, Miami, FL 33136, United States of America ARTICLE INFO Keywords: Fibromuscular dysplasia Ischemic stroke Cerebrovascular disease Rare causes of stroke Cerebral vasculopathy 1. Introduction difficulty and mild right hemiparesis. Lab workup revealed normal complete blood count, chemistry, lipid Fibromuscular dysplasia (FMD) is a non-atheromatous, non-in- panel and glycosylated hemoglobin (A1C). Thyroid stimulating hor- flammatory vasculopathy of unknown etiology, affecting primarily mone (TSH) was low and free T4 level was normal. C reactive protein small and medium sized arteries [1]. FMD is typically seen in women (CRP) was elevated at 7(?units?) and erythrocyte sedimentation rate and the renal, extracranial carotid and vertebral arteries are most (ESR) and antinuclear antibody (ANA) were normal. EKG revealed commonly affected. Very rarely, FMD has been reported in the in- normal sinus rhythm. Brain MRA at our facility showed MCA stenosis tracranial carotid arteries and it is here that we report on a case of along the distal M1 and proximal M2 branches. Subsequent digital recurrent ischemic stroke in a middle-aged woman with autopsy-proven subtraction angiogram (DSA) showed mild bilateral segmental beading intracranial FMD.
    [Show full text]
  • Fibromuscular Dysplasia (FMD) Fact Sheet
    Fibromuscular Dysplasia (FMD) Fact Sheet What is Fibromuscular Dysplasia? Fibromuscular dysplasia (also known as FMD) is a medical condition characterized by abnormal cell growth within the arterial wall. FMD is different from other blood vessel disorders that affect the arteries, such as atherosclerosis (blockage of arteries secondary to cholesterol plaque), vasculitis (inflammation of the arteries), and thrombosis (formation of blood clots). FMD can lead to different abnormalities of arteries, such as narrowing, a beaded appearance, or even serious complications such as aneurysms (bulging of the arteries) or dissections (tears of the arteries). FMD is not known to involve the veins or the lymphatic system. What causes Fibromuscular Dysplasia? The cause of FMD is not yet clearly known. Likely genetic and environmental factors play a role. FMD is much more common in women (Approximately 90% in the US Registry for FMD). What population does it affect? Predominately middle-aged women, but it has presented across the lifespan of men, women and children What are common signs and symptoms? High blood pressure in patients under age 40, or uncontrolled high blood pressure at any age Unrelenting headaches (migraine type), neck pain, dizziness, or asymmetry of the pupils A pulsatile whooshing or swooshing sound in the ears (pulsatile tinnitus) A bruit (abnormal sound when listening with a stethoscope) in the abdomen (abdominal bruit) or neck (cervical bruit); findings suggest there is turbulence of blood flow in the artery; Dissection or aneurysm of an artery Transient ischemic attack (TIA) or stroke Asymptomatic (no symptoms present) FMD can be silent and be detected by testing during a routine physical.
    [Show full text]
  • Variable Clinical Spectrum of Fibromuscular Dysplasia of the Brachial Arteries
    Variable Clinical Spectrum of Fibromuscular Dysplasia of the Brachial Cleveland, Ohio Arteries. Ana Casanegra, MD1, Vikram Kashyap, MD2, Sandra Yesenko, BA, RVT1, Carmela Tan, MD3, Heather L. Gornik, MD, MHS1 1: Vascular Medicine Section, Department of Cardiovascular Medicine, 2: Department of Vascular Surgery, 3: Department of Anatomic Pathology. Cleveland Clinic, Cleveland, Ohio. Abstract Case 1 Case 2 Findings 62 year-old female 63 year-old female Figure 4: Brachial Background: Fibromuscular dysplasia (FMD) is an uncommon artery surgical vascular disorder most frequently manifest in the renal and carotid • Referred to FMD clinic for a second opinion. • Developed acute pain and paleness in her left arm from the elbow to pathology. arteries. Involvement of the upper extremity arteries has been • FMD was diagnosed 15 years before with a carotid ultrasound and the hand. Hematoxylin & Eosin reportedly rarely in the medical literature and is usually unilateral. We (Panel A) and Movat’s subsequent angiogram as workup for pulsatile tinnitus. • Patient was anticoagulated and transferred to our institution. identified two patients in a single center with bilateral brachial FMD. stain (panel B) with elastic fibers in black. Case 1: 62 year-old woman with pulsatile tinnitus due to FMD of • She had known FMD involvement of internal carotid and renal • Cardioembolic sources were ruled out, as well as hypercoagulable arteries bilaterally. Arrowheads mark the bilateral internal carotid arteries. She also had renal artery FMD with states. external elastic well-controlled hypertension on two agents. She was found to have a • HTN controlled with two antihypertensive medications. • Upper extremity angiogram demonstrated bilateral beaded lamina.
    [Show full text]