Pankaj Tiwari MD
Michelle Coriddi MD
Susan Lamp BSN, RN, CPSN
Plastic Surgical Nursing
**For information only. There is much controversery regarding surgical treatment for lymphedema**
The goal of this article was to define lymphedema as a disease entity, to introduce the American Lymphedema Framework Project, and to summarize current surgical strategies on the horizon in the surgical treatment of lymphedema.
Alongside the arterial and venous vasculature, the lymphatic system is a part of the circulatory system. Lymphatic channels primarily regulate the flow of fluid in the interstitium (Ellis, 2006). Under normal conditions, venous capillaries reabsorb 90% of the fluid in the tissues, and lymphatic channels absorb the remaining 10% of lymph fluid, proteins, and other molecules (Warren, Brorson, Borud, & Slavin, 2007). Lymphatic fluid passes to regional lymph node basins. Ultimately, the lymphatic fluid is transported back into the subclavian vein to enter the venous system via the thoracic duct.
Lymphedema is an external or internal manifestation of lymphatic insufficiency and deranged lymph transport (International Society of Lymphology, 2009). This insufficiency causes an accumulation of protein-rich interstitial fluid, leading to distention, proliferation of fatty tissue, and progressive fibrosis. Skin changes such as thickening and hair loss may occur. Progressive lymphedema without adequate management can lead to functional impairment, compromised quality of life, and deformity. Clinically, lymphedema is noted as swelling of the involved extremity. The head and neck, breast, or genitalia may also be affected (McWayne, & Heiney, 2005; Rockson, 2010; Smeltzer, & Stickler, 1985).
Lymphedema is generally classified as either primary or secondary. Primary lymphedema (hereditary) is related to congenital malformation of the lymphatic channels. Secondary lymphedema results from disruption to the lymphatic system. Primary lymphedema can result from any one of a number of disorders that may be sporadic or hereditary. Syndromes such as Milroy's disease and Prader-Willi syndrome have lymphedema as an element of their clinical manifestations to varying degrees. The estimated prevalence of primary lymphedema is 1.15 in 100,000 persons under the age of 20 years Milroy's Disease (Smeltzer & Stickler, 1985). In children, the two main causes are Milroy's disease and lymphedema distichiasis (International Society of Lymphology, 2009).
Secondary lymphedema is a consequence of removal or damage to lymph nodes, fibrosis of the nodes (postradiotherapy), and trauma or infection secondary_lymphedema Rockson, 2010). Upper extremity lymphedema is commonly associated with the treatment of breast cancer. The degree of lymphedema has been well recognized to correlate with the number of lymph nodes that have been removed and the extent of radiotherapy to the axillary region. Lower extremity lymphedema is most often seen in survivors of uterine and prostate cancer, as well as melanoma and lymphoma survivors (Meneses & McNees, 2007). Most cancer survivors develop lymphedema within 3 years of treatment (Petrek, Senie, Peters, & Rosen, 2001).
In addition to cancer ablation, side effects of advanced diseases such as congestive heart failure, neurological and liver disease, and end-stage renal disease can cause chronic edema. An increase in the bariatric population has also seen an increase in lymphedema incidence. Lympedema caused by the parasite wucheria bancrofti and transmitted by mosquitoes remains the most common cause of lymphedema worldwide. Unfortunately, no strategies employed to prevent the onset of lymphedema have proven fruitful to date. The term chronic edema has been adopted by European investigators to define a population of patients with long-standing edema (>3 months). Prevalence estimates for chronic edema are between 1.3 and 1.5 per thousand.
New clinical data suggest that some patients may have a primary disposition to lymphedema but that this first becomes clinically evident after a secondary eliciting event (Rockson, 2010). Lymphedema tarda is defined as debut after the age of 35 years. It is often associated with an eliciting factor such as trauma or an inflammatory reaction (Kerchner, Fleischer, & Yosipovitch, 2008).