Lymphangioma is a collection of abnormal benign lymphatic vessels that form a mass that consists of cystic spaces with the size of the variance, which have the potential for local extension, so it can infiltrate surrounding structures. Lymphangioma, cystic hygroma, and limfangiomatosis can affect almost any area of the body where there is lymphatic tissue, but mostly in the head, neck and axilla.Approximately 5.6% of all benign lesions on infant and childhood, lymphangioma do not have a tendency to gender or race. Lymphangioma can also occur in the lungs, gastrointestinal tract, liver, spleen, and bone, and can be a manifestation of multifocal disease if it is found in the bones, liver, and spleen. Abdominal lymphangioma are rare.
Lymphangioma should not be confused with primary lymphoedema, a disorder in children and adolescents that causes swelling in the extremities. This condition is associated with some genetic syndromes and contrast with lymphangioma has the potential for malignant transformation in line with berjalannnya time.
Gross observed that 65% of lymphangioma apparent at birth, and that 90% appears at the end of the second year. The main mode of presentation is that of the mass that usually grows slowly. Rapid enlargement, sometimes overnight, can occur after systemic infection or bleeding into the tumor.
Etiology and Pathogenesis
Lymphangioma abnormality etiology is believed to be a defect in the development of the lymphatic pathway that evolved from the sixth week of pregnancy, and so on, so that the efferent duct is obstructed, or inadequate and dilatation of the proximal afferent vessels. The lymphatic system evolved from the five primitive sac derived from the venous system. Lymphatic development has been divided into five stages between the sixth week and the tenth week of pregnancy. Lymphangioma is traditionally classified into three groups: lymphangioma simplex consists of a small capillary channel size, cavernous lymphangioma consist of dilated lymphatic vessels that are often covered by a fibrous adventitia, and cystic lymphangioma or hygroma. Mikrokistik and makrokistik forms can coexist in different areas of the same lymphangioma.
Pathogenesis and radiologic appearance of lymphatic malformations can be explained by a embryology Lymphatic as proposed by Zadvinskis et al. The authors propose that lymphangioma descriptive classifications should not be based only on the size of the lymphatic vessels, but that these lesions should be considered as a spectrum of the same pathological process.
Thus, cystic hygroma occurs due to failure of the embryonic lymph sacs to re-establish communication with the venous system. These lesions can reach a large enough size because of the growth in the loose connective tissue. Lymphangioma with a smaller capillary and cavernous developed later than sequestration abnormal lymphatic bud mesenchyme, which have lost their connection with the primordia in the peripheral lymphatic branching.
Inhibited growth in dermal and Epidermal element, produces a form capillaries. Subcutaneous cavernous form occurs in muscle or glandular tissue. Because they are derived from branched tissues more towards more peripheral than the developing lymphatic cystic hygroma, so the size is smaller, and smaller lymphatic space. Limfangiohemangioma formed when the buds lymphatic distorted maintain a relationship with the venous system in which they arise and thus can not fully differentiate into lymphatic.
Because of the broad spectrum of abnormalities that occur in the lymphatic system and different presentations, the new classification is based on the presentation of disease have been proposed by Hilliard, McKendry and Phillips.
Clinical classification of congenital abnormalities in the lymphatic seems to provide a more useful guide for the diagnosis and management of problems and are summarized as follows: (i) mass, (ii) bone lesion, (iii) presentation of abnormal lymphatic function (eg, lymphedema, ascites, or pleural effusion ), (iv) presentation of the combination of abnormal lymphatic function, (v) related disorders (eg, lymphopenia, hypogammaglobulinemia), and (vi) the symptoms associated with angiomatosis mixture (blood and lymphatic vessels).