Lymphangioma( or lymphatic malformation) is one of the relatively rare( not more than 10% of the total) congenital benign formations in children that occur with equal frequency in both boys and girls.
Being congenital, lymphangioma is already present in the baby's body, although it is not always possible to detect it in the course of intrauterine development, nor in the first months of its life. Scientific studies have data that the beginning of the formation of a benign tumor belongs to the second month of embryo development.
In the overwhelming majority of cases, the disease in the baby is revealed by the end of the first year of life( sometimes the diagnosis becomes evident only three years after birth).
What is lymphangioma?
Benign neoplasm, called lymphangioma, is nothing more than a defect in the intrauterine development of enlarged and improperly located lymphatic vessels.
The basis for the classification of lymphangiomas is their appearance, structural features and the nature of the clinical picture.
There is a classification based on the size of the lesions. According to her, lymphangiomas are microcystic( not exceeding 5 cm) and macro-cystic( more than 5 cm).
Capillary( sometimes called simple) lymphangiomas have the appearance of congenital pale stained large and small nodules with a glassy surface. Skin covers affected by the tumor, very similar to the skin of the orange.
Photo of capillary form of lymphangioma in children
Such uneven grain of the skin is caused by the accumulation of lymphatic capillaries filled with lymph. Along with the vessels, a number of fairly large cavities containing a certain amount of lymph enter the structure of capillary lymphangiomas. Their thin walls from the inside are lined with a layer of endothelium.
Capillary lymphangiomas are usually soft, easily contracting when pressed. However, over time, the formation of connective tissues makes them sufficiently dense and not so pliable.
Cystic lymphangiomas in children can consist of either one or several chambers( cysts), the sizes of which vary from a few millimeters to several tens of centimeters. Cameras filled with lymphatic fluid may not communicate with each other.
Among tumors of the lymphangiomas, neoplasms of the cavernous type are the most common. A feature of their structure is the presence of several cavities. The lymphatic fluid fills each of them very unevenly: some remain virtually empty, others are overflowing.
The base of the cavities is spongy, connective tissue, consisting of an elastic framework, small lymph vessels and bundles of smooth muscle fibers. Usually cavernous lymphangiomas have clear boundaries. If the tissues of a cavernous neoplasm differ diffusively( occurring by multiple cell division), their edges appear to be blurred.
When you press a cavernous new growth, it compresses. After cessation of compression, the lymph again fills the abandoned cavity.
The location of lymphatic malformations is noted at the sites of regional lymph nodes and is often associated with the type of tumor.
- The preferred locations for localization of cavernous lymphangiomas are: subcutaneous tissue, neck, deep cheek layers, tongue and lips .Most often, the size of the organs affected by the tumor multiplies many times.
- Cystic lymphangiomas are usually located in the armpits, on the neck, on the chest and abdominal wall of the .
Lymphangiomas are much less common in the retroperitoneal space, in the popliteal fossa, in the groin area, in the root of the mesentery of the small intestine and in the mediastinum. Very rarely, lymphangiomas affect the spleen, liver and kidneys.
Lymphangiomas are primary( congenital) and secondary.
- The reason for the formation of congenital lymphangiomas is a defect in the connection of the blood and lymph vessels, which occurs even during the fetal development stage.
- Secondary lymphangiomas that develop after infectious diseases( such as erysipelas) are a manifestation of lymphostasis.
- One of the characteristic symptoms of lymphangiomas is their slow growth. Increase neoplasm usually occurs as the child grows or slightly ahead of him. In rare cases, lymphatic malformation begins to increase rapidly in size immediately after the infant appears on the light. Sometimes the tumor behaves unpredictably: remaining unchanged for many years, it suddenly begins to grow rapidly. This pathology is typical for the combination of defects in the lymphatic and circulatory systems.
Clinical manifestations of lymphangiomas depend on their type.
- With capillary lymphangioma , the affected areas of the skin are hilly seals with indistinct edges, sometimes having the appearance of dense small nodules. Depth of capillary lymphangiomas in soft tissues can be different. Some neoplasms are located in the uppermost layers of the skin with a slight deepening into the subcutaneous tissue, others are located so deep that they germinate into the muscle fibers. The color of the skin over the tumor can be unchanged, often including areas with a purple or cyanotic color. Cyanotic inclusions in case of skin damage can cause bleeding. Capillary lymphangiomas can be accompanied by lymphatic drainage - the flow of lymph fluid as a result of spontaneous rupture of enlarged lymphatic vessels.
- Distinctive features of cavernous lymphangioma is a soft consistency, absence of clear boundaries and considerable swelling. When you feel the tumor, there is often a fluctuation( fluctuation) of its contents. Under the pressure of the fingers, cavernous lymphangioma contracts, after loosening the grip the lymph is slowly filled again.
- A clear symptom of cystic lymphangioma is the presence of a clear fluctuation. Cystic neoplasm is elastic. Skin over it remain unchanged, but much thinner and stretched. The walls of adjacent cysts are characterized by unevenness: this is felt during palpation. Cystic tumors increase slowly, but often there is a squeezing of a number of located nerves, vessels and internal organs( including the esophagus and trachea).
Methods of diagnosis
Diagnosis of benign tumors is made by non-invasive( safe, gentle) methods:
- ultrasound( ultrasound);
- magnetic resonance imaging( MRI);
- computed tomography( CT);
- X-ray diffraphraphy. This method allows us to clarify the anatomical features of the location of tumors.
What is the danger of lymphangioma?
- The greatest danger is represented by lymphangiomas located on the neck, in the mediastinum and in the abdominal cavity of , since their growth can lead to compression of the trachea and esophagus, which is inevitably accompanied by a violation of breathing and difficulty swallowing. For newborn infants with too narrow airways, this poses a direct threat to life.
- The cause of breathing problems can also be inflammation of the lymphangioma of the tongue.
- The contents of lymphangioma can become inflamed and lead to a rapid purulent intoxication of the child's body. The duration of the inflammatory process accompanied by high fever, significant increase and soreness of the tumor and lymph nodes is not less than two weeks.
Treatment in adults and children
- Lymphangiomas are usually treated by surgery. During the operation, a radical removal of the entire neoplasm or a significant part of it is performed. Indication for urgent surgery is the rapid growth of the tumor, the location of lymphangioma, which poses a direct threat to the life of a sick person and a significant deterioration in the quality of life associated with the presence of lymphangioma. Most often, in surgical intervention, a benign neoplasm is excised to the boundaries of healthy tissues. If the lymphangioma is not fully excised, the remaining sections are stitched with silk or capron threads.
- To prevent recurrence of excised lymphangioma , the procedure of electrocoagulation ( cauterization of non-excised areas by high-frequency currents) helps.
- For emergency treatment of newborns born with large lymphangioma on the neck, preventing them from breathing fully, sucking and swallowing, sometimes uses the method of sucking lymphangioma contents by performing a puncture and removing excess fluid from the outside of the .With the help of this measure, which helps to prepare a baby for surgery, a temporary improvement in his condition is achieved.
- If emergency indications for surgery are not available, treatment of small lesions( located on the lips, near the nose or near the ear) is performed using sclerosing therapy .Sclerotherapy is also used for the treatment of excised tumors.
- Inflammatory lymphangiomas are treated in the same way as any purulent processes. Having opened the tumor, drain it. Cases of significant reduction, as well as complete disappearance of lymphangiomas after the completed inflammatory process, are described.
Traditional healers for the treatment of lymphangia recommend to apply:
- Juice of walnut, reached the phase of milk ripeness. A teaspoon of the drug is bred in 50 ml of boiled water and taken three to four times a day.
- Collection "Monastic", prepared from sixteen herbs.
- An "anticancer" collection containing 25 components.
The above mentioned means can be purchased through the Internet.
This video shows the removal of lymphangioma of the small intestine by laparoscopy: