Vascular leg pain – Venous disease
Acute deep vein thrombosis
In many patients who present with DVT there is a history of predisposing risk factors including previous DVT, specific hypercoagulable states, pregnancy, malignancy or events such as a prolonged recumbency during travel, injury to the leg, hospitalization or recent surgery. Leg pain from DVT may first be noticeable upon arising after a night’s rest or a long trip, or may have a more insidious onset. In general, the pain is constant and aching in quality, located in the muscle compartment surrounding and distal to the obstruction, with an element of sharpness referable to the involved vein segments. The patient will generally report aggravation of the pain with walking and dependency and some relief with elevation of the limb. Swelling is often reported and cyanosis from venous congestion may have been noticed. With complete ileofemoral obstruction resulting in Phlegmasia alba dolens and subsequent Phlegmasia cerulea dolens, pain will evolve into neuropathic numbness and paralysis as actual tissue ischemia supervenes on the basis of absence of sufficient venous outflow from the limb.
On inspection, swelling of the lower extremity may be evident, with an element of cyanosis and plethora. The swollen and involved portions of the leg will be tense and tender on the basis of venous inflammation and increased compartment pressure. Low-grade fever may be present. Flexion of the ankle may produce discomfort in the calf in the case of calf thrombosis (Homans’ sign), though this finding is non-specific. An enhanced pattern of superficial venous channels may indicate presence of collaterals stimulated by obstruction of the deeper proximal veins. Elevation of the abnormal limb in tandem with the normal limb may demonstrate persistent filling of the superficial foot veins in the obstructed leg at a level when the normal leg veins have emptied. In extreme cases of ileofemoral thrombosis, the limb may have reduced arterial pulses or be pulseless, with evidence of sluggish flow in the toes and forefoot with impending capillary stasis and infarction of tissue.
It is important to recall that the clinical presentation of acute venous occlusion is notoriously non-specific, and that many patients experience significant venous thrombosis with no signs or symptoms whatsoever. Most prominent in the differential diagnosis are the postphlebitic syndrome, superficial thrombophlebitis, cellulitis and muscle or ligamentous injury.
The most accurate means of differentiating DVT from other conditions is duplex ultrasound. Ultrasound performed in an experienced laboratory can identify both anatomic obstruction and physiological changes in flow with over 90% accuracy in veins proximal to the calf. Calf veins are also often visible, though with a somewhat higher incidence of inaccuracy. Though not routinely imaged in most peripheral vascular laboratories, the iliac veins and inferior vena cava may be interrogated indirectly by characterization of ultrasound flow patterns at the femoral level. Impedance plethysmography, pneumoplethysmography and phleborheography are less available but useful indirect means of identifying significant proximal venous obstruction. MRI may also be helpful, as may contrast venography, although both of these modalities are relatively expensive and unpleasant and add little to the initial diagnostic evaluation. All modalities are confounded to variable extents by the differential between acute thrombus and old postphlebitic changes in the vein.
A sizable minority of patients with venous thrombosis have a hypercoagulable state for which anticoagulation may be best prolonged. Laboratory assays should be performed for antiphospholipid syndrome, activated protein C resistance (factor V Leiden) and prothrombin G20210A mutant.
Early management consists of prompt presumptive diagnosis followed by bed rest with leg elevation above the level of the heart. Confirmatory duplex ultrasound should be performed in conjunction with anticoagulation using intravenous unfractionated heparin or subcutaneous low-molecular-weight heparin in order to halt the process of thrombosis and enhance the natural fibrinolytic cascade which will result in partial or complete recanalization of the vein in the great majority of patients. Ordinarily, bed rest with elevation and heparin will result in improvement in symptoms due to decrease in swelling and inflammation and increase in collateral. Intravenous heparin or subcutaneous low-molecular-weight heparin should be continued until coumadin, which may be initiated on day 2 or 3, has resulted in elevation of prothrombin time and international normalized ratio (INR) to 18–20 s and 2.5–3.0, respectively. Mobilization of the patient is usually appropriate when the pain and swelling are sufficiently improved. Consultation with a vascular or hematology specialist may be appropriate should there be unusual circum-stances such as phlegmasia, failure to respond, evidence of pulmonary embolus or difficulty with persistent symptoms. Selected patients may be treated with surgical thrombectomy or percutaneous suction thrombectomy and fibrinolysis. In general, inferior vena caval filters are not indicated unless there is a firm contraindication to anticoagulation or evidence of pulmonary embolus while adequately anticoagulated.
Superficial thrombophlebitis involves branches of the veins in the subcuta-neous tissue. Though sometimes associated with systemic disease, classically malignancy (Trousseau’s syndrome), superficial phlebitis is more common among patients with a recent injury at the site of a superficial vein or with pre-existing varicose veins. A particularly bothersome time for superficial phlebitis is during the later stages of pregnancy. A minority of patients with superficial phlebitis have systemic hypercoagulable states.
The typical history is abrupt onset of sharply focused pain located in a linear pattern in the general distribution of the superficial venous trunks such as the greater saphenous or lesser saphenous vein. The region is indurated, red, very tender and surrounded by local subcutaneous swelling. There may be more than one focus of inflammation or evidence of previous burned out episodes from chronic hyperpigmentation and induration. Varicose veins are often present. In a minority, phlebitis may also involve the deep veins, either by direct extension or as part of a more generalized illness involving two sites simultaneously.
Superficial phlebitis is a non-bacterial sterile process. The most common alternative diagnoses in the differential are soft tissue cellulitis and traumatic contusion. Septic thrombophlebitis occurs rarely and usually is related to intravenous access involving the upper extremity. Presence of a history of venipuncture or drug abuse, high fever, diffuse linear pattern along the course of the vein and purulent drainage from a venipuncture site make septic phlebitis highly suspect.
The diagnosis of superficial phlebitis is nearly always clear enough on clinical grounds to make confirmatory laboratory study unnecessary. Nonetheless, duplex ultrasound can confirm phlebitis in a superficial vein and exclude concurrent involvement of the deep veins. Ultrasound is most valuable among patients with superficial phlebitis near the popliteal space or saphenofemoral junction in whom contiguous deep veins may be involved.
A review of the patient’s general health is important to exclude an under-lying predisposing condition such as malignancy. Superficial phlebitis is best managed with non-steroidal anti-inflammatory medications such as indomethacin (25–50 mg tid) together with rest and elevation. Topical heat may help the symptoms. There is no role for antibiotics. The clinical picture should be followed closely to be certain that there is improvement rather than extension of the process. Should there be no improvement, increased doses of non-steroidals or intravenous heparin may be necessary. In selected resistant cases, superficial phlebitis may be treated by surgical excision of the involved vein. Care of pregnant patients must be coordinated with the involved obstetrician.
Chronic deep venous insufficiency
Chronic obstruction or valvular incompetence of the deep veins is most commonly manifest as the postplebitic syndrome resulting from previous DVT, at times undocumented. A sizable minority of patients with deep venous insufficiency have primary valvular incompetence unrelated to DVT. The pain is typically located at the distal portion of the leg near the calf and ankle and consists of heaviness, tightness or pressure within the muscle compartments. It is aggravated by dependency and relieved by elevation. There is often associated swelling, varicose veins, and hyperpigmentation in the skin. Over time, eczema and ultimately ulceration with cellulitis supervene around the medial portion of the ankle just above the malleolus. In the small number of patients with chronic venous obstruction at a proximal level, swelling may be more prominent. Venous claudication may occur with such proximal obstruction, with a tightness and bursting sensation which is relieved after cessation of exercise as the leg empties via venous collaterals.
Examination may be completely normal, but most often a pattern of mild swelling over the ankle, varicosities, hyperpigmentation (‘brawny induration’) in the skin of the distal calf and ankle with a medial preponderance indicate the presence of longstanding deep venous insufficiency. Eczema and weeping or overt ulceration with surrounding cellulitis may be present.
Laboratory non-invasive study by duplex ultrasound provides valuable confirmation of the presence of deep venous scarring, obstruction or valvular incompetence. A baseline examination is generally valuable for later compari-son in the event of the need for differentiation between chronic stable post-phlebitic syndrome and superimposed acute DVT. Further anatomic study using MRI or venography may be appropriate should surgical intervention be recommended after consultation.
Symptomatic relief is generally provided by a combination of frequent limb elevation and external elastic compression by means of knee length ace bandage or 30–40 mmHg graduated compression support stockings. Antibiotics, possibly by intravenous route in severe cases, are important in the presence of inflammation with or without ulceration. Intermittent gauze dressings with wet to dry saline or an occlusive dressing using one of a variety of available products can reduce pain and exudate and foster healing of eczema and ulcers. The Unna paste boot is a reliable option which provides both topical dressing and support to the leg while assuring patient compliance and convenience. Early consultation with an appropriate specialist is usually indicated when ulceration is present. Life-long knee length elastic support is a prerequisite for successful management.
Chronic superficial venous disease
Varicose enlargement of the superficial veins (greater saphenous and lesser saphenous) presents as a syndrome of localized heaviness and aching after
prolonged standing. Elevation or external elastic support generally provides relief. A localized injury may result in superficial phlebitis. A few patients with extraordinarily superficial thin-walled tributaries may experience painless bleeding from the varicosities. Patients with secondary varicose veins will generally have a history of associated deep venous insufficiency and previous DVT. In the case of primary varicose veins, most patients present in their third or fourth decades and have a family history of varicose veins. The veins follow a course of gradual progression, greatly exacerbated by pregnancies in the case of females.
Examination is best performed with the patient standing. The varicosities are most prominent distal to the knee where branches are larger and more superficial. There may be some focal areas of hyperpigmenation over the shin and medial ankle. Swelling is generally absent or mild unless there is deep venous involvement. The source of filling of the varicose system can be demonstrated by Trendelenberg test. The leg is elevated to empty the veins and a tourniquet is applied to prevent refilling of the vein from a superficial source when the leg is returned to a dependent position. If such refilling occurs despite the tourniquet, it must be via an incompetent perforator resulting from deep vein disease.
Because physical findings are usually obvious, laboratory has a limited role in confirming the diagnosis of superficial venous disease. In cases where the history and physical suggest that the varicosities are secondary rather than primary, duplex ultrasound may help characterize the deep venous abnormalities and identify any important perforating veins filling the superficial varicosities.
Early therapy consists of reassurance with respect to any particular threat of blood clots or limb jeopardy. Support garments of a sensible nature such as pantyhose or leotards may completely relieve symptoms. With sufficient symptomatology, consultation may be appropriate, particularly in younger individuals in whom the problem usually progresses with time. If veins are relatively isolated and small, injection sclerotherapy or laser ablation may be helpful. Larger varicosities related to one of the saphenous trunks are generally best treated with surgery to ligate and remove (‘strip’) the veins.
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