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Erythromelalgia

Definition and historical perspective Erythromelalgia is a rare condition of the extremities characterized by the triad of redness, warmth, and pain. The symptom complex of intermittent acral warmth, pain, and erythema that defines erythromelalgia has been well documented in the medical literature for more than 150 years. Graves described cases of “hot and painful legs” in 1834. The term erythromelalgia was coined in 1878 by Mitchell…

Acrocyanosis

The term acrocyanosis is derived from the Greek words akron (meaning “extremity”) and kyanos (meaning “blue”). As a medical condition, acrocyanosis is an uncommon functional vasospastic disorder characterized by persistent bluish discoloration, primarily of the hands and feet. However, the term acrocyanosis is often used to describe diverse conditions and lacks a uniform definition. The original description of acrocyanosis is credited to Crocq in 1896, thus…

Raynaud Phenomenon

In its simplest form, local syncope is a condition perfectly compatible with health. Persons who are attacked with it are ordinarily females. Under the least stimulus, sometimes without appreciable cause, one or many fingers become pale and cold all at once; in many cases, it is the same finger that is always first attacked; the others become dead successively and in the same order. It is…

Atheroembolism

Atheroembolism is a rare but serious disorder with significant morbidity from stroke, renal failure, and limb loss. This systemic disorder affects multiple organs and carries a high mortality rate. Atheroembolism can be a single event or recurrent. It can occur spontaneously or following an invasive vascular procedure. It can originate from atherosclerotic or aneurysmal disease and involve single or multiple sites. There is no specific laboratory…

Acute Arterial Occlusion

Nontraumatic, acute occlusion of arterial supply to a limb or organ presents with a constellation of symptoms specific to the tissue suddenly deprived of arterial perfusion. Irrespective of the arterial segment involved, this syndrome represents a vascular emergency. Irreversible organ injury may occur within seconds in the case of acute embolic occlusion of a middle cerebral artery or take hours when arterial supply of a lower…

Kawasaki Disease

Kawasaki disease (KD), initially described by Dr. Tomisaku Kawasaki in 1967, is an acute systemic vasculitis of uncertain etiology that predominantly affects infants and young children. The disease has been described worldwide and occurs in all populations. The acute illness is self-limited and is characterized by a nonexudative conjunctivitis, inflammation of the oral mucosa, rash, cervical adenopathy, and findings in the extremities, including swollen hands and…

Thromboangiitis Obliterans (Buerger Disease)

Thromboangiitis obliterans (TAO) describes a segmental, nonatherosclerotic inflammatory disorder that primarily involves the small and medium arteries, veins, and nerves of the extremities. Although TAO was initially thought to be a disease confined exclusively to men, epidemiologic studies demonstrate a growing population of women with the disorder. Also known as Buerger disease, TAO has an extremely strong pathophysiological relationship with tobacco use, usually in the form…

Medium and Small Vessel Vasculitis

Medium vessel vasculitis Polyarteritis Nodosa Polyarteritis nodosa (PAN) is a medium vessel vasculitis affecting the main visceral arteries and their branches. The 2012 International Chapel Hill Consensus Conference (CHCC) defines PAN as a necrotizing arteritis of medium and small arteries without glomerulonephritis or vasculitis in arterioles, capillaries, or venules, and not associated with antineutrophil cytoplasmic antibodies (ANCA). The association of PAN with hepatitis B virus (HBV)…

Large Vessel Vasculitis

Acknowledgment The authors acknowledge Dr. Kathleen Maksimowicz-McKinnon and Dr. Gary S. Hoffman for their contributions to parts of this chapter in an earlier edition of this textbook. Large vessel vasculitis refers to a group of diseases involving inflammation of the aorta and/or its major large artery branches not caused by infection, radiation, or trauma. These diseases may come to the attention of vascular medicine specialists through…

Overview of Vasculitis

The vasculitides are a group of rare diseases linked by the pathological consequences of vascular inflammation, including bleeding, ischemia, and infarction of downstream organs ( Box 39.1 ). However, the clinical spectrum of these diseases is wide ranging and includes a myriad of clinical and pathological findings. Not all disease phenotypes that occur in the vasculitides are due to true “vasculitis” (i.e., inflammation of vascular structures).…

Endovascular Therapy for Abdominal Aortic Aneurysm

The surgical treatment of abdominal aortic aneurysms (AAAs) dates back centuries. Some of the initial approaches involved techniques similar, in some fashion, to modern endovascular techniques. In 1684, Moore reported on the use of large quantities of wire placed intraluminally into the aneurysm sac in order to induce thrombosis of AAA. Later, electrical currents were passed through the wire to further promote thrombosis. A self-expanding endoluminally…

Surgical Treatment of Abdominal Aortic Aneurysms

Abdominal aortic aneurysms (AAAs) remain a leading cause of death in the elderly. In the United States, ruptured AAAs are the 15th leading cause of death overall and the 10th leading cause of death in men older than age 55. AAAs account for more than 5500 hospital deaths in the United States, which likely underestimates their true number because 30% to 50% of all patients with…

Clinical Evaluation of Aortic Aneurysms

The vast majority of aortic aneurysms are asymptomatic, accounting for a much higher disease prevalence than hospitalization and mortality statistics would suggest (see Chapter 35 ). These data underscore the central challenge in aortic aneurysmal disease: a common clinical problem that is silent until rupture and death. Aortic aneurysms typically increase in size slowly over years or decades, with few warning signs. The management of aortic…

Epidemiology and Prognosis of Aortic Aneurysms

Aortic aneurysms result in significant morbidity and mortality, accounting for nearly 10,000 deaths and 69,000 hospital discharges per year in the United States. A wide variety of pathological states are associated with aortic aneurysms, including degenerative diseases, genetic disorders, infections, inflammatory conditions, and trauma. Although aneurysms may affect any part of the aorta from the aortic root to the abdominal aorta, the prognosis and outcome in…

Endovascular Therapy for Aortic Dissection

Acute aortic dissection (AAD) is a precipitous event associated with a wide range of outcomes from uncomplicated to catastrophic. Current endovascular strategies are based on identifying features that portend increased risk of death or other poor outcome, and applying interventional techniques to prevent the life-threatening complications of the dissection. During the last 2 decades, there has been increasing interest in exploring endovascular procedures for management of…

Surgical Therapy for Aortic Dissection

Morgagni described the first cases of aortic dissection in 1773, and Maunoir coined the entity “aortic dissection.” Despite these early reports of thoracic aortic disease, it was not until 1952 that Drs. De Bakey and Cooley first successfully operated on a patient with a descending thoracic aortic aneurysm using a lateral resection. In 1956 De Bakey and Cooley replaced the ascending aorta using cardiopulmonary bypass and…

Pathophysiology, Clinical Evaluation, and Medical Management of Aortic Dissection

Acute aortic dissection is an uncommon but life-threatening emergency that requires prompt diagnosis, rapid triage, and immediate treatment. A unified effort across several international centers over the past 20 years has resulted in the establishment of a detailed, prospective registry that describes the major aspects of the presentation, management, and outcomes of patients with acute aortic dissection. This longitudinal experience has led to important clinical insights…

Carotid Artery Revascularization

The management of carotid artery disease remains a topic of current investigation and vigorous debate regarding optimal treatments. Multiple prospective randomized controlled trials have compared the standard therapies, which include carotid endarterectomy (CEA), carotid artery stenting (CAS), and best medical therapy (BMT). In addition, hybrid procedures to treat carotid artery stenosis have been added to the therapeutic armamentarium. This chapter focuses on the evidence base, clinical…

Prevention and Treatment of Stroke

The medical management of stroke encompasses a wide range of therapies that include managing physiological parameters in the acute phase, reducing the extent of acute injury, and preventing recurrent strokes. Ischemic stroke is the most common form of cerebrovascular disease and will be the focus of this chapter. Only a brief mention of primary intracerebral hemorrhage (ICH) with regard to acute management and secondary prevention will…

Clinical Presentation and Diagnosis of Cerebrovascular Disease

Stroke is a common and serious disorder. Each year stroke affects almost 800,000 people in the United States, at least 16 million people globally, and is the second leading cause of death in the world (see Chapter 28 ). The associated high morbidity and mortality of stroke, combined with its high cost for acute and chronic care, provide impetus for improving the diagnosis, acute management, and…