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Lymphoedema, or elephantiasis, is a tissue swelling caused by lymph stagnation as a result of congenital defects or acquired damage to the lymphatic vessels. This swelling is so advanced that it can even lead to amputation of the limbs where it most often develops. What are the causes and symptoms of elephantiasis? What is the treatment of lymphedema?

Elephantiasis , also known as elephantiasis or elephanthiasis, islymphedema(tissue swelling) of the last degree. It is caused by a disturbance in the drainage of the lymph (lymph, lymph fluid) that is produced by the lymph nodes. Normally, the lymph absorbs unnecessary substances (including proteins) from tissues to remove them. Damage to the lymphatic vessels means that these substances are not removed but remain in the tissues between cells.

Initially, the lymphatic system can cope with the overproduction of lymph (thanks to compensating mechanisms - it is the degree of latent edema), however, the excessive accumulation of these substances sooner or later leads to a slight swelling (1st degree lymphoedema), then to it slight hardening (2nd degree of edema), and finally to a large and constantly increasing swelling of the skin and subcutaneous tissue, i.e. elephantiasis. These changes most often affect the upper and lower limbs as well as the perineum (mainly in men).

Lymphoedema (elephantiasis): causes

Primary (congenital) lymphedemausually results from capillary development disorders - lack of capillaries (aplasia of the lymphatic vessels), their small number or defects (when they are too narrow - hypoplasia). Then the vessels are not able to drain the sufficient amount of lymph.

Another inherited cause of elephantiasis may beMilroy's disease(caused by a mutation of the vascular endothelial growth factor type 3 receptor), which she is inheriting.

Secondary lymphedema is the result of damage to these vessels, the concept of damage is very broad and includes:

  • chronic venous insufficiency (this is venous stasis, which is the result of backward blood flow in the veins, or a narrowing or obstruction of the veins);
  • injuries or sprains;
  • infections caused by fungi, bacteria, viruses, which lead to inflammation of the lymphatic vessels (in tropical countries, lymphatic obstruction by parasites - nematodes: pillars);
  • connective tissue diseases - rheumatoid arthritis, systemic sclerosis, psoriatic arthritis;
  • postoperative complications (e.g. after vascular surgeries - saphenous vein retrieval for coronary artery bypass grafting - CABG).

However, the most common cause of elephantiasis istreatment of malignant neoplasms , e.g. during mastectomy and other procedures that include removal of lymph nodes. It is associated with damage to the lymphatic system. Radiation therapy also often damages the lymphatic vessels.

Studies show that upper limb edema associated with breast cancer treatment occurs in 25-40% of patients. sick women undergoing treatment. Lymphedema of the lower limbs is also a very common (up to 60% of cases) complication of the treatment of pelvic tumors.

Lymphoedema (elephantiasis): symptoms

Lymphoedema usually develops gradually, which is caused by the gradual overloading of the lymphatic vessels. When the edema takes an advanced form, i.e. elephantiasis, the following appear:

  • huge swelling that keeps getting bigger;
  • hardened skin;
  • lumpy growths;
  • bumps;
  • dull feelings of heaviness;
  • problems with moving the limb;
  • pain (rarely).

The skin becomes susceptible to bacterial (e.g. rose) and fungal infections, which aggravate the swelling. In addition, the risk of developing deep, difficult to heal wounds increases.

Lymphoedema (elephantiasis): diagnosis

Usually the symptoms of the disease, which are very characteristic, are sufficient to make a final diagnosis.

Lymphoedema (elephantiasis): treatment

Treatment for elephantiasis can takeeven several monthsand includes:

  • application of special creams that protect the skin against infections;
  • administering medications to reduce puffiness;
  • lymphatic drainage - a special massage can displace lymph from the swollen area of ​​the skin to the part that functions properly, and thus - to drain the lymph;
  • kompresjoterapię (compression therapy) - bandages and compression clothing improve muscle function, and thus also support lymph flow;
  • motor rehabilitation - exercises help drain the lymph.

However, in elephantiasis, the swelling is so severe thatoften requires surgeryto excise the overgrown and swollen subcutaneous tissue. However, then the risk of skin necrosis increases. Microsurgery, which involves the transplantation of lymph vessels in order to create lymphatic connections, may also be helpful.

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Immune system