LYMPHATIC MALFORMATIONS

For over 100 years, doctors referred to these disorders as tumors. They often identified the disorders with the term "lymphangioma" and "cystic hygroma." Now, doctors believe that these conditions are not really tumors, but rather they are deformed tissues that result from abnormal embryonic development of the lymphatic vascular system. Thus, the term,"lymphatic malformation" (LM), is more appropriate.

Usually, LM's are usually seen in the first year of life, but they can appear later. In addition, modern advances in medical technology have made it possible to detect LM's in utero with the use of "ultra sonic" imaging devices. They present as lesions, are easily infected and result in soft tissue, as well as, skeletal disfigurement. This often results in secondary problems, as difficult, if not more difficult to treat than the primary malformation.

LM's can occur in the skin and in just about any portion of the body. The most common locations are in the face, neck, torso, groin and the extremities. They can also occur in the intestines, abdominal cavity and even in bones.

Presently, it is believed that LM's arise from a defect in the early stages of fetal development, sometime between the sixth and ninth week of gestation. Once the defect occurs, the abnormal lymphatics develop and expand during embryonic and fetal life up to birth. The lesions are of varying size and extent. They do not tend to grow by cellular proliferation and may be combined with capillary or venous (vascular) malformations. They are benign in nature and tend to infiltrate surrounding tissues, be they skeletal, muscular or dermal. In general, the lesions grow in proportion to the child.

At the present time, there is no cure for these malformations. However, although the treatment options are limited they can be managed some cases being more difficult to manage than others. The most prevalent treatment being surgical removal primarily when the lesions pose problems to physiological development. Please keep this foremost in mind: Surgery in infants and toddlers is discouraged except in extreme life threatening situations.

With respect to treatment of these lesions, it should be noted that they do not tend to undergo malignant degeneration. Diffuse lymphangiomas infiltrate surrounding tissues and rarely is it feasible to perform a total excision of the LM.

In some cases, where a well localized (encapsulated) lymphangioma occurs, it is possible to remove the lesion completely.

However, as mentioned above, often, surgical excision is incomplete and persistent swelling and difficulty with long term healing is the norm. Repeated surgical forays into regions withal become progressively more risky, as dent scarring distorts normal anatomic planes, placing nerves and blood vessels at increased risk in subsequent surgical interventions.

Familiarity with LM has improved significantly over the past 10 years, as it appears that there are more cases being reported.

Our organization of parents and supporters seeks to educate and improve the quality of life for those anguished with LM. They must face the world, a world too often concerned with and pre-occupied with physical appearances. Please join us in our efforts to offer them hope for the future.

Stay calm. You are not alone.
Call 877-570-2256 (972-377-4326 from outside the USA)