eMedicine Specialties > General Surgery > Lymphatic System

Lymphedema: Treatment

Author: Don R Revis Jr, MD, Consulting Staff, Department of Surgery, Division of Plastic and Reconstructive Surgery, University of Florida College of Medicine
Contributor Information and Disclosures

Updated: Sep 29, 2009

Treatment

Medical Therapy

The goal of conservative therapy is to eliminate protein stagnation and to restore normal lymphatic circulation. Initiate therapy as early as possible before extensive irreversible fibrosclerotic changes occur in the interstitium. These techniques are often cumbersome, uncomfortable, inconvenient, and time-consuming. Strict compliance is essential, and treatment lasts throughout the lifetime of the individual. The majority of compliant patients can be treated successfully with conservative measures.^3,4

Meticulous hygiene is necessary to remove keratinaceous debris and bacteria. Cleanse the skin regularly and dry thoroughly. Regular inspection is necessary to identify any open wounds or developing cellulitis. Bland skin moisturizers applied conservatively may ameliorate cracking and furrowing. Even with excellent skin care, chronic cellulitis may occur. At the earliest signs of infection, institute topical or systemic antifungal or antimicrobial therapy to prevent the development of sepsis. Long-term prophylactic antimicrobial treatment with agents, such as penicillin, cephalexin, or erythromycin, may be required in 15-25% of patients experiencing recurrent lymphangitis or cellulitis. Although relatively uncommon in this country, filariasis is treated with diethylcarbamazine.

Encourage patients to lose weight, avoid even minor trauma, and avoid constrictive clothing that might have a tourniquet effect. Encourage elevation of the affected extremity whenever possible, particularly at night. For lower extremity lymphedema, this may be accomplished by elevating the foot of the bed to an appropriate level.

Patients should use compression garments continuously during the day. They may be removed at night when the extremity is elevated in bed, but they should be replaced promptly each morning. To encourage compliance, the elastic compression garments must fit appropriately. Garments should be custom fit when the extremity is decompressed, they should be comfortable, and they should not have a tourniquet effect. They should also have graduated compression, increasing from distal to proximal, on the affected extremity.

Intermittent pneumatic pump compression therapy may also be instituted on an outpatient basis or in the home. These manual lymphatic devices are most appropriate prior to fibrosclerotic evolution, and they assist in preventing fibrosclerotic evolution of the condition. These devices provide sequential active compression from distal to proximal, effectively milking the lymph from the extremity. In Europe, the best available nonsurgical therapy is manual lymphatic drainage according to the Vodder and/or Leduc techniques. Compression garments are essential between treatments. Contraindications to this therapy include congestive heart failure, deep vein thrombosis, and active infection. Similarly, other authors advocate manual massage of the affected extremity to recruit collateral vessels so that the accumulated lymph can be drained into neighboring regions with normally functioning lymphatics.

Diuretics have no role in the treatment of lymphedema.

Benzopyrenes, including flavonoids and coumarin, have become a useful adjuvant in other countries but are currently not available for clinical use in the United States. These drugs bind to accumulated interstitial proteins, inducing macrophage phagocytosis and proteolysis. The resulting protein fragments pass more readily into the venous capillaries and are removed by the vascular system.

Surgical Therapy

Surgical treatment is palliative, not curative, and it does not obviate the need for continued medical therapy. Moreover, it is rarely indicated as the primary treatment modality. Rather, reserve surgical treatment for those who do not improve with conservative measures or in cases where the extremity is so large that it impairs daily activities and prevents successful conservative management. The goals of surgical therapy are volume reduction to improve function, facilitation of conservative therapy, and prevention of complications. A myriad of surgical procedures have been advocated, reflecting a lack of clear superiority of one procedure over the others. In general, surgical procedures are classified as physiologic or excisional.⁵

Physiologic procedures attempt to improve lymphatic drainage. Multiple techniques have been described, including omental transposition, buried dermal flaps, enteromesenteric bridging, lymphangioplasty, and microvascular lympholymphatic or lymphovenous anastomoses. None of these techniques has clearly documented favorable long-term results. Further evaluation is necessary. Moreover, many of these physiologic techniques also include an excisional component, making it difficult to distinguish between the 2 approaches.

Excisional techniques remove the affected tissues, thus reducing the lymphedema load. Some authors advocate suction-assisted removal of subcutaneous tissues, but this technique is difficult because of the extensive subcutaneous fibrosis that is present. Additionally, this approach does not reduce the skin envelope, and the lymphedema often rapidly recurs. Suction-assisted removal of subcutaneous tissue followed by excision of the excess skin envelope has no clear advantage over direct excisional techniques alone.

The Charles procedure is another quite radical excisional technique. This procedure involves the total excision of all skin and subcutaneous tissue from the affected extremity. The underlying fascia is then grafted, using the skin that has been excised. This technique is extreme and is reserved for only the most severe cases. Complications include ulceration, hyperkeratosis, keloid formation, hyperpigmentation, weeping dermatitis, and severe cosmetic deformity.

Van der Walt et al developed a modified Charles procedure in which negative-pressure dressing was employed following debulking surgery, with skin grafting delayed for 5-7 days.⁶ In a report on 8 patients suffering from severe primary lymphedema who underwent the procedure, the authors reported that the patients experienced no major complications. Minor complications, including operative blood loss and, in 3 patients, the need for additional grafting, did occur.

Staged excision has become the option of choice for many authors. This procedure involves removing only a portion of skin and subcutaneous tissue, followed by primary closure. After approximately 3 months, the procedure is repeated on a different area of the extremity. This procedure is safe, reliable, and demonstrates the most consistent improvement with the lowest incidence of complications.

Preoperative Details

Prior to surgery, appropriate documentation is necessary to evaluate the outcome of treatment. This includes photographic documentation as well as extremity measurements. Ideally, these measurements are of limb volume by water displacement, although some rely on circumferential measurements alone. Obtain measurements and photographs at the same time of day each time, document affected extremities and contralateral extremities, and preferably conduct documentation in the morning after extremity elevation in bed overnight.

Institute strict elevation and pneumatic compression, if available, 24-72 hours prior to surgery. This allows maximum excision to be performed. The extremity must also be free of infection at the time of surgery, and a single dose of preoperative intravenous antibiotic is administered.

Intraoperative Details

• After the establishment of appropriate anesthesia, the operative field is sterilized and draped according to surgeon preference.
• A pneumatic tourniquet is placed at the root of the extremity and insufflated after the extremity has been exsanguinated.
• A longitudinal incision is made along the entire extremity, and skin flaps, 1.0-1.5 cm thick, are elevated.
• Subcutaneous tissue is then excised, taking care not to injure peripheral sensory nerves.
• Some authors also excise a strip of deep fascia, but this should not be performed around joints because it may cause instability.
• Once the subcutaneous excision is complete, redundant skin is resected. Often, a strip that is 5-10 cm wide may be removed.
• The wound is closed over suction drains.

Postoperative Details

• Postoperatively, the extremity is immobilized in a splint and elevated while the patient is placed on strict bed rest.
• Antibiotics may be continued until drain removal, according to surgeon preference.
• Drains are typically removed at 5-7 days postoperatively, as dictated by a decrease in drain output.
• Sutures are removed at 10-14 days and replaced by Steri-Strips.
• Measure the patient for a new compression garment when the new dimensions of the extremity have stabilized.
• After approximately 10 days, the patient may gradually begin dependency on the extremity with compression bandages or an elastic garment in place.

Follow-up

• Once discharged from the hospital, the patient should be seen regularly in the outpatient clinic.
• Patients must wear compression garments for 4-6 weeks continuously, and dependency on the involved extremity may be gradually increased at the discretion of the treating physician.
• Once healed to physician satisfaction, the patient may return to a normal routine of elevation at night and compression garment therapy during the day.
• Follow-up visits should include documentation of circumferential measurement or water displacement of the affected and contralateral extremities as well as photographic documentation.
• When staging procedures, allow approximately 3 months between procedures to allow complete healing of the initial operative site.

Complications

Patients with chronic lymphedema for 10 years have a 10% risk of developing lymphangiosarcoma, the most dreaded complication of this disease. Patients with this tumor commonly present with a reddish purple discoloration or nodule that tends to form satellite lesions. It may be confused with Kaposi sarcoma or traumatic ecchymosis. This tumor is highly aggressive, requires radical amputation of the involved extremity, and has a very poor prognosis. The 5-year survival rate is less than 10%, and the average survival following diagnosis is 19 months. This malignant degeneration is most commonly observed in patients with postmastectomy lymphedema (Stewart-Treves syndrome), where incidence is estimated to be 0.5%.

Other complications of lymphedema include recurrent bouts of cellulitis and/or lymphangitis, deep venous thrombosis, severe functional impairment, cosmetic embarrassment, and necessary amputation. Complications following surgery are common and include partial wound separation, seroma, hematoma, skin necrosis, and exacerbation of foot or hand edema.

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