Lymphedema

ALSO KNOWN AS: Lymphatic obstruction, Milroy’s disease, Meige’s disease

RELATED CONDITIONS: Cancer, malformations of the lymph system

DEFINITION: Lymphedema is a blockage in the lymphatic system that results in swelling, or edema. The lymph system is a network of channels that move lymph, a clear fluid, around cells and through nodes that filter harmful substances such as bacteria.

Risk factors: Surgery and radiation are most often associated with lymphedema. If the patient receives radiation to the underarm or has removed during surgery or as part of a sentinel lymph node biopsy, the lymph channel may be damaged. Lymph node removal for related to cancer spread is common and may also be done in the chest, groin, pelvic, and neck areas. Tumor growth may also cause lymphedema by compression.

Etiology and the disease process: There are two kinds of lymphedemaprimary and secondary. Primary lymphedema is a rare inherited condition, and secondary lymphedema is caused by blockages from infection, surgery, radiation therapy scar tissue, pressure on lymph nodes from a growing tumor, or removal of lymph nodes during surgery. Primary lymphedema is due to a malformation in the lymph system present at birth. Secondary lymphedema is a mechanical interruption in the normal flow of lymph.

When an interruption in the lymph system occurs, the ability to transport fluid is impaired, leading to swelling as the fluid collects in the tissues below the area of blockage. For example, if lymph nodes under the arm or in the groin are removed, the arm or leg may swell. The fluid that collects is interstitial fluid, which causes inflammation. As the fluid collects, the swelling progresses, and the patients note an increase in the size of their arms, legs, or abdomen, depending on the blockage site. Because the disease is progressive, the swelling continues and may lead to fatigue, the inability to fit clothing over the affected area, and the inability to carry on the activities of daily living. As the disease progresses, the skin in the affected area may become thickened and begin to resemble an orange peel, known as peau d’orange. The skin may break down easily, leading to the oozing of fluid through the skin and, ultimately, infection. Rarely, this results in lymphangiosarcoma.

Incidence: Incidence rates for secondary lymphedema vary significantly by site. Some 8 to 56 percent of breast cancer patients with lymph node removal under the arm develop lymphedema within two years after the surgery. Patients who received treatment for vaginal, ovarian, endometrial, cervical, prostate, colorectal, or vulvar cancer are most likely to develop lymphodema in the abdomen, genitals, or legs. Individuals with head and neck cancer may develop lymphodema in areas not easily visible, such as in the throat.

Symptoms: Swelling is the most common and obvious symptom of lymphedema. The patient may also report feeling heaviness in the affected body part. Pain and weakness in the limb may be evident to the patient. Sensations may decrease, including an inability to feel heat or cold. As the disease progresses, the skin becomes hard and loses elasticity, and the limb may become two to three times its normal size. Lymphedema in the abdominal area may result in bowel and bladder problems. If untreated, fluid oozing from the skin may be noticed, and the skin may seem to disintegrate, leading to open sores.

Screening and diagnosis: Patients at risk for lymphedema should be screened at each doctor’s visit and educated on the signs and symptoms. Lymphedema is generally diagnosed after patients complain of symptoms such as swelling and fullness. Physical examination, medical history, medicines taken, and changes since the previous examination are important in diagnosis. A lymphedema diagnosis is presumed if a patient's arm or leg is 2 centimeters (4/5 of an inch) larger than the other arm or leg. Lymphoscintigraphy, computed tomography (CT), or X-ray may be used in the diagnostic process to determine the source of the condition.

To stage lymphedema, medical professionals often use the International Society of Lymphology (ISL) staging system. This system ranges from Stage 0, characterized by an abnormal flow of lymph with no physical symptoms, to Stage III, characterized by swollen, hard, stiff skin causing the patient pain and limited mobility. Measurement and recording of the affected area's circumference are essential to monitor the progression of the disease.

Treatment and therapy: Treatment of lymphedema is primarily mechanical—elevation of the affected area, manual lymphatic drainage using gentle massage to move fluid toward the center of the body (decongestive therapy), wearing of custom-fitted compression garments on the affected limb, and practicing good skin care and injury protection. Antibiotics may be necessary to prevent or treat infections, but other drugs are not generally effective. Patients are often encouraged to watch their weight, exercise appropriately, and eat protein-rich foods. Bowel and bladder complications, such as constipation and urine retention, may require treatment. Because the disease is progressive and treatment is based on symptoms, management, and control are the therapies of choice. Patient education is critical to treatment and must be an essential part of the therapy for lymphedema. Surgery for lymphedema may be appropriate for patients with severe symptoms. In a vascularized lymph node transfer, healthy lymph nodes are surgically placed in the affected area, allowing normal lymph flow to return. A Charles procedure or liposuction may be performed to remove the affected tissue. Lymphaticovenous anastomosis is a less invasive option in which a surgeon connects healthy veins to the lymph system.

Prognosis, prevention, and outcomes: No clinical studies support actions to prevent lymphedema. Lymphedema has no cure, so the prognosis depends on the patient’s compliance with treatment measures. The use of sentinel lymph node biopsy rather than aggressive lymph node removal may contribute to decreasing disease incidence. The key to an optimal outcome is early recognition and treatment of the symptoms and compliance with ongoing treatment.

Bibliography

Cheng, Ming-Huei, et al. Principles and Practice of Lymphedema Surgery. 2nd ed., Elsevier, 2022.

"Lymphedema." American Cancer Society, 9 Apr. 2024, www.cancer.org/cancer/managing-cancer/side-effects/swelling/lymphedema.html. Accessed 20 June 2024.

"Lymphedema." National Cancer Institute, 6 Mar. 2024, www.cancer.gov/about-cancer/treatment/side-effects/lymphedema. Accessed 20 June 2024.

"Lymphedema (PDQ®)–Health Professional Version." National Cancer Institute, 30 Apr. 2024, www.cancer.gov/about-cancer/treatment/side-effects/lymphedema/lymphedema-hp-pdq. Accessed 20 June 2024.

Sachdev, Poonam. "Lymphedema: What to Know." WebMD, 3 Apr. 2024, www.webmd.com/a-to-z-guides/what-is-lymphedema. Accessed 20 June 2024.

Shallwani, Shirin M., et al. “Feasibility of a Pilot Randomized Controlled Trial Examining a Multidimensional Intervention in Women with Gynecological Cancer at Risk of Lymphedema.” Current Oncology, vol. 28, no. 1, 13 Jan. 2021, pp. 455-470. doi:10.3390/curroncol28010048.

Wagner, Johannes M., et al. “Prophylactic Lymphaticovenous Anastomoses for Resection of Soft Tissue Tumors of the Thigh to Prevent Secondary Lymphedema: A Retrospective Comparative Cohort Analysis.” Microsurgery, vol. 42, no. 3, 2022, pp. 239-245. doi:10.1002/micr.30850.