There is currently no cure for isolated lymphatic malformations (LMs). Treatment focuses on reducing symptoms, preventing complications, and improving quality of life.
The treatment approach depends on the size, type (macrocystic, microcystic, or mixed), location, and severity of the malformation, as well as how it affects daily function.
Many individuals benefit from care coordinated through a vascular anomalies center, often involving an interdisciplinary team.
Observation (“Watch and Wait”)
For some isolated LMs, regular monitoring with clinical exams and imaging may be recommended. Treatment can be started if the condition changes or causes new symptoms, such as pain, infection, bleeding, or functional problems.
Sclerotherapy
Sclerotherapy is a minimally invasive procedure in which fluid is drained from a lymphatic cyst and a medication (called a sclerosant) is injected to irritate the cyst lining. This causes the cyst to shrink or close. This is most effective for macrocystic LMs. Most commonly requires multiple treatments and not everyone responds.
Surgery
Surgery (sometimes called debulking) may be used to reduce the size of lymphatic malformations and relieve symptoms such as pressure on the airway or organs, pain, difficulty breathing, or repeated infections. Because LMs often involve multiple tissue layers and may surround important structures—such as nerves, blood vessels, or organs—surgery is not always recommended or possible. Even when surgery is performed, some abnormal lymphatic tissue often remains, and regrowth often occurs over time.
Laser Therapy
Laser therapy or radiofrequency ablation may be helpful for selected microcystic lymphatic malformations, particularly when superficial skin blebs are present, bleeding or leakage occurs frequently, or surface symptoms affect comfort or hygiene. These treatments are generally aimed at improving symptoms and managing surface-related issues rather than eliminating deeper lymphatic malformations.
Medication
Topical sirolimus may help reduce bleeding or leakage from superficial skin lesions. Oral sirolimus may be used when a lymphatic malformation is diffuse, causing significant symptoms, or not responding to procedural treatments. Alpelisib targets the PI3K protein, which is overactive in people with PIK3CA gene mutations. It may be used in selected patients to reduce abnormal tissue growth and related complications. Decisions about medication are individualized and guided by a specialist based on the specific presentation.
Important note:
This is not a complete list of all possible medications. Treatment plans are individualized, and other medications may be used based on diagnosis, symptoms, overall health, and response to previous therapies.
Supportive Care
Other treatments and supportive strategies that may be helpful include:
- Compression garments (sleeves, stockings, or bandages) to help manage swelling
- Manual lymphatic drainage therapy to support lymphatic flow
- Clinical trials, which may offer access to new and emerging therapies
