Infection of the skin or soft tissues (cellulitis) may occur due to stagnant (non-moving) lymphatic fluid in the malformation or due to bacteria entering the body through open lymphatic vesicles. Cellulitis could potentially lead to a more serious or even life-threatening infection. Immediate treatment with antibiotics is essential to treat these skin and soft tissue infections. Bleeding into the area of the malformation may cause rapid enlargement of the malformation and pain. Bleeding may occur spontaneously or from trauma to the area. There may also be spontaneous leakage of lymphatic fluid from blebs on the skin. These lymphatic vesicles can also bleed. Chronic pain and/or cosmetic disfigurement may occur from the malformation itself or as a result of complications from the malformation.
Additional complications may be possible, depending upon the specific area of the body that is affected by the lesion:
- A lesion involving the trachea (windpipe), tongue or chest can cause difficulty breathing.
- A lesion in the gastrointestinal tract (stomach and intestines) can cause protein loss.
- A lesion in the thorax (chest) can cause chylothorax, which is a leakage of lymphatic fluid into the chest cavity. It can also cause heart and lung complications.
- A lesion in the abdomen can cause chylous ascites, which is a leakage of lymphatic fluid into the abdomen.
Although certain complications cannot be prevented, they can sometimes be diminished.
Developing an infection in a lymphatic malformation of the head/neck region is less likely if careful attention is paid to oral hygiene. Regular tooth brushing and visits to the dentist are recommended.
Diffuse Lymphatic Malformations
Generalized lymphatic anomaly (GLA) and Gorham-Stout disease (GSD) are rare diffuse lymphatic malformations that involve multiple body sites, most commonly the bones, chest, skin, spleen, liver and gastrointestinal tract. In both GLA and GSD, the lymphatic malformations involve the bones but appear differently on imaging. Gorham-Stout disease, also called “vanishing bone disease,” is characterized by LMs that cause destruction of the bony cortex (outermost layer of the bone) and progressive bone loss. In GLA, the lymphatic malformations appear “punched out” or as holes within the bone. These bony lesions may result in fractures.It is not uncommon that these conditions are not identified until after a bone fracture. If the spine is involved, there is a risk of neurological issues.
Patients with GLA and GSD have an increased risk of developing chylous effusions or leakage of lymphatic fluid into the lungs or abdomen or around the heart, particularly if the ribs are involved. In addition to the potential complications listed above, individuals with GLA and GSD may have other complications depending on locations of the malformation involvement.
Kaposiform lymphangiomatosis (KLA) is a rare disorder characterized by abnormal “kaposiform” lymphatic clusters in multiple body sites and is complicated by effusions, similar to GLA. However, KLA is more aggressive than GLA and is associated with coagulopathy (clotting and bleeding issues). Complications are directly related to the extent of the organ involvement and coagulopathy severity. Because of its aggressive nature and similarities to a vascular tumor called kaposiform hemangioendothelioma (KHE), it is unclear whether KLA is a lymphatic malformation or a vascular tumor.