Lymphedema is a chronic and progressive condition caused by disruption of lymphatic system function. Interruption of the functioning of the lymphatic system leads to fluid accumulation in the tissues, causing edema in various parts of the body where the lymphatic system is affected. The most commonly affected sites are the lower extremity and the genitalia; However, upper extremity lymphedema is commonly seen secondary to breast cancer surgery.
Lymphedema can be classified into primary and secondary type. Primary lymphedema is hereditary and is caused by mutation in specific genes, presenting as an autosomal dominant trait, leading to abnormalities in the development of the lymphatic system. Primary lymphedema can be subdivided into Milroy’s disease, early lymphedema and late lymphedema. Secondary lymphedema is acquired mainly due to surgery, trauma, infection infestation, tumor or post venous thrombosis. Both primary and secondary lymphedema are clinically characteristic of chronic edema (swelling), localized pain or discomfort, atrophic skin changes, and secondary infections.
The diagnosis of lymphedema is made clinically through thorough evaluation and physical examination. Family history is crucial in the diagnosis of primary lymphedema. Characteristic symptoms include chronic swelling, progressive atrophic skin changes, and recurrent infections. History of trauma and surgery should be treated appropriately.
The circumference and volume of the affected versus unaffected limb can be measured and the circumference> 2 cm and volume> 200 ml can confirm the diagnosis. Volume can be measured by tape, water displacement or perometry. Perometry involves infrared optical electronic scanner to demonstrate small changes in the volume of the affected limb. Tonometry is used to assess tissue strength by applying compression with a tonometer and this can be used to measure fibrosis and skin texture. Bioimpedance Spectroscopy (BIS) measures the body’s water content and helps assess the electrical conductance of extracellular fluid; It is effective for detecting the early stages of lymphedema.
Imaging techniques include lymphoscintigraphy, magnetic resonance lymphangiography (MRL), ultrasound, computed tomography, and near-infrared fluorescence imaging (NIFR). Lymphoscintigraphy utilizes 99m technetium sulfur colloid for lymph flow assessment. NIFR uses green indocyanine to visualize superficial lymphatic flow and functional lymphatic vessels; It is used to determine the severity of the condition. Magnetic resonance lymphangiography (MRL) is a new test involving gadolinium injection to determine lymphatic channels.
Treatment is based on the severity of lymphedema. However, early and accurate diagnosis is crucial, as effective lymphedema treatment can be established in the early stages of the disease.
Lymphedema and lymphedema-related fibrosis use guidelines supported by the National Cancer Institute (NCI) for the diagnosis of lymphedema. Lymphedema classification is based on the measurement of swelling as well as changes in the appearance of the affected skin.
Grade I: There is a 5-10% difference between the affected and unaffected limbs in volume and circumference. Sting edema is present and, on close examination, there is swelling or loss of anatomical architecture. Soft tissue response is minimal to moderate at limb elevation or compression and texture is spongy to moderately firm.
Grade II: There is more than 10 to 30% difference between the affected and unaffected limbs in volume and circumference. There is an obvious loss of anatomical architecture, skin folds and normal shape of the affected limb. There is a marked increase in the density and firmness of the affected skin and the texture of the skin shows signs of ‘binding’.
Grade III: There is more than 30% difference between the affected and unaffected limbs, with major changes in limb shape. There is an inability to perform daily activities and there may be lymph leakage on the surface of the skin. There is marked density and firmness of the skin with evident binding.
Grade IV: The condition becomes disabling and lymphangiosarcoma is diagnosed, which is a rare tumor that develops into severe, chronic, untreated lymphedema.
The International Society of Lympology uses the staging system for staging lymphedema.
Stage 0: Latent or Subclinical Lymphedema
Stage I: Early or mild lymphedema with or without corrosion
Stage II: Moderate lymphedema with corrosion and skin changes
Stage III: Severe lymphedema without corrosion and marked skin changes.