Introduction

The ideal treatment for lymphedema of the limbs must restore function and normal cosmetic appearance. Physiotherapy (manual drainages, pressotherapy, compression, bandages) is the usual treatment for chronic lymphedema and is considered by many as the only treatment for long-term management. It is not a curative therapy, but helps to control the evolution of the disease. Chronic lymphedema is a progressive condition, characterized by a degenerative and inflammatory process resulting in diffuse, irreversible tissue fibrosis. Surgical treatment is an alternative method of controlling chronic lymphedema.

Precise diagnosis of lymphedema has achieved major progress: lymphangiography with oil and lymphoscintigraphy were once very useful, but these examinations have important drawbacks (eg, infection, pulmonary embolism). Magnetic resonance lymphangiography (MRL) with T2-weighted imaging has greater sensitivity, and allows complete visualization of the lymphatic system without any injection.

Free autologous lymph node transplantation in hypoplastic forms of lymphedema is a new approach for lymphatic reconstruction, a more anatomic strategy compared with the multiple lymphovenous anastomoses. Recent findings on the growth hormones produced by the lymph nodes permit further understanding of the efficacy of these procedures. Three lymph node flaps can be used, depending on the affected segment and available donor sites. These flaps are located on the inguinal, thoracic, or cervical area. The transplanted nodes pump the extracellular liquid responsible for lymphedema formation, and contain germinative cells that improve immune functions ( Figs. 1 and 2 ).

A 34-year-old woman suffering from lymphedema since puberty, resistant to intensive physiotherapy.

Patient in Fig. 1 , 1 year after free node transplantation at the right inguinal region.

Human lymph nodes express high levels of the lymphatic vessel growth factor vascular endothelial growth factor (VEGF)-c. This growth hormone is responsible for stimulating lymphangiogenesis in the treated limb. Saaristo and colleagues compared the production of this growth factor by different tissues of the immune and hematopoietic systems. Lymph nodes expressed the highest levels of VEGF-c among the tissues tested ( Fig. 3 ).

High expression of VEGF-c by lymph node tissue.

( From Saaristo AM, Niemi TS, Viitanen TP, et al. Microvascular breast reconstruction and lymph node transfer for postmastectomy lymphedema patients. Ann Surg 2012;255:472; with permission.)

Each living node contains a plexus between the lymphatic and venous systems. The transplanted lymph nodes also probably work as a biologic lymphovenous anastomosis ( Fig. 4 ).

Anastomotic plexus between veins and lymphatic vessels in the node.

( Courtesy of S. Godart.)

Clinical presentation

Alterations of lymph drainage induce stasis of the lymph and progressive tissue changes with enlargement of the subcutaneous tissue and thickening of the skin. Secondary infections, immune disorders, and cosmetic and psychosocial impairment can severely affect patients with lymphedema.

Diagnosis of lymphedema is done mainly by clinical assessment. A detailed history, clinical evaluation, and physical examination are necessary. Age of inset, episodes of infections and inflammatory attacks, previous medical treatments, and visits to tropical countries with endemic filariasis should be recorded. Transitory edema of the affected limb and a family history of limb edema should be questioned. Lymphedema is usually painless, but a sensation of heaviness of the affected limb is a common complain. Acute inset or worsening of the lymphedema can produce pain, caused by distention of the aponevrosis around the deep lymphatic system.

In the lower limbs, lymphedema is usually unilateral and, if it is bilateral, it is generally asymmetric. In young adults the lymphedema in the lower limb is unilateral in 70% of cases, and bilateral in the other 30%. The lower body, upper extremities (unilateral or bilateral), the abdomen, genital area, and trunk can be involved. The skin folds at the base of the toes and fingers are broadened. This is caused by excessive skin thickness and fluid accumulation with tissue overgrowth (Stemmer sign).

Skin changes include pinky-red discoloration, hyperkeratosis, papillomatosis, and lymph vesicles. Interdigital mycosis must be treated to prevent secondary infections. Associated venous insufficiency may cause ulcerations of the skin.

Elephantiasis can be observed in hypoplastic and hyperplasic forms, but the disorders of the skin and the multiple folds are very difficult to treat. Evolution into lymphangiosarcoma is rare, but can occur if elephantiasis is not treated.

Perimetry of the limb is an indirect assessment of its volume. It is a traditional tool to evaluate limb changes. Measurements are made at the level of the two distal major joints (knee and ankle in the lower limb), 10 and 20 cm proximal to them.

Clinical presentation

Alterations of lymph drainage induce stasis of the lymph and progressive tissue changes with enlargement of the subcutaneous tissue and thickening of the skin. Secondary infections, immune disorders, and cosmetic and psychosocial impairment can severely affect patients with lymphedema.

Diagnosis of lymphedema is done mainly by clinical assessment. A detailed history, clinical evaluation, and physical examination are necessary. Age of inset, episodes of infections and inflammatory attacks, previous medical treatments, and visits to tropical countries with endemic filariasis should be recorded. Transitory edema of the affected limb and a family history of limb edema should be questioned. Lymphedema is usually painless, but a sensation of heaviness of the affected limb is a common complain. Acute inset or worsening of the lymphedema can produce pain, caused by distention of the aponevrosis around the deep lymphatic system.

In the lower limbs, lymphedema is usually unilateral and, if it is bilateral, it is generally asymmetric. In young adults the lymphedema in the lower limb is unilateral in 70% of cases, and bilateral in the other 30%. The lower body, upper extremities (unilateral or bilateral), the abdomen, genital area, and trunk can be involved. The skin folds at the base of the toes and fingers are broadened. This is caused by excessive skin thickness and fluid accumulation with tissue overgrowth (Stemmer sign).

Skin changes include pinky-red discoloration, hyperkeratosis, papillomatosis, and lymph vesicles. Interdigital mycosis must be treated to prevent secondary infections. Associated venous insufficiency may cause ulcerations of the skin.

Elephantiasis can be observed in hypoplastic and hyperplasic forms, but the disorders of the skin and the multiple folds are very difficult to treat. Evolution into lymphangiosarcoma is rare, but can occur if elephantiasis is not treated.

Perimetry of the limb is an indirect assessment of its volume. It is a traditional tool to evaluate limb changes. Measurements are made at the level of the two distal major joints (knee and ankle in the lower limb), 10 and 20 cm proximal to them.