Cystic Lymphatic Malformations factsheet
Introduction
The lymphatic system is a network of vessels and organs that helps the body:
- balance fluid levels
- fight infections
- remove waste.
Lymphatic vessels are found all over the body, including in the:
- organs
- muscles
- fat
- skin.
These vessels carry a clear fluid called lymph, which contains:
- white blood cells that fight infection
- water
- protein
- other substances like bacteria.
Cystic lymphatic malformations happen when the lymphatic vessels form sponge-like cysts filled with lymph in or under the skin.
Signs and symptoms
Cystic lymphatic malformations are present at birth and usually grow over time. They may not be noticeable until fluid builds up slowly or suddenly due to infection or bleeding.
On the skin, they may look like fluid-filled blisters or wart-like lumps. Under the skin, they can cause swelling and enlargement of body parts, including:
- neck
- ears
- lips
- tongue
- fingers
- limbs.
Cysts can be different sizes:
- macro-cysts – larger than 1 cm
- micro-cysts – smaller than 1 cm.
Other symptoms
Tiny blood vessels inside the cysts can break, leading to blood clots. This may cause:
- swelling
- bruising
- pain
- pressure on nearby body parts.
Cysts can also become infected, leading to:
- swelling
- pain
- general illness.
Symptoms depend on where the cystic lymphatic malformation is located:
- neck – swelling near the airway can cause feeding and breathing problems
- belly or chest – can affect nutrient absorption or cause fluid buildup around organs
- skin – small blisters may leak fluid, increasing the risk of infection.
Some people with cystic lymphatic malformations may also have differences in their capillaries, veins, or arteries.
Most cystic lymphatic malformations will grow larger if untreated.
Diagnosis
Doctors diagnose cystic lymphatic malformations using:
- MRI scan or doppler ultrasound – to confirm the diagnosis, check the size, and see if it affects nearby tissues or blood vessels.
- genetic testing – a small fluid sample may be taken to check for genetic changes.
Treatment
Treatment depends on the size, location, and type of cysts. Cystic lymphatic malformations cannot always be completely cured. Treatment focuses on reducing their size and managing symptoms.
Common treatment options include:
- Sclerotherapy – a doctor drains fluid from the cyst and injects medicine to shrink it. Since cysts can refill, multiple treatments may be needed.
- Medications – oral or topical medicines can help manage cysts on the skin or tongue.
- Surgery – some small, well-defined cysts can be removed or reduced with surgery. Surgery can be risky if cysts are near airways, blood vessels, or nerves.
Management
Long-Term Care
Cystic lymphatic malformations are a lifelong condition. Your child will need regular check-ups with their treatment team to monitor changes and adjust the treatment plan if needed.
Support for families
Managing long-term treatment can be challenging for families. Speak to your child’s treatment team about support, such as:
- seeing a social worker to help with stress or anxiety
- getting help with travel and accommodation related to appointments.
When to see your doctor
See a doctor as soon as possible if your child’s cystic lymphatic malformation:
- suddenly changes in size or appearance
- becomes red, hot, or swollen
- causes pain or other concerning symptoms.
