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Lymphatic malformations (LMs) result from improper formation of lymphatic vessels. The goal of the lymphatic vessels is to recover the body's tissue fluid — which may pass through lymph nodes — and to return it to the bloodstream. When the vessels do not properly form, the transfer slows or stops, and masses (malformations) may develop as a result of fluid accumulation. The masses may resemble sponge-like solid tissue (microcystic) or include cystic spaces of variable size (macrocystic). Lymphatic malformations can also result in abnormal accumulation of lymphatic fluid in body cavities (pleural effusions in the chest, ascites in the abdomen).
It is believed that lymphatic malformations form during embryonic or fetal development. Nothing a pregnant mother does is known to cause or prevent LM formation.
Lymphatic malformations can appear anywhere in the body, and may resemble tumors, overgrowth or swelling of the affected area. LMs also may occur in internal organs. Depending on the location of the malformation, it may cause enlargement of the involved body part, potentially causing pressure on other anatomic structures (organs or body parts).
LMs may become discolored and swell suddenly, due to internal bleeding, after minor trauma that would not normally cause a bruise. LMs are also prone to infection, sometimes from minor skin breaks and sometimes arising without clear cause. Depending on the area and extent of the body involved by the LM and the age of the patient, bleeding or infection can cause a range of symptoms. Some of these may be monitored outside the hospital and others may require hospitalization.
Some lymphatic malformations are detectable on antenatal ultrasound and, as a result, may be diagnosed in utero. In other cases, a physician experienced with vascular anomalies may be able to diagnose lymphatic malformations based on a patient's medical history and a physical exam. An imaging study may provide additional information about the type and extent of the malformation.
Treatment for lymphatic malformations is determined by the anatomic location, microcystic or macrocystic type, extent, and involvement of neighboring normal tissues. Thus, treatment is highly individualized. Conservative management may be appropriate for some patients and is aimed at reducing swelling, preserving normal activities and body functions, and preventing infections.
Interventional therapy may include:
The vascular anomalies team will review all the data available and recommend the best treatment option for your child's lymphatic malformation.
The vascular anomalies program at Comer Children's offers an integrated and comprehensive approach to the diagnosis, care and management of vascular malformations. Our multidisciplinary team of pediatric experts works together to educate families and to evaluate and treat children with all types of these vascular lesions.