Infection of the skin or soft tissues (cellulitis) may occur due to non-moving lymphatic fluid in the malformation. This may also be due to bacteria entering the body through open lymphatic blebs. Cellulitis could lead to a more serious or life-threatening infection. Immediate treatment with antibiotics is required to treat these skin and soft tissue infections. Bleeding into the area of the malformation may cause pain and growth of the malformation. This may occur from trauma to the area or without reason. The blebs on the skin may bleed or leak lymphatic fluid. Chronic pain and deformity may also occur.
Additional complications may be possible, such as:
- A lesion involving the windpipe, tongue or chest can cause difficulty breathing.
- A lesion in the stomach and intestines can cause protein loss.
- A lesion in the chest can cause chylothorax. This is a leakage of lymphatic fluid into the chest cavity and/or lungs. It can also cause heart and breathing complications.
- A lesion in the abdomen can cause chylous ascites. This is a leakage of lymphatic fluid into the abdomen.
Individuals with lymphatic malformations in the head and neck area, especially those involving the mouth, can get frequent and serious infections. This happens because of bacteria from the mouth get into the malformation. Good oral hygiene decreases these infections. Regular tooth brushing and visits to the dentist are recommended.
Diffuse Lymphatic Malformations
Generalized lymphatic anomaly (GLA) and Gorham-Stout disease (GSD) are complicated lymphatic malformations that involve multiple body sites. The areas most affected include: the bones, chest, skin, spleen, liver and gastrointestinal tract. In both GLA and GSD, the lymphatic malformations involve the bones but look different on imaging. Gorham-Stout disease, also called “vanishing bone disease”, is characterized by lymphatic malformations that cause destruction of the outermost layer of the bone (cortex) and bone loss. In GLA, the bones have “punched out” lesions or holes. These bony lesions may result in fractures in both GSD and GLA. These conditions are frequently 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 effusions, which occurs when lymphatic fluid leaks into the lungs or abdomen or around the heart. Individuals with GLA and GSD have other complications depending on locations of the malformation.
Kaposiform lymphangiomatosis (KLA) is rare disorder that is similar to GLA but is more aggressive and can be life-threatening. It is characterized by unusual “kaposiform” lymphatic clusters in multiple body sites. Individuals with KLA often have effusions, bone lesions and involvement of one or more organ. It is associated with coagulopathy (clotting and bleeding issues). Complications are related to the extent of the organ involvement and severity of the blood issues. It is unclear whether KLA is a lymphatic malformation or a vascular tumor.