Abstract
Many patients with cancer and post-treatment of cancer develop lymphedema months to years post-treatment. A clinical picture including the abnormal enlargement of a part of the body, associated with lymphostasis from dysfunction of the lymphatic system, is characteristic of lymphedema. Early lymphedema diagnosis and intervention appear to improve treatment outcomes. A person’s function and mobility may be negatively impacted by lymphedema. The patient with limb edema requires a thorough physical examination before the initiation of rehabilitation. Treatment options may include physical modalities, physical therapies, skin care and hygiene, and surgical, pharmacologic, and alternative methods, with complete decongestive therapy as the standard of care for lymphedema. More randomized controlled clinical studies and meta-analyses are needed to support treatment practices and compare efficacies.
Keywords
limb enlargement, lymphatic system, lymphedema, malignancy
Acknowledgment
We would like to acknowledge and thank Dr. Mabel Caban, MD, MS, and Lori Hall, PT, MS, CLT-LANA, who were the authors of this chapter in the previous text edition. Their writing and contribution was the framework and basis of this chapter update.
| Synonyms | |
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| ICD-10 Codes | |
| I97.2 | Post-mastectomy lymphedema syndrome |
| I89.0 | Lymphedema, not elsewhere classified, praecox and secondary |
| Q82.0 | Hereditary lymphedema |
Definition
Lymphedema is a condition of an abnormal enlargement of part of the body associated with lymphostasis from dysfunction of the lymphatic system ( Fig. 132.1 ). The dysfunction is due to the abnormal morphology of the lymphatic system, high production of lymph, or blockage in drainage. The lymphatic system consists of vessels with lymph traveling through lymph nodes. The lymphatic vessels transport fluid, plasma proteins, and other substances from the tissue back into the circulatory system. Lymphedema results from the inability to drain the lymphatic fluid and the accumulation of it in the limb. Chronic edema initiates a cascade of events that cause transformation of the tissue with skin thickening, fibrosis, deposition of fat, and unhealthy skin changes after months to years. A classification of lymphedema includes primary (heritable) and secondary (acquired) causes. Phlebolymphedema pertains to dysfunction of the lymphatic system in association with chronic venous insufficiency. Chronic venous insufficiency is a manifestation of deep venous thrombosis, angiodysplasia, lack of valves in the venous system, or varicosities.
Between 3 and 5 million people in the United States suffer from lymphedema, which in a significant number develops from cancer and its treatment. The incidence of post-mastectomy lymphedema ranges from 6% to 48%, depending on whether the patient received axillary radiation and surgery; if only lumpectomy is performed, the incidence drops to only 6%. A literature review, published in 2015, shows the incidence of breast cancer-related lymphedema changes to a range of 0% to 23% with use of sentinel lymph node biopsy, with a pooled incidence of 6.3%, while the incidence of lymphedema post-axillary lymph node dissection ranges from 11% to 57%, with a pooled incidence of 22.3%. About 80% of lymphedema cases involve the lower limbs and relate to peripheral vascular disease. Cervical cancer, melanoma, and pelvic cancers increase the frequency of secondary lymphedema of the lower extremity. An incidence of 27% is reported after cervical cancer treatment, and in women with endometrial and vulvar cancer, about 1% to 16% and 30%, respectively, developed lower limb lymphedema after treatment. Patients with melanoma and sentinel lymph node biopsy have a pooled incidence of 4.1%. Patients surviving treatment of head and neck cancer also experience lymphedema; however, more studies are needed to assess the incidence. Around the world, infection in the form of filariasis is the most common cause of lymphedema. An estimated 15 million people present with lower extremity lymphedema in filariasis-endemic regions in the world.
The radical excision of lymph nodes for malignant disease does not always cause lymphedema. Lymphedema could occur as a late complication of surgery for malignant disease because of gradual failure, making the distal lymphatics pump harder through the most proximal damaged ducts over time. The body compensates by regenerating the transected lymphatics to some extent. With irradiation, fibrous scarring forms, increasing the risk of lymphedema. The diagnosis depends on the history and characteristic changes of the skin on clinical examination (see Fig. 132.1 ). Some data suggest that monitoring for lymphedema should continue for many years, as lymphedema may not develop or be diagnosed until many months or years post-treatment.
Symptoms
The hallmark of lymphedema is painless swelling. Although many people believe that pain is a major component of lymphedema, this is not typically present, especially in the early stages. One of the first symptoms of lymphedema is tightness of the cutis and subcutis. Chronic indolent swelling of a limb is the most common presentation of lymphedema. Patients may complain that the arm feels heavy or that rings feel tight. Hypertrophy of fatty tissue occurs and eventually fibrosis too. In true lower extremity lymphedema, the feet are involved. The skin condition changes from being soft initially to becoming harder over time. Early or postsurgical lymphedema can spread proximally, an unusual finding after the first year, and lasts about 3 months after surgery.
Physical Examination
The patient with limb edema requires a thorough physical examination before the initiation of rehabilitation. Swelling of a limb with or without pain may be the presentation of an infection, deep venous thrombosis, or obstruction caused by a tumor at the level of the lymph nodes. Limb temperature and discoloration should be noted. In early stages, the edema is pitting, but as lymphedema progresses, the edema becomes non-pitting. In the absence of treatment, distal enlargement of the wrist or ankle occurs. A discrepancy of 2 cm in circumference or a difference of 200 mL in volume from side to side has been accepted for the diagnosis of lymphedema. A multitude of skin changes occur over time ( Table 132.1 ). Signs of lymphedema progression include a cool limb, skin thickening, limited range of motion, increased presence of papillomas, mycosis, and bacterial infection. Infection, such as cellulitis, erysipelas, and lymphangitis, must be recognized to prevent systemic malaise and worsening of the swelling.
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Staging of the condition helps assess progression and evaluate better treatment options. Stages of lymphedema are as follows:
Stage 0 Preclinical: disruption in the lymphatic system occurs; however, there is no swelling; patients may complain of other subjective symptoms such as a heaviness or fatigue in the limb. It can last months or years.
Stage I Reversible stage: swelling is soft and pitting and improved with limb elevation.
Stage II Spontaneously irreversible stage: increased swelling and change in tissues with fibrosis formed. Elevation of the limb is much less likely to be effective.
Stage III Lymphostatic elephantiasis: limb becomes extremely swollen with thickened skin.
Lymphedema does not cause neurologic deficits, but paresthesias and numbness may occur in the same distribution of the edema. Explanations include coexistence of neurologic damage from node dissection, peripheral nerve injury, plexopathy, and chemotherapy-induced polyneuropathy. The possibility of cancer recurrence must be ruled out with imaging, such as computed tomography or magnetic resonance imaging with contrast enhancement.
Lipedema can occur together with lymphedema. This is an abnormality of the deposition of fat involving the lower body, most commonly in women, of symmetric appearance. Swelling starts at the waist and finishes at the ankles without inclusion of the feet as opposed to lymphedema, in which the feet are involved. Lipedema does not respond to dietary intervention or complete decongestive therapy. If underlying hormonal imbalance exists, correction will improve this condition, and although it is controversial, liposuction can improve it in some cases.
Unilateral or bilateral venous insufficiency may be manifested with varicosities, fibrosis, thickened cutis, and brownish skin discoloration. Superimposed venous ulcers may occur in advanced cases.
Functional Limitations
Impaired mobility due to the excessive weight of the limb may occur in severe cases ( Table 131.2 ). Tight clothing, restriction of movement, and loss of body image and self-esteem are frequent complaints leading to psychosocial impairment. Women with breast cancer-related lymphedema report problems with their sexual well-being and social interaction, and report a lower health-related quality of life. In the past, avoidance of physical activity was the norm for fear of worsening the edema. This has changed with new evidence supporting the benefits of physical activity and exercise. Examination by a physician prior to starting an exercise routine, and follow-up monitoring to assess for worsening of symptoms of lymphedema are recommended. Wearing of a compression garment during exercise is advised ( Fig. 132.2 ). Further discussion of compression garment use is discussed in the Treatment section.
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