Lymphangioma Research Paper

Lymphangiomatosis is a disease in which many tumors or cysts grow in the lymph system of the body. In medical terminology lymphangiomatosis means: lymphatic system (lymph) vessel (angi) tumor or cyst (oma) condition (tosis). The tumors are not cancerous, but they invade the body tissues and cause a variety of symptoms. The tumors may grow in the bones, connective tissue, and organs of the body, and they may block, compress, or destroy tissues. They can spread to any area, but are more commonly found in the lungs, bones, kidney, the thoracic cavity, connective tissue and spleen. Over time, one or more bones may be replaced by lymphangioma tissue, which is known as Gorham’s disease. Lymphangiomas do not grow in the brain because the lymph system
If they are growing in the lungs, they can cause chronic cough, wheezing, shortness of breath, and collection of fluid around the lungs. In the heart, they cause rapid heartbeat and chest pain. When growing in the stomach or intestines, abdominal pain, nausea, vomiting, diarrhea may occur. Tumors in the kidneys cause pain and increased blood pressure. In the bones, the tumors cause pain, weakening of the bone leading to fracture, and pinched nerves in the spine. (Sweson, 2012)

Because lymphangiomatosis is rare and difficult to diagnose correctly, it is not known exactly how many people are affected by it throughout the world. There are no other known causes of lymphangiomatosis, although much about the disorder is not
The symptoms, clinical presentation, and typical radiographic findings can assist in the diagnosis. A biopsy is the major method for diagnosis, but is not always possible. Whole body magnetic resonance imaging has good capability for evaluating the extent of disease . MRI has been found to give better results than CT scans, but CT is commonly used too. Lymphangiomatosis can be confused with other diseases, including lymphatic displasia, lymphangiectasis, and the similar-sounding lymphangioleiomyomatosis (LAM). (Weiss, 2008)

Sometimes it is possible to remove the abnormal lymphatic tissue through surgery. However, most cases are inoperable. Most treatment is therefore aimed at relieving symptoms or aimed at slowing the progress of the disease. Some treatments include;

◦drainage of the spaces around the lungs and around the heart to remove fluid from these areas (pleural and pericardial drainage)

◦pleurodesis-a procedure to restrict the space around the lung where fluid can collect

◦a restricted diet that avoids fats except for medium chain