Primary and secondary lymphedema

Lymphedema may be primary or secondary. Primary lymphedema is a lymphatic malformation developing during the later stage of lymphangiogenesis. In contrast, secondary lymphedema is the result of disruption or obstruction of the lymphatic system.

Secondary lymphedema can occur as a consequence of tumors, surgery, infection, inflammation, radiation therapy and trauma. Secondary lymphedema is one of the most significant complications after the surgical treatment of breast cancer, with significant impact on the quality of life.

Lymphedema is divided in three stages.
Stage IA (latent lymphedema) presents without clinical evidence of edema, but with impaired lymph transport capacity.
Stage IB (initial lymphedema) is characterized by edema that totally or partially decreases by rest and draining position.
In Stage IIA (increasing lymphedema) vanishing lymph transport capacity is seen and fibroindurative skin changes appear.
Stage IIB (column shaped limb fibrolymphedema) presents with lymphostatic skin changes and worsening disability.
In Stage IIIA (elephantiasis) scleroindurative pachydermitis and papillomatous lymphostatic verrucosis is observed together with life-threatening disability, and Stage IIIIB is extreme elephantiasis with total disability.

Diagnosis of lymphedema is based on the clinical features of the disease (extremity circumference measurement before and after surgery; a difference of more than 2 cm points to the development of lymphedema). Diagnostic imaging (plain radiographs, duplex ultrasonography, radionuclide lymphoscintigraphy and other imaging modalities) should be considered to rule out other causes of increased extremity circumferences, or to establish the diagnosis of lymphedema when in doubt.

Treatment should start with manual lymphatic drainage and compression bandage-centered decongestive lymphatic therapy. An alternative is sequential intermittent pneumatic compression using pumping devices. Radial shock wave therapy (RSWT) has been demonstrated being efficient for lymphedema stages IIA and IIB. Surgery should be considered for recalcitrant cases of lymphedema not responding or responding poorly to the aforementioned treatment options. Surgical options include lympho-venous or lympho-venous-lymphatic bypass anastomosis, lympho-lymphatic segmental interposition, free lymph node transplantation, and ablative surgery in case of massive limb changes or fibrotic induration.