33: Chronic Venous Insufficiency & Lymphedema

From epidemiologic studies, the prevalence of chronic venous insufficiency (CVI) is estimated to be between 5% and 30% in the general population. CVI is more common in women than in men, with a ratio of approximately 3:1. United States expenses related to CVI have been estimated at $1.9 billion to $2.5 billion annually.

The venous system is made up of deep veins within the subfascial, muscular compartment of the limbs, superficial veins, which are located in the epifascial, subcutaneous compartment and perforator veins, which communicate between the 2 compartments. Normal venous outflow depends on patency of the veins, intact venous valves, and a normal functioning calf muscle pump to return blood from the periphery to the right side of the heart.

CVI results venous hypertension or sustained venous pressure within the deep or superficial venous system. Venous hypertension may be related to failure of any of the required components: abnormal or damaged venous valves and reflux, venous outflow obstruction either as a result of intrinsic or extrinsic injury, or loss of the normal calf muscle pump. Venous insufficiency may be primary or secondary. Risk factors for CVI include advancing age, obesity, pregnancy, history of lower-extremity injury, and prolong standing or dependency.

Patients with limited mobility, using walking aids, stroke, or using ankle-foot orthoses will frequently have decreased calf muscle pump and secondary CVI. Patients should always be questioned regarding sleeping habits. Chair or recliner sleeping is common in older adults because of back or joint pain, limited mobility, cardiopulmonary disease, or poor sleep habits.

Postthrombotic syndrome is 1 form of venous insufficiency related to valve damage or incomplete recanalization of the veins following deep vein thrombosis or phlebitis. Many venous thromboses are asymptomatic and the patient may not be aware of the risk for injury. This can be easily identified using duplex ultrasound.

Signs & Symptoms

Patients with CVI may range from virtually asymptomatic to severe disease with the presence of venous ulceration. The clinical staging is best identified using the CEAP classification (Table 33–1). Symptoms associated with CVI include pain, itching, burning, aching, and heaviness or fatigue of the legs. Symptoms may improve dramatically with leg elevation, essentially relieving the venous hypertension.

The most prominent clinical finding in CVI is edema. Early in the disease, the edema is usually soft and pitting; however, as time progresses, many patients will develop thickening and fibrosis of the subcutaneous tissue termed lipodermatosclerosis. Unlike lymphedema, the edema of CVI usually involves the ankle and lower calf, but spares the dorsum of the foot. The edema is usually responsive to elevation. Patients will frequently report minimal swelling upon awakening but increasing edema as the day progresses.

Skin changes are also common. Patients may have dry, hyperkeratotic skin, inflammation or stasis dermatitis, hyperpigmentation or hemosiderin staining, atrophie blanche or white atrophic scarring of the subcutaneous tissue, or even venous ulceration. Venous ulceration may be differentiated from arterial ulceration by the characteristics of the ulcer (Table 33–2). Although mixed venous and arterial disease is common especially in older adults. Varicose veins are another prominent feature of CVI. Varicosities are typical of superficial vein involvement and may range from small venous telangiectasias or spider veins, to subdermal reticular veins that are 1–3 mm in size, to ropey, bulging varicosities.

Diagnostic Testing

History and physical examination are usually sufficient to make the diagnosis of CVI. However, both bedside and vascular laboratory testing may be used to confirm the diagnosis and to better localize the abnormality. The most basic office assessment includes having the patient stand and examining the patient for bulging varicosities. Holding your hand over the groin at the saphenofemoral junction while the patient performs a Valsalva maneuver will confirm reflux if the veins pressurize. Photoplethysmography and air plethysmography are simple noninvasive tests that can evaluate for reflux, obstruction, and the calf muscle pump. However, testing is not widely performed.

Duplex ultrasonography for venous insufficiency is considered the “gold standard” for diagnosis venous insufficiency. Performed by the vascular laboratory, the testing is usually done standing or in deep Trendelenburg to augment valvular incompetency and reflux. Both Valsalva maneuvers and distal compression may be used to elicit reflux during imaging. The vascular laboratory should also be used to exclude peripheral arterial disease (PAD) in patients with absent or diminished pulses prior to commencing compression therapy. Patients with an ankle-brachial index (ABI) <0.7 require care and caution when using compression for managing edema or venous ulcer healing.

Varicosities that extend from the buttocks or are over the perineum or anterior abdominal wall may need further evaluation with magnetic resonance venography (MRV) to exclude pelvic reflux through the ovarian veins. Abdominal and pelvic CT imaging to exclude intrinsic or extrinsic injury to the inferior vena cava (IVC) from tumor or fibrosis should be considered with bilateral symmetrical swelling, especially if it is of recent onset or rapidly progressive.

Most edema in older adults has multifactorial contributions from systemic illness, CVI or loss of the calf muscle pump, and medications. A thorough history and physical examination are required to exclude other secondary causes of edema apart from CVI or lymphedema. Systemic conditions related to heart failure, increased right-heart pressures from pulmonary hypertension or valvular heart disease, protein loss related to renal or enteric disease, decreased protein from cirrhosis, other liver disease or malnutrition, and endocrine disorders, such as Cushing disease, may cause swelling. Myxedema related to thyroid disease may also be confused with edema and should be excluded by biopsy.

Medications are a frequent cause for lower-extremity edema. Hormone therapy, steroids, dihydropyridine calcium channel blockers, thiazolidines, and nonsteroidal antiinflammatory agents are all associated with edema. In addition, gabapentin and pramipexole are common offenders.

Pain, swelling, impaired mobility, and skin changes are the typical complications experienced with CVI. The most problematic complication is that of venous stasis ulceration. Conservative estimates suggest that >20,000 patients are diagnosed with venous stasis ulcers annually. Ulcer care requires frequent office visits. Patients may experience pain associated with debridement. Some patients may feel isolated because of the appearance of the dressings or odor associated with active wounds. Bleeding from superficial varicosities, while dramatic, usually responds well to light compression and limb elevation. Secondary sclerotherapy may prevent recurrent bleeding.

General Considerations

The goals for treating CVI are to reduce edema, alleviate pain, and improve the overall condition of the skin. In patients with venous stasis ulcers, this translates further into wound healing and preventing recurrence. Conservative care for the skin is required. Water-based emollients will improve the skin texture and prevent dryness and cracking that may promote ulceration. Patients who develop venous eczema or stasis dermatitis may benefit from a low- or medium-potency topical corticosteroid for a short duration. If there is maceration and breakdown in the webspaces, an antifungal powder used twice daily should be recommended. Tinea pedis is a common source of cellulitis. For patients who develop wounds, standard wound care practices to keep the exudates well managed and the base of the wound moist should be used.

In addition to overall skin care, elevation and compression therapy are the mainstay of treatment for CVI. Patients should be questioned regarding sleeping habits as previously noted. Patients who are sleeping in a chair or recliner should be strongly advised to return to the bed. The opportunity to use elevation to provide passive decongestion of the legs cannot be underestimated. Elevation decreases the venous hypertension and reduces swelling and pain. Patients should be advised to elevate their legs several times a day. Elevation needs to be done with the legs above the level of the right atrium. In addition, patients should be encouraged to elevate the foot of their bed using a 3–4-inch brick under the bed posts. This will provide approximately 10 degrees of elevation of the foot of the bed and passive decongestion of their legs while they are sleeping. Elevating the ankles and legs with pillow is not as efficient as patients are required to maintain a still sleeping position on their back lest the pillows are kicked off the bed. Elevating the foot of the bed allows the patient to sleep comfortably in any position and maintain elevation. Unless the patient has significant esophageal reflux, most patients and spouses tolerate this change in sleeping position without too much difficulty.

Compression

Depending on the etiology of the CVI, the commitment to compression is generally a lifelong endeavor. Compression decreases venous capacitance, decreases capillary venous exudation, and improves the calf muscle pump function with respect to the ejection fraction and ejection volume with ambulation. The type of compression garment and the amount of compression or strength of the garment needs to be individually tailored to the patient. It is imperative that the compression is measured to fit appropriately. Most patients are sufficiently managed with a knee-high garment.

In general, patients with C1 to C2 or early C3 disease may be sufficiently managed with 15–20 mm Hg compression. Patients with C3 and C4 disease typically are best managed with 20–30 mm Hg compression. Patients with more severe disease including venous ulcers or healed ulceration, C5 and C6 disease, are best managed with 30–40 mm Hg compression. In reality, most older patients cannot apply stockings in excess of 20 mm Hg. Caregivers or family members may be required to assist with the stocking application. In addition, patients with moderate or severe PAD should also not be in higher grades of compression. The compression must be matched to the severity of the PAD.

Patients who are limited by osteoarthritis, limited mobility, prior hip replacement, or obesity frequently cannot reach their foot to apply the garments. Stocking donning aides may be helpful. In addition, using rubber gloves and a cotton-based stocking may be helpful for patients with arthritis of their hands. Patients should be advised to lose weight, if needed. Central obesity increases pressure in the venous system and further limits compliance with stockings. Exercise is helpful to increase venous return. If possible patients should walk on a regular basis to improve venous circulation. Pool exercise or walking may be helpful for patients with arthritis who find weight-bearing exercise uncomfortable. Regular foot and ankle exercises that augment the calf muscle pump action can be used to improve venous return.

Intervention

Patients who have persistent symptoms despite adequate conservative treatment or patients with recurrent superficial thrombophlebitis or venous stasis ulcers should be considered for intervention. For larger axial varicose veins venous stripping was historically used for most patients. Although still used, traditional venous stripping has been largely replaced by endovenous laser ablation therapy (EVLT) or radiofrequency ablation (RFA). These are catheter-based techniques that obliterate the vein from the lumen of the vessel using heat injury and thrombosis (endothermal ablation). Typically these procedures can be performed in an ambulatory setting and the patients experience little pain or bruising. Many patients return to normal activities the day after the procedure. Another technique, called foam sclerotherapy, is being used with increasing frequency, even for larger varicose veins. This is also performed in an office setting. Sclerosant foam is injected into the vein, typically using ultrasound guidance to injure the venous wall and facilitate scarring and closure of the vessel. Which procedure is best is determined by clinical factors and operator and patient preference. The surgeon or operator should choose the procedure likely to offer the best opportunity for successful venous closure. It is important to recognize that venous closure is not associated with improved venous ulcer healing but does decrease the rate of recurrence and, therefore, may be warranted after wound healing.

Reticular veins and venous telangiectasias may be managed by injection sclerotherapy. In many cases, this is considered cosmetic; however, when symptomatic, additional therapy may be considered. In similar fashion, symptomatic venous clusters and varicosities not related to the axial veins may benefit. Foam sclerotherapy or ambulatory phlebectomy can be used successfully for many of these cases. Again, the choice of therapy is operator dependent.

General Principles in Older Adults

The lymphatic system is responsible for removing excess tissue fluid as well as proteinaceous debris and cellular matter from the tissues. Lymphedema is the pathologic accumulation of this protein rich fluid in the subcutaneous tissues. Lymphedema occurs when the lymphatics are either reduced in number, injured or destroyed, or malformed.

Lymphedema may be considered primary or secondary. Primary lymphedema is a result of the inherent absence or dysfunction of the lymphatics without a history of insult or injury. Primary congenital lymphedema is present at birth or within the first year of life. Lymphedema praecox begins in adolescence through the third decade of life. Lymphedema tarda has onset after age 40 years. These may be familial or sporadic in nature. Secondary lymphedema occurs as a result of injury or trauma that disrupts or obstructs lymphatic flow. The most common causes of secondary lymphedema are malignancy as a result of tumor infiltration, obstruction, or related to the therapies, including radiation or surgery; surgery, including lymph node dissection, vein harvest, or hernia repair; and repeated infection, including lymphangiitis or cellulitis.

A more recently recognized phenomenon of phlebolymphedema is the result of the interaction between the venous system and the lymphatics. Phlebolymphedema has characteristics of both venous insufficiency and lymphedema. This occurs when chronic venous hypertension results in excess fluid filtration such that the lymphatic transport capacity is exceeded. Once the venous hypertension is relieved the lymphedema usually spontaneously regresses.

Clinical Findings

Signs & Symptoms

It is important to distinguish among edema, venous insufficiency, and lymphedema. Each of these clinical conditions are distinct entities and managed using different modalities. Several clinical findings may be helpful to distinguish lymphedema from edema or venous insufficiency. Unlike venous insufficiency, which usually involves the ankles and distal calf, lymphedema begins distally, and the toes and foot are virtually always involved at the outset. Lymphedema is classically identified by the association of squared toes, a prominent dorsal foot hump, and a positive Stemmer sign. The Stemmer sign is the inability to pinch the skin at the base of the toes. It is not pathognomonic for lymphedema, but it is a common clinical finding. Ulceration is not common, but may be fairly difficult to manage given the fibrotic skin and excess exudates associated with skin injury.

Lymphedema is a chronic and progressive condition. It is graded in stages:

Stage 0: There are no observable clinical findings but the patient may be at risk as a result of lymphatic injury or insufficiency. This is latent or subclinical disease. This stage is usually unrecognized.

Stage 1: There is usually mild or intermittent swelling. The edema may still be pitting. Elevation may decrease the swelling. Skin findings such as thickening or fibrosis and pigmentation are not identified.

Stage 2: The swelling is usually persistent. Elevation typically has little effect on the swelling. The skin may be more fibrotic and hyperpigmentation may be noted.

Stage 3: This stage is consistent with elephantiasis or late-stage lymphedema. There is marked fibrosis of the skin. Hyperpigmentation may be pronounced. Secondary skin changes of hyperkeratosis and papillomatosis are common.

Within each stage of lymphedema, the degree of swelling can further be classified as mild (<20% increase in limb girth), moderate (20% to 40% increase in limb girth), or severe (>40% increase in limb girth).

Laboratory Findings

There are currently no laboratory markers to identify patients with lymphedema. When appropriate, laboratory evaluation to differentiate edema from lymphedema may be appropriate. This may include complete metabolic panel (CMP) to evaluate serum protein and albumin concentration. Urinalysis to exclude significant urinary protein losses may also be helpful.

Diagnostic Testing

Lymphedema is frequently diagnosed clinically based on the history and physical examination. Diagnostic imaging is fairly limited. Traditional contrast lymphangiography is rarely necessary or performed. In some centers, nuclear lymphoscintigraphy, sometimes also referred to as nuclear lymphangiography, is available and may be helpful. Lymphoscintigraphy uses a radiolabeled colloid to observe lymphatic uptake and transport from the point if a distal injection.

CT and MRI imaging will usually demonstrate soft-tissue swelling. Venous duplex ultrasound for deep vein thrombosis and to exclude significant deep or superficial venous reflux is included in the diagnostic algorithm. In patients with unilateral limb swelling, CT imaging of the chest, abdomen, or pelvis should be considered to exclude the presence of occult malignancy or obstruction of the lymphatics.

Differential Diagnosis

Edema is the accumulation of interstitial fluid. This may be from systemic causes such as cardiac or renal disease, medications, or protein maldistribution or losses. Venous insufficiency occurs when there is excess tissue fluid accumulation as a result of increased venous filtration or decreased venous reabsorption. In both cases, the interstitial fluid is mostly water with a lesser protein content. Lymphedema is a protein-rich fluid as a result of an inability of the lymphatic to reabsorb filtered fluid as well as protein. The differential diagnosis of extremity swelling is beyond the scope of this chapter. The differential diagnosis, clinical evaluation, and imaging should be individualized. In general, if the edema affects both of the upper or lower extremities, the differential should focus on systemic etiologies or a centrally obstructing process. If a single limb is involved, the pathology will usually be confined to a single body quadrant.

Lipedema is frequently confused with lymphedema. Lipedema is the accumulation of excess adipose subcutaneously. Patients may have fairly severe involvement of the extremities. One key differentiating feature is the foot-sparing associated with lipedema. Limb or muscular hypertrophy may also be confused for lymphedema. In these patients, MRI may be very helpful. The identification of hypertrophic musculature as opposed to the subcutaneous honeycombing of lymphedema can be readily identified. Klippel-Trenaunay and Parks-Weber syndromes are 2 other disorders associated with limb hypertrophy, usually in the absence of lymphedema.

Complications

Lymphedema is a chronic and disabling condition for many patients. It requires ongoing and aggressive care to prevent worsening of the disease process. Recurrent cellulites, lymphangitis, and wounds may also complicate the condition. Rarely, patients develop a lymphedema-associated arthropathy that can be painful and disabling. Even less common is the development of a lymphedema-associated angiosarcoma in the affected limb. However, patients who develop blue or purple skin lesions should be referred for biopsy and further evaluation.

Treatment

General Considerations

Prevention and education are key components to managing lymphedema. Education regarding the pathology and dysfunction of the lymphatics and their role in infection and injury modification is required. Patients need to be engaged in the long-term care because lymphedema will typically not improve, but certainly may worsen if care is not maintained.

Patients need to be advised of meticulous skin care to prevent minor injuries, ulceration, or trauma. Patients should moisturize their skin regularly. Keratolytic emollients or lactic-acid-based products may be recommended when there are extensive hyperkeratotic or papillomatous changes. Patient should see a podiatrist for nail care. Avoid trimming the cuticles or hangnails. Gloves are recommended for gardening, housework, and dishwashing. Early treatment of minor trauma or insect bites is recommended. Patients should be advised to use sunscreen and avoid sunburn or prolonged exposure. Avoid iatrogenic trauma, such as venipuncture, injections, and blood pressure measurement, in the affected extremity. The use of razors, hair waxing, or chemical hair removers is ill advised. Moderate exercise should be encouraged. Traditionally, patients have been advised to avoid vigorous exercise, but there are little data to support this recommendation. In contrast to venous insufficiency, elevation for passive decongestion of the limb has little effect in lymphedema.

Limb Decongestion

Treatment in lymphedema focuses on reducing and maintaining decongestion of the limb. Manual lymphatic drainage (MLD) is a physical therapy- or occupational therapy-directed program of limb and trunk massage and multilayered wrapping designed to decongest the lymphedematous limb. It is part of a program of complex decongestive therapy (CDT), which includes MLD, along with skin care, education, and exercises designed to decongest and maintain the condition of the limb. The MLD/CDT requires specialized training. The therapist should be specifically trained and certified in MLD.

In conjunction with MLD the use of pneumatic pumps may be helpful. The pumps may also be incorporated into a maintenance program and used at home on a regular basis. Newer pump designs are available that simulate MLD providing decongestion of the trunk as well as the extremity.

Once the limb is decongested, maintaining the gains of therapy is required. Most patients will use compression garments during the day. The daytime garment should provide continuous and graded compression. Compression of 30–40 mm Hg is usually recommended, but older patients may find this degree of compression difficult or impossible to apply. The use of the highest grade of compression that the patient can apply is likely to be the most helpful. Caregivers, family members, and other resources may also be required to assist with donning and doffing the garment. In addition, the compression much be matched to any degree of arterial insufficiency. A nighttime program with continued wrapping or the use of other devices may be recommended.

Pharmacotherapy

In general diuretics are of little benefit in lymphedema and are best avoided unless there are other indications for systemic diuretic therapy. Benzopyrenes including coumarin, rutoside, and bioflavonoids, are touted as beneficial for treating lymphedema. They are not available in the United States, and their exact roles in lymphedema are not well defined.

Antibiotics should be administered promptly for infections such as cellulitis or erysipelas in the setting of lymphedema. Injury related to the infection and inflammation can further damage the fragile lymphatics. If patients have more than 1 episode annually, consideration of preventive therapy may be appropriate. Many patients with lymphedema also have associated tinea pedis and use of antifungal powder to the webspaces is prudent. Secondary cutaneous fungal infections are also encountered, especially in stage 3 lymphedema. Aggressive and prolonged treatment may be required.

If patients develop wounds or persistent lymph drainage from skin fissure, they may have significant protein loss. Evaluation of their protein, albumin, and prealbumin may be warranted. Nutritional supplementation should be provided to optimize wound healing.

Surgical Therapy

Surgical advances in lymphedema have been disappointing. Microsurgical lymphatic bypass or anastomosis procedures typically do not have adequate durability in this progressive condition. In addition, few surgeons have the expertise or experience in these procedures. Debulking surgeries are rarely performed, except in cases of massive lymphedema. Again, few surgeons have experience with these techniques. Limb volume-reduction surgeries using liposuction are becoming more frequent. Patients with less fibrotic lymphedema may be surgical candidates. After the procedure, patients must be diligent with decongestive therapy, including MLD and compression.