Complications Associated with Lymphedema
Lymphedema is a chronic disease that requires lifelong management. Early recognition and diagnosis improve patient outcomes and reduce the likelihood of associated complications. This article will highlight the more common complications associated with lymphedema to enable the health-care provider to more readily identify these issues early.
Most complications associated with lymphedema are due to the excess fluid burden placed upon the tissues. This can lead to a variety of impairments and complications, many of which are interrelated. Other complications may arise from medical interventions that may lead to a secondary lymphedema as well as other medical challenges for the patient. Complications can include but are not limited to the following:
disturbance of local cellular metabolism
increased rates of infection due to fluid stagnation and associated integumentary changes
integumentary dysfunction (i.e., fibrosis, papillomatosis, denudement/ulceration, alterations in skin barrier function/acidmantle)
progressive lymphatic damage and associated impairments in the arterial and venous vascular systems
lymphatic arthropathy and related orthopedic impairments
It is also important to note that the underlying cause of lymphedema and the associated body areas affected will play a part in the potential complications that may arise. It is incumbent upon the health-care provider to thoroughly evaluate the patient, review all the body systems, and ask specific questions to elicit a complete clinical picture.
It is well recognized that many secondary lymphedemas are due to various forms of cancer. Most forms of cancer are treated with surgery, radiation and/or chemotherapy, or any combination thereof. Certain complications may arise secondary to lymphedema and the related cancer interventions. For example, breast-cancer-related lymphedema is often associated with axillary web syndrome (AWS), radiation injury/dermatitis and fibrosis, and radiation-induced brachial plexopathy.
AWS (also known as Mondor’s disease, lymphatic cording, subcutaneous fibrous banding, fiddle-string phenomenon, lymph vessel fibrosis, lymphangiofibrosis thrombotica occlusive, and lymph thrombosis) is due to disruption of axillary lymphatics usually related to node dissection, sentinel lymph node dissection, trauma, or obstruction from cancer. The injury interrupts the lympho-venous channels resulting in thrombosis and stagnation of fluid, which in turn results in inflammation, fibrosis, and a shortening of surrounding tissue. AWS is a visible and palpable web of tissue that becomes taut with shoulder abduction. It is typically located in axilla and can extend distally along the anterior, medial upper arm toward antecubital space; it may also extend to the thumb. It is best managed with specific manual techniques and at times is self-limiting.
Radiation injury results from morphological and functional changes that occur in noncancerous tissue due to ionizing radiation. These changes can be mild to life-threatening. Irradiation of the skin causes dose-dependent damage to the skin and can result in acute or chronic radiation dermatitis. Acute radiation dermatitis is an inflammatory reaction due to direct injury to the cell, whereas chronic radiation damage occurs as a summation effect after multiple exposures to low-level radiation. It is important to note that roughly 85% of patients treated with radiation therapy will experience a moderate to severe skin reaction.1
PHOTO 1. Papillomatosis and fibrosclerotic skin changes
Typically, within the first week of radiation there may be visible or faint erythema due to capillary dilatation and an increase in vascular permeability. During the second to third week, radiation inhibits mitotic activity in germinal cells of the epidermis, causing hair follicle and sebaceous gland epilation and xerosis. Cell production decreases, and dry desquamation or scaling begins. Endothelial swelling and proliferation causes obstruction, which is due to fibrin thrombi in the arterioles. During the third to fourth week, moist desquamation may result from skin peeling, vascular dilatation, edema, and oozing of serum from denuded areas; the skin is red, warm, tender, and edematous with telangiectasias. Chronic radiation dermatitis develops after a more severe acute radiation reaction or as a result of high amounts of total radiation. Latency, however, can be 2 to 10 years following radiation therapy.² In either situation, patients with concomitant lymphedema should have modifications made during complete decongestive therapy (CDT), particularly with manual lymph drainage and compression, so further disruption to the radiated tissue is mitigated.
Radiation fibrosis is another potential complication with a unique presentation that may present in patients with lymphedema. It is a reaction of the skin to irradiation with visible and palpable changes. The skin is bound down with reddish-brown discoloration and telangiectasia. Radiation causes slow retreat of microcirculation in soft tissues, leading to ischemia, scar-tissue formation, and fragile skin. Health-care providers must consider the secondary effects of radiation fibrosis and the potential impact these have on patient function and quality of life. Patients with radiation fibrosis also require modification with CDT to prevent skin integrity issues at the already compromised areas. Deeper manual lymph drainage and fibrosis techniques are not recommended on areas with radiation fibrosis due to the tissues fragility.
Another complication related to radiation therapy is radiation-induced brachial plexopathy (RIBP). This is caused by radiation damage to the brachial plexus. Symptoms may include paresthesia (tingling, pricking, numbness), dysesthesia (abnormal sense of touch, such as burning, itching, feeling of an electric current, “pins and needles,” pain), decreased sensitivity, partial loss of movement (muscle weakness and difficulty performing simple tasks such as opening jars or containers, holding objects), complete paralysis of the arm, muscular atrophy, impaired mobility, and even partial dislocation of the shoulder joint. The exact mechanism of RIBP is not completely understood. Damage to the brachial plexus results from a combination of direct nerve cell damage from ionizing radiation and progressive damage from scar tissue (radiation fibrosis) in and around the nerves, combined with damage to adjacent vessels that supply these nerves with oxygen and nutrients. Most patients develop symptoms within the first three years; however, the average interval between the last dose of radiation and the onset of RIBP symptoms reported in the literature varies widely with a range between 6 months and 20 years. The prevalence of RIBP is reported to be between 1.8% and 4.9%, and RIBP is more common after radiation in combination with chemotherapy.³
Other specific anatomical sites subject to lymphedema include the genitals as well as the head and neck. Genital lymphedema in men can lead to complications including loss of sexual function, infertility (depending upon the severity), difficulties in urination, severe social stigma, skin rashes, and fungal infections. Head and neck lymphedema can lead to significant impairments in eating and mastication, swallowing, speech, and breathing. Both require specific management techniques and interventions. These conditions, however, are readily manageable with good functional outcomes and usually a return to a near-normal morphology.
The most common complications seen in patients with lymphedema are secondary infections of the skin and underlying tissues. Cellulitis is an inflammation of the skin and connective tissues, whereas lymphangitis is an inflammation of the lymph vessels often seen as red streaking up the extremity. Moisture lesions and fungal infections, particularly of the lower extremities and toenails, are also common in this patient population. These secondary infections and complications develop due to the protein-rich fluid that can stagnate in the interstitial tissues. Stagnating high-protein edema in the interstitial tissues leads to a pathohistological state of chronic inflammation.4 This creates a migration and infiltration of macrophages leading to excess collagen deposition. Fibroblasts migrate into the connective tissues, which become fibrocytes (creating collagen tissue) and adipocytes (creating fatty tissue). Ultimately, fibrosis and fibrosclerosis result in the typical skin changes often associated with Stage III lymphostatic elephantiasis.
Photo 1 depicts a classic representation of the associated skin changes seen in patients with Stage III lymphostatic elephantiasis. Table 1 highlights the common skin lesions and conditions associated with lymphedema.
Deep vein thrombosis (DVT) is another potential complication more readily seen in patients with mobility impairments as they do not have an adequate muscle pump to support proper hemodynamics. The impaired lymphatic system along with the associated fluid burden and fibrotic changes lead to fatigue and impairments in the venous system, making the patient more susceptible to the development of DVT.
Patients with chronic long-standing lymphedema are at risk for developing a rare and deadly cutaneous angiosarcoma known as Stewart-Treves Syndrome.5 In most cases, the tumor is a result of lymphedema secondary to radical mastectomy. The syndrome can be broadly applied to any angiosarcoma present in a chronically lymphedematous body partly due to any cause. For point of clarification, the malignancy actually arises from blood vessels rather than lymphatic vessels and is more accurately described as a hemangiosarcoma. Although prognosis is poor, early recognition can improve a patients’ quality of life and reduce other deleterious complications associated with this syndrome.
|Atrophy||Thinning of the skin|
|Acute inflammatory episode||Probable infection/acute inflammation; cellulitis|
|Dermatitis||Inflammation and pruritic skin from irritant or allergen|
|Hyperkeratosis||Overgrowth of stratum corneum; thickening|
|Lichenified||Thick, leathery patches of skin that occurs in response to excessive itching or rubbing. Epidermis thickens in response to extra irritation.|
|Lymphangioma||Dilation of the initial lymphatics which herniate through skin|
|Lymphorrhea||Weeping of lymphatic fluid through skin; when dry, forms yellow crusts on skin, may lead to denudement|
|Lymphatic cysts||Blister like vesicles due to lymph fluid reflux; common locations include axilla, cubital, genital and popliteal areas|
|Papillomatosis||Cobblestone appearance relating to fibrosis of dilated lymphatics|
|Scaly telangiectasia||Flaking/scaling of skin; abnormal visible dilation of blood vessels|
|Tinea pedis||Fungal infection/athlete's foot|
|Vesicle||Small fluid filled blister|
Although there is currently no cure, patients with lymphedema can achieve good functional outcomes and quality-of-life improvements with proper interventions by trained health-care providers. Challenges arise when associated complications exacerbate lymphedema and/ or impact complete decongestive therapy. Although the more common complications were addressed in this article, many issues can arise in patients with lymphedema. The recognition of such complications, followed by proper management in concert with the patient’s lymphedema, requires astute awareness by health-care providers and access to trained practitioners who can manage the issues safely and individually for each patient.
Salvo, B, van Draanen, et al. Prophylaxis and management of acute radiation-induced skin reactions: a systematicreview of the literature. Curr Oncol. 2010 17(4):94–112.
Bray, S, Wolfson, et al. Acute and chronic cutaneous reactions to ionizing radiation therapy. Dermatol Ther (Heidelb). 2016; 6:185–206.
Radiation Induced Brachial medscape.com/article/316497-overview; accessedNovember 29, 2016.
Foldi, Folid, (eds). of Lymphology for Physicians and Lymphedema Therapists. 3rd Edition. San Francisco: Urban & Fischer;2003.
Stewart-Treves Syndrome. http://emedicine.medscape.com/ article/1102114-overview; accessed November 20,2016.