Lymphedema is a pathological condition accompanied by increasing swelling of the soft tissues of the affected area (most often the lower extremities). It is manifested by an increase in the volume of the affected part of the body, a feeling of bursting and heaviness, trophic disorders. Lymphedema is diagnosed by vascular ultrasound, lymphangiography, lymphoscintigraphy. Treatment in the initial stages is conservative (bandaging, massage, pneumocompression), with its ineffectiveness, surgical techniques are used.
Lymphedema is a congenital or acquired condition based on lymphostasis and lymphatic edema of the affected part of the body. Edema in lymphedema develops due to a violation of the outflow of fluid through the lymphatic vessels. The disease is widespread. Violation of lymph outflow of varying degrees is observed in every tenth inhabitant of the Earth. More than 10 million people worldwide suffer from lymphedema, which has developed against the background of a chronic infection. Patients account for 2.5-7% of all patients with peripheral vascular lesions.
Depending on the etiological prerequisites , there are two types of lymphedema:
- Primary (congenital) lymphedema. It is caused by congenital pathology of lymphatic vessels. As a rule, it develops in childhood and adolescence (80% of patients are girls under the age of 18). More often affects the lower extremities, less often – the upper ones. It is usually two-sided. In 6% of cases, hereditary lymphedema occurs (Nonne-Milroy syndrome, Meige syndrome). In the remaining 94% of patients, primary lymphedema develops due to congenital aplasia or hypoplasia of lymphatic vessels. Compensated primary lymphedema often progresses rapidly after pregnancy or injury.
- Secondary (acquired) lymphedema. The defeat of the lymphatic system is caused by infection (for example, erysipelas), trauma, burn, surgical intervention associated with the removal or damage of lymph nodes. It is most pronounced if the difficulty of lymph outflow is combined with a violation of venous outflow (during irradiation or extirpation of inguinal and axillary lymph nodes). Secondary lymphedema is more common than primary.
Patients with lymphedema complain of pain, weakness, a feeling of bursting, heaviness in the affected limb, restriction of joint mobility. The skin in the affected area is pale, the pattern of the network of subcutaneous veins is not determined, the skin fold is thickened.
Congenital lymphedema initially affects the distal parts of the extremities (feet or hands). Patients develop a painless dense swelling of the fingers, spreading to the foot and ankle joint (if the upper limb is affected, to the hand and wrist joint). As the lymphedema progresses, the swelling spreads to the lower leg and thigh. The patient’s legs become like columns. In the area of the joints, folds of edematous soft tissues form over time. The folds on the back of the foot are not pronounced. The skin resembles an orange peel.
With acquired lymphedema, the proximal parts of the extremities are primarily affected, just below the level of the damaged lymph nodes on the hip or shoulder. Subsequently, the edema spreads to the distal parts of the extremities. When collecting anamnesis, it should be borne in mind that lymphedema can develop in the long term after the defeat or removal of lymph nodes. Sometimes the first signs of the disease appear 10-15 years after surgery, injury or radiation exposure.
With lymphedema, tissue nutrition is disrupted. There are areas of hyperkeratosis. The altered skin cracks, ulcers appear. Sometimes lymphatic fistulas develop between the fingers. Lymphedema of any genesis is often complicated by erysipelas, which can cause phlegmon, causes obliteration of preserved lymphatic vessels and leads to rapid progression of the disease. With a prolonged course of the disease (especially in the case of secondary lymphedema), lymphangiosarcoma may develop.
The diagnosis is made by a phlebologist surgeon based on an external examination, measurement of the volume of the limbs and anamnesis data. Lymphangiography is used to assess the condition of lymphangions (small lymphatic vessels). If vessels shaped like beads or spindles are found on the lymphogram, we can talk about the safety of motor skills. If the vessel is evenly filled with a contrast agent, and its diameter is the same throughout, this indicates normal patency in case of damage to the contractile apparatus and impaired lymph transport. With primary lymphedema, hypoplasia of lymphangions is detected, with secondary – a change in the shape of blood vessels, extravasation, filling of the cutaneous lymphatic network.
Lymphoscintigraphy is used to study the dynamics of the disease. The nature of the isotope distribution makes it possible to assess the main, diffuse, collateral lymph flow and identify complete lymphostasis. In recent years, lymphangiography has been gradually replaced by new research methods. MRI, computed tomography and duplex scanning are increasingly used to assess lymph outflow and the state of the lymphatic system.
Lymphedema should be differentiated from edema caused by post-thrombophlebitis syndrome, kidney and heart diseases. If primary lymphedema is suspected, differential diagnosis with lipedema (syndrome of morbid obesity of the legs) is performed. Unlike lymphedema, lipedema is characterized by a symmetrical lesion of the lower legs. The feet with lipedema remain intact.
Conservative therapy is possible in the absence of organic changes in soft tissues. Patients are prescribed a special diet, physical therapy courses, balneological and physiotherapy procedures. To reduce the volume of the affected limb, the following are indicated: wearing a bandage, lymphatic drainage massage, variable hardware pneumocompression. Complex methods of treatment used in modern phlebology can slow down the development of lymphedema, and in some cases even achieve regression of the symptoms of the disease.
To create bypasses of lymph drainage, operations are performed on the lymphatic pathways. Lymphatic vessels are pre-stained. During the operation, the maximum number of vessels in the popliteal fossa or Scarpa triangle on the thigh is isolated. The vessels are crossed, after which their central ends coagulate. Then anastomoses are created between the peripheral ends of the vessels and the nearest branches of the subcutaneous veins. The operation performed in the early stages allows you to completely normalize the outflow of lymph. With a prolonged course of the disease after surgery, it is possible to achieve a significant reduction in swelling of the skin and subcutaneous tissue.
Prognosis and prevention
If lymphedema is detected at the initial stages, in most cases it is possible not only to stop the development of the disease, but also to eliminate existing problems, therefore, at the first signs of lymphedema, you should consult a doctor. Patients with impaired lymph outflow and an increased risk of lymphedema are advised not to create additional obstacles to lymph outflow (you should not carry a purse on a sick arm, cross your legs while sitting or measure blood pressure on the affected arm). It is necessary to keep the affected limb clean. It is not recommended to walk barefoot or cook food without protective gloves.
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