Description, Causes and Risk Factors:
Peripheral lymphedema often poses a dilemma for the clinician because it is a nonspecific finding common to a host of diseases ranging from the benign to the potentially life threatening. A rational and systematic approach to the patient with peripheral lymphedema allows for prompt and cost-effective diagnosis and treatment.
Lymphedema is an external (or internal) manifestation of lymphatic system insufficiency and deranged lymph transport. It may be an isolated phenomenon or associated with a multitude of other disabling local sequelae or even life-threatening systemic syndromes. In its purest form, the central disturbance is a low output failure of the lymphovascular system, that is, overall lymphatic transport is reduced. This derangement arises either from congenital lymphatic dysplasia (primary lymphedema) or anatomical obliteration, such as after radical operative dissection (e g., axillary or retroperitoneal nodal sampling), irradiation, or from repeated lymphangitis with lymphangiosclerosis (secondary lymphedema) or as a consequence of functional deficiency (e.g., lymphangiospasm, paralysis, and valvular insufficiency) (primary or secondary lymphedema). The common denominator, nonetheless, is that lymphatic transport has fallen below the capacity needed to handle the presented load of microvascular filtrate including plasma protein and cells that normally leak from the bloodstream into the interstitium. Swelling is produced by accumulation in the extracellular space of excess water, filtered plasma proteins, extravascular blood cells and parenchymal cell products. This process culminates in proliferation of parenchymal and stromal elements with excessive deposition of ground matrix substances. High output failure of the lymph circulation, on the other hand, occurs when a normal or increased transport capacity of intact lymphatics is overwhelmed by an excessive burden of blood capillary filtrate. Examples include hepatic cirrhosis (ascites), nephrotic syndrome (anasarca), and deep venous insufficiency of the leg (peripheral edema). Although the final pathway is the manifestation of tissue edema whenever lymph formation exceeds lymph absorption, the latter entities should properly be distinguished from lymphedema, which is characterized by decreased lymphatic transport. In some syndromes where high output lymphatic transport failure is longstanding, a gradual functional deterioration of the draining lymphatics may supervene and thereby reduce overall transport capacity. A reduced lymphatic circulatory capacity then develops in the face of increased blood capillary filtration. Examples include recurring infection, thermal burns, and repeated allergic reactions. These latter conditions are associated with “safety valve insufficiency” of the lymphatic system and can be considered a mixed form of edema/lymphedema and as such are particularly troublesome to treat.
Peripheral lymphedema associated with chylous (a milky fluid consisting of lymph and emulsified fats; formed in the small intestine during digestion of ingested fats) and non-chylous reflux syndromes is an infrequent but complex condition that requires specific diagnostic measures and treatment methods.
Generally rely on a three stage scale for classification of a lymphedematous limb, an increasing number recognize Stage 0 which refers to a latent or sub-clinical condition where swelling is not evident despite impaired lymph transport. It may exist months or years before overt edema occurs (Stages I-III). Stage I represents an early accumulation of fluid relatively high in protein content (e.g., in comparison with “venous” edema) and subsides with limb elevation. Pitting may occur. Stage II signifies that limb elevation alone rarely reduces tissue swelling and pitting is manifest. Late in Stage II, the limb may or may not pit as tissue fibrosis supervenes. Stage III encompasses lymphostatic elephantiasis where pitting is absent and trophic skin changes such as acanthosis, fat deposits, and warty overgrow this develop. Within each Stage, severity based on volume difference can be assessed as minimal (<20% increase) in limb volume, moderate (20-40% increase), or severe (>40% increase).
These Stages only refer to the physical condition of the extremities. A more detailed and inclusive classification needs to be formulated in accordance with improved understanding of the pathogenetic mechanisms of lymphedema (e.g., nature and degree of lymphangiodysplasia, lymph flow perturbations and nodal dysfunction as defined by anatomic features and physiologic imaging and testing) and underlying genetic disturbances, which are gradually being elucidated.
Primary lymphedema is an inherited condition where the patient is born without lymph vessels and nodes. The swelling associated with primary lymphedema usually occurs during adolescence and affects the foot or calf. A rare form of primary lymphedema, called Milroy's disease, occurs in pregnancy. However, secondary lymphedema, or acquired lymphedema, develops as a result of an injury to the lymph system. Specific causes include surgical treatments for certain types of cancers, especially those cancers that currently require the removal of lymph nodes. Radiation treatment for cancer or for some AIDS-related diseases such as Kaposi's sarcoma may also result in lymphedema, as radiation may damage or destroy lymph nodes or cause the formation of scar tissue that can interrupt the normal flow of the lymphatic fluid. Specific cancers and their treatment that may result in lymphedema include malignant melanoma, breast (in both women and men), gynecological, head and neck, prostate, testicular, bladder, and colon cancer. Other causes of lymphedema include trauma to the lymphatic system from burns, liposuction, tattooing, injuries, surgery, radiation, obesity, heart or circulatory disease, and multiple sclerosis. People at risk may not develop the condition immediately, but develop the condition weeks, months, or even years later. Aircraft travel has been linked to the development of lymphedema in patients after cancer surgery, possible due to the decreased cabin pressure.
In Western countries, one of the most common causes of lymphedema is mastectomy with axillary dissection (removal of the breast and underarm lymph tissue for treatment of breast cancer), which may result in lymphedema of the breast, underarm, or arm on the side of the surgery in 10-20% of patients. This occurs because the lymphatic drainage of the arm passes through the axilla (armpit), and tissue in the axilla is removed during the mastectomy. To reduce the risk of developing lymphedema after breast cancer treatment, there is an alternative treatment that avoids axillary lymph node dissection. Sentinel lymph node biopsy is a new diagnostic procedure used to determine whether the breast cancer has spread (metastasized) to axillary lymph nodes. A sentinel lymph node biopsy requires the removal of only one to three lymph nodes for close review by a pathologist. If the sentinel nodes do not contain tumor (cancer) cells, this may eliminate the need to remove additional lymph nodes in the axillary area. Early research on this technique indicates that sentinel lymph node biopsy may be associated with less pain and fewer complications than standard axillary dissection. Because the procedure is so new, long-term data are not yet available. However, there is still a risk of developing lymphedema because of follow-up radiation treatments or chemotherapy, which may also damage the lymph nodes.
People who have developed lymphedema after cancer treatment should be checked for a possible recurrence of cancer if they experience a sudden increase in swelling; the tumor growth may be responsible for blocking lymphatic flow.
Risk factors for lymphedema include the following:
Surgical removal of lymph nodes in the underarm, groin, or pelvic regions.
Radiation therapy to the underarm, groin, pelvic, or neck regions.
Scar tissue in the lymphatic ducts or veins and under the collarbones, caused by surgery or radiation therapy.
Cancer that has spread to the lymph nodes in the neck, chest, underarm, pelvis, or abdomen.
Tumors growing in the pelvis or abdomen that involve or put pressure on the lymphatic vessels and/or the large lymphatic duct in the chest and block lymph drainage.
Having an inadequate diet or being overweight. These conditions may delay recovery and increase the risk for lymphedema.
Breast cancer, if the patient received radiation therapy or had lymph nodes removed. Radiation therapy to the underarm area after surgical removal of the lymph nodes and having a larger number of lymph nodes removed increases the risk of lymphedema.
Peripheral lymphedema causes swelling with a feeling of heaviness, tightness or fullness, usually in an arm or leg. In most cases, only one arm or leg is affected. Swelling in the leg usually begins at the foot, and then moves up if it worsens to include the ankle, calf and knee. Additional symptoms can include:
A feeling of tightness in the skin of the affected limb.
Difficulty moving a limb or bending at a joint because of swelling and skin tightness.
Shoes, rings or watches that suddenly feel too tight.
A dull ache in the affected limb.
Peripheral lymphedema can make it easier to develop a skin infection. Signs of infection include fever, pain, heat and redness. If peripheral lymphedema becomes chronic (long lasting), the skin in the affected area often becomes thickened and hard.
An accurate diagnosis of lymphedemais essential for appropriate therapy. In mostpatients, the diagnosis of lymphedema canbe readily determined from the clinicalhistory and physical examination. In otherpatients confounding conditions such asmorbid obesity, venous insufficiency, occulttrauma, and repeated infection maycomplicate the clinical picture. Moreover, inconsidering the basis of unilateral extremitylymphedema, especially in adults, an occultvisceral tumor obstructing or invading moreproximal lymphatics needs to be considered.For these reasons, a thorough medicalevaluation is indispensable before embarkingon lymphedema treatment. Co-morbidconditions such as congestive heart failure,hypertension, and cerebrovascular diseaseincluding stroke may also influence thetherapeutic approach undertaken.
If the diagnosis of lymphedema isunclear or in need of better definition forprognostic considerations, consultation with aclinical lymphologist or referral to alymphologic center if accessible isrecommended. The diagnostic tool of isotopelymphography (also termedlymphoscintigraphy orlymphangioscintigraphy) has provedextremely useful for depicting the specificlymphatic abnormality. Where specialists innuclear medicine are available,lymphangioscintigraphy (LAS) has largelyreplaced conventional oil contrastlymphography for visualizing the lymphaticnetwork. Although LAS has not beenstandardized (various radiotracers andradioactivity doses, different injectionvolumes, intracutaneous versus subcutaneousinjection site, epi-or subfascial injection, oneor more injections, different protocols ofpassive and active physical activity, varyingimaging times, static and/or dynamictechniques), the images, which can be easilyrepeated, offer remarkable insight intolymphatic dysfunction.
LAS provides both images of lymphaticsand lymph nodes as w ell as semi-quantitativedata on radiotracer (lymph) transport, and itdoes not require dermal injections of blue-dye(as used for example in axillary or groinsentinel node visualization i.e.,lymphangioscintigraphy). Dye injection isoccasionally complicated by an allergic skinreaction or serious anaphylaxis. Moreover,clinical interpretation of lymphatic functionafter vital dye injection alone (“the blue test”)is often misleading. Direct oil contrastlymphography, which is cumbersome andoccasionally associated with minor and majorcomplications, is usually reserved for complexconditions such as chylous reflux syndromeand thoracic duct injury. Non-invasiveduplex-Doppler studies and occasionallyphlebography are useful for examining thedeep venous system and supplement orcomplement the evaluation of extremityedema. Other diagnostic and investigationaltools used to elucidatelymphangiodysplasia/lymphedemasyndromes include magnetic resonanceimaging (MRI), computed tomography (CT),ultrasonography (US), indirect (watersoluble) lymphography (IL) and fluorescentmicrolymphangiography (FM). DEXA, or biphotonic absorptiometry, may help classifyand diagnose a lymphedematous limb but itsgreatest potential use may be to assess thechemical components of limb swelling (%fat, water, lean mass) before and aftertreatment. IL and FM are best suited todepict initial and terminal lymphatics andaccordingly have limited clinical usefulness.US has found its most practical value indepicting the dance of the living adult wormsin scrotal lymphatic filariasis.
Genetic Testing: Genetic testing is almost becomingpractical to define a limited number ofspecific hereditary syndromes with discretegene mutations such as lymphedema-distichiasis (FOXC2) and some forms ofMilroy disease (VEGFR-3). The futureholds promise that such testing combined with careful phenotypic descriptions willbecome routine to classify familiallymphangiodysplastic syndromes and othercongenital/genetic-dysmorphogenic disorderscharacterized by lymphedema,lymphangiectasia, and lymphangiomatosis.
Biopsy: Caution should be exercised beforeremoving enlarged regional lymph nodes inthe setting of longstanding peripherallymphedema as the histologic information isseldom helpful, and such excision mayaggravate distal swelling. Fine needleaspiration with cytological examination by askilled pathologist is a useful alternative ifmalignancy is suspected. Use of sentinelnode biopsy in the groin or axilla in stagingmalignancy such as breast and melanoma, ifvalidated for determining metastasis, maylessen the incidence of peripherallymphedema by discouraging removal ofnormal lymph nodes.
In the treatment of "classical" lymphedema of the limbs (that is, peripheral lymphedema), improvement in swelling can usually be achieved by non-operative therapy. Because lymphedema is a chronic, generally incurable ailment, it requires, as do other chronic disorders, lifelong care and attention along with psychosocial support. The continued need for therapy does not mean a priori that treatment is unsatisfactory, although often it is less than ideal. For example, patients with diabetes mellitus continue to need drugs (insulin) or special diet (low calorie, low sugar) in order to maintain metabolic homeostasis. Similarly, patients with chronic venous insufficiency require lifelong external compression therapy to minimize edema, lipodermatosclerosis and skin ulceration. The compliance and commitment of the patient is also essential to an improved outcome. For example, in a patient with diabetes, poor compliance can result in w eight loss, polyuria, and even coma and, long-term, also blindness, renal failure, and stroke. With chronic venous insufficiency, poor patient cooperation may be associated with progressive skin ulceration, hyperpigmentation, and other trophic changes in the lower leg. Similarly, failure to control lymphedema may lead to repeated infections (cellulitis/lymphangitis), progressive elephantine trophic changes in the skin, sometimes crippling invalidism and on rare occasions, the development of a highly lethal angiosarcoma (Stewart-Treves syndrome).
Operations designed to alleviate peripheral lymphedema by enhancing lymph return have not as yet been accepted worldwide and often require combined physiotherapy after the procedure to maintain edema reduction. In selected patients, these procedures may act as an adjunct to CPT (combine physical therapy) or be undertaken when CPT has clearly been unsuccessful. In some specialized centers, operative treatment within specific guidelines may be a preferred approach.
Other treatment options may include: Combined physical therapy (CPT) and complex decongestive physiotherapy (CDP), massage alone, wringing out, thermal therapy, mesotherapy, immunological therapy, and psychological rehabilitation.
NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.
DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.
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