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Peripheral Vascular Anomalies, Malformations, and Vascular Tumors

Nonmalignant vascular anomalies can be functionally divided into two groups: vascular tumors and vascular malformations. Unfortunately, this distinction is not universally appreciated, due to “terminologic imprecision.” Despite efforts by vascular anomalies specialists to publicize the accurate lexicon, the term hemangioma (a vascular tumor) is often incorrectly applied to describe vascular malformations. The original classification proposed by Mulliken and Glowaki has since been updated to include syndromic…

Vascular Compression Syndromes

Vascular compression syndromes are uncommon conditions caused by narrowing or occlusion of vascular structures by adjacent tissues in disparate regions of the body. This chapter will focus on the anatomy, pathophysiology, and management of the more commonly encountered vascular compression syndromes, namely, vascular thoracic outlet syndromes (TOSs), median arcuate ligament syndrome (MALS), nutcracker syndrome, May-Thurner syndrome, popliteal entrapment syndrome, and cystic adventitial disease. Thoracic outlet syndrome…

Vascular Trauma

Vascular trauma occurs in varying injury patterns, with involved structures ranging from large caliber, high-flow central vessels to small, peripheral extremity vessels. Due to the variability in affected vessels and concomitant injuries, management strategies can vary considerably. Often, evaluation and treatment of vascular trauma occurs concurrently with that of other associated injuries. Therefore, management of vascular trauma requires knowledge of specific techniques for vascular repair throughout…

Lower Extremity Ulceration

Ulceration of the lower extremity is a relatively common condition that causes significant discomfort and disability. An ulcer is defined as a disruption of the skin with erosion of the underlying subcutaneous tissue. This breach may extend further to the contiguous muscle and bone. The pathophysiologic mechanisms underlying ulcer formation are multifactorial and include neuropathy, infection, ischemia, and abnormal foot structure and biomechanics. It is not…

Dermatological Manifestations of Vascular Disease

In the modern-day era of medical diagnosis facilitated by sophisticated radiographic imaging and/or comprehensive serological testing, a thorough physical examination remains vital in recognizing the dermatological manifestations of chronic vascular diseases. Accurately identifying cutaneous signs of lymphovenous hypertension and peripheral artery disease (PAD) assists in their diagnosis as well as associated staging and classification. Similarly, the vasomotor diseases are principally diagnosed by their unique and often…

Vascular Infections

Vascular infections represent difficult diagnostic and technical challenges for even the most experienced physicians. Multiple comorbidities frequently afflict the patients, resulting in nutritional and immunologic deficiencies. Patients are often near the end of life as well, further complicating the decision-making for the patients, caregivers, and physicians. Moreover, published series regarding vascular infections are underpowered, which limits the ability for physicians to provide data-driven care. Vascular infections…

Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) is a nonatherosclerotic vascular disease that primarily affects medium-sized arteries but can be observed in nearly every artery of the body. It is not known to involve the venous system. Although FMD most commonly presents in middle-aged women, it can affect both sexes, as well as children and the elderly. The clinical manifestations of FMD are variable based upon the type and severity…

Diseases of the Lymphatic Circulation

Diseases of the lymphatic circulation reflect either intrinsic, presumptively heritable (primary) defects or the aftermath of an acquired (secondary) disruption of lymphatic structure or function. Primary lymphatic disorders are thought to arise from faulty development of the lymphatic vasculature or from intrinsic functional derangements. Without regard to the mechanism, each of these disorders results in a disturbance in lymph transit. Secondary lymphatic dysfunction most often occurs…

Pulmonary Hypertension in Patients With Nonpulmonary Arterial Hypertension

Overview of pulmonary hypertension Definition and Nomenclature Pulmonary hypertension (PH) is defined classically as a sustained mean pulmonary arterial blood pressure (mPAP) ≥ 25 mm Hg measured in the supine position by right heart catheterization. The PH clinical syndrome typically includes dyspnea, diminished exercise capacity, and hypoxemia, which may result from several different pathophysiologic and molecular mechanisms. The contemporary PH classification system was created by an…

Pulmonary Arterial Hypertension

Definition and classification of pulmonary arterial hypertension Until 2018, pulmonary arterial hypertension (PAH) was formally defined as a mean pulmonary artery pressure (mPAP) of greater than or equal to 25 mm Hg at rest, accompanied by a pulmonary vascular resistance (PVR) of greater than 3 Wood units with a normal pulmonary artery wedge pressure (<15 mm Hg) in the absence of any known cause of pulmonary…

Chronic Venous Insufficiency

Chronic venous insufficiency (CVI) refers to clinical conditions of varying severity, from varicose veins at one end of the spectrum to venous ulceration. Advanced chronic venous disease (CVD) has a more severe clinical presentation than simple varicose veins, reticular veins, or telangiectasia but may be part of the clinical syndrome of CVI that typically presents with swelling, skin changes, and/or leg ulcers. The prevalence of varicose…

Varicose Veins

Epidemiology Varicose veins (VVs) are tortuous, dilated, bulging, superficial veins typically measuring 3 mm or larger. They are the most common manifestation of chronic venous insufficiency (CVI) and affect up to 25% of women and 15% of men. In the Framingham Study, which includes men and women between the ages of 30 and 62 from the town of Framingham, Massachusetts, the annual incidence of VVs is…

Management of Venous Thromboembolism

Optimal management of venous thromboembolism (VTE) hinges upon understanding the medical, economic, and psychosocial consequences of pulmonary embolism (PE) and deep vein thrombosis (DVT). In an international registry of 23,858 patients with acute PE over 13 years, the mean length of hospital stay decreased 32% and the risk of all-cause 30-day mortality decreased from 6.6% to 4.9%. The risk-adjusted 30-day PE-related mortality decreased from 3.3% to…

Clinical Evaluation of Venous Thromboembolism

Venous thromboembolism (VTE), most often presenting as deep vein thrombosis (DVT) or pulmonary embolism (PE), is a frequently encountered cardiovascular disorder. After myocardial infarction and stroke, VTE is the third most common cardiovascular disease with an estimated 10 million cases per year globally. VTE is prevalent across all ages; however, the incidence increases significantly after the age of 60, with an 8% lifetime risk in those…

Epidemiology of Venous Thromboembolic Disease

Incidence and clinical relevance of venous thromboembolism Venous thromboembolism (VTE), consisting of deep vein thrombosis (DVT) and pulmonary embolism (PE), is the third leading cardiovascular disease after coronary heart disease and stroke. The age-standardized incidence rate is 1 to 2 per 1000 people per year. There is no surveillance for VTE, so the precise incidence and prevalence are not clear; three data sources present disparate results…

Pernio (Chilblains)

Pernio , commonly known as chilblains , is a cold-induced localized inflammatory condition presenting as skin lesions predominantly on unprotected acral areas. Typically there is swelling of the dorsa of the proximal phalanges of fingers and toes ( Fig. 49.1 ). Pernio is a Latin term meaning “frostbite.” Chilblains is an Anglo-Saxon term used in older literature and means “cold sore.” The tissue and vascular damage…

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…