Essentials of Diagnosis
Unilateral limb involvement (infrequently involves both limbs).
Nonpitting edema involving the dorsum of the foot with squaring of the toes.
History of cellulitis, malignancy, surgery, or trauma.
Skin changes of CVI are usually absent.
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.
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).
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.
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.
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.
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.
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.
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.
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 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.
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