The Campisi Approach for Lymphatic Surgery

Current Concepts of Peripheral Lymphedema

Chronic peripheral lymphedema is progressive and relatively painless swelling of any peripheral tissue, including the limbs, head and neck, breast, trunk, or genitals, that is the result of a reduced transport capacity of the lymphatic system. Chronic lymphedema can be classified as primary or secondary, according to the etiology. In patients with secondary lymphedema, a specific external cause (filariasis, previous surgery, radiation, malignancy, infection or inflammation, trauma, etc.) can be identified and is believed to impact on a presumed previously normally functioning lymphatic system by causing an obstruction in the lymphatic flow (either from the direct removal of lymph nodes and/or lymphatic vessels or damage to the same).

The majority of the clinical conditions that are considered to be primary lymphedema are due to truncular lymphatic malformations that arise during the final stages of the lymphangiogenesis, when there is the development of lymphatic trunks, vessels, and nodes. These malformations result in hypoplasia, hyperplasia, or aplasia of the lymphatic vessels and/or the lymph nodes and may clinically manifest as obstruction or dilatation of the lymphatic vessels. In reality, it is not possible to determine if a peripheral lymphedema is primary or secondary simply from a physical or instrumental examination of the affected limb, as the clinical picture is the same regardless of etiology. What is useful, however, is to classify the lymphedema by stage of disease, as this predicts treatment outcome.

In order to provide such a comprehensive classification system of lymphedema that encompasses immunohistopathological criteria, level of clinically evident edema, lymphoscintigraphic findings, and level of physical disability, we developed a three-stage model ( Table 21.1 ). In clinical practice, stages IA, IB, IIA, and IIB can be considered as early manifestations of the disease, and stages IIIA and IIIB (properly known as elephantiasis), as chronic and advanced. It should be noted that lymphedema is a progressive disease and, without adequate treatment, can move rapidly between the stages.

The development, within the last 50 years, of newer surgical techniques to restore lymphatic flow in patients with lymphedema offers a treatment that targets more than symptomatic relief but provides a functional repair of the underlying problem of lymph-stasis. Initial surgical methods were ablative and employed in the advanced stages of disease, with significant levels of fibrotic tissue. However, these were often characterized by significant scarring, poor wound healing, and infection and have largely been abandoned as microsurgical techniques gained popularity.

Initial microsurgical procedures involved lymph nodal-venous shunts, but these had a high failure rate due to the thrombogenic effect of the lymph node pulp entering the venous system and re-endothelization of the lymph node surface. Subsequent approaches involved anastomosing lymphatic vessels directly to collateral veins (lymphatic-venous anastomoses [LVAs]) or to venular vessels (lymphatic-venular anastomoses; supermicrosurgery). These modifications improved the long-term outcome of lymphatic microsurgery, but the efficacy, in terms of volume reduction and long-term stability, remains highly variable between surgical centers worldwide.

The Center of Lymphatic Surgery and Microsurgery in Genoa, Italy, has obtained excellent stable clinical outcomes for over 40 years by utilizing multiple lymphatic-venous anastomoses (MLVA)/multiple lymphatic-venous-lymphatic anastomoses (MLVLA) techniques. Anastomoses are performed at a single surgical site using larger lymphatic vessels attached to collateral branches of the main veins close to vein valves to avoid backflow of blood and the closure of the anastomosis. A single-site approach also minimizes the number of incisions and, thereby, potential entry sites for infection. The retrospective evaluation of this considerable surgical experience is described with reference to the treatment of both primary and secondary peripheral lymphedema.

Patient Selection

Refractory lymphedema unresponsive to conservative treatment measures may be appropriately managed by surgical means. Indications for surgery include insufficient volume reduction by appropriate conservative methods (<50% reduction), recurrent lymphangitis or erysipelas episodes, intractable pain or discomfort usually associated with the excess swelling and inflammation, loss of limb function and increasing disability, patient dissatisfaction with previous treatment outcomes, and willingness to proceed with surgery. Relative contraindications to lymphatic microsurgery are few but include lymphatic-lymph nodal aplasia (exceedingly rare), diffuse metastatic carcinomas, and extremely advanced lymphedema (stage IIIB) unresponsive to conservative measures.

Preoperative Management