Lymphedema in Turner Syndrome: Diagnosis & Treatment

In part one of this blog, we covered the lymphatic system, what lymphedema is, and how to recognize early signs. In this blog, we will discuss how lymphedema is diagnosed, treatment options, and how to advocate for yourself or your child.

Information presented in this article comes from the webinar “Understanding Lymphedema.”

Diagnosis and Clinical Assessment

Lymphedema is diagnosed through clinical evaluation, where clinicians can perform a physical exam and review medical history.

Clinical assessment for lymphedema may include:

Evaluation of swelling and skin texture
Discussion of symptoms
Circumferential limb measurements at standard landmarks
Comparing left and right limbs
History of infection or cellulitis review

With lymphedema, cellulitis (a bacterial skin infection that causes redness, swelling, pain, and warmth) can be very common. For adults, clinicians may see recurrent cellulitis, indicating lymphedema. For children, it is important to watch for cellulitis as it may or may not indicate lymphedema, as an infection may be from an outside source.

A common finding with lymphedema is Stemmer sign, which is the inability to pinch the skin at the base of the finger or toe, at the knuckle. Although this sign can be important, it is not always needed for a lymphedema diagnosis.

Imaging

Indocyane Green (ICG) Fluoroscopy

ICG Fluoroscopy is a tool used to measure the peripheral lymphatic vessels, the vessels closest to the skin. ICG is injected in the web space of the hand or the feet. A specialized light is shined onto the skin while the lymphatic system starts to absorb the dye. This is a safe procedure as the dye is not radioactive. This can be done during manual lymphatic drainage (MLD).

MRL (Magnetic Resonance Lymphangiography)

MRL visualizes superficial and deep vessels of the lymphatic system. Patients are sedated and the radioactive dye can be harmful. This tool is not very accessible.

Lymphoscintigraphy

Lymphoscintigraphy is a nuclear medicine imaging technique that visualizes the lymphatic system using small amounts of radioactive materials. This procedure is usually done in adults with cancer and can be quite costly.

Imaging studies are useful but you should not wait to get imaging before starting treatment.

Treatment

The universal goals of care are optimizing soft tissue health, decreasing the risk of infection/cellulitis, increasing mobility, and fitting better into clothing to improve quality of life.

The gold standard treatment for lymphedema is Complete Decongestive Therapy (CDT). Before starting CDT, patients must be cleared by their physician to ensure the kidneys and heart are functioning well. A certified lymphedema therapist (CLT) is qualified to perform CDT.

Components of CDT

Manual Lymphatic Drainage (MLD)

MLD expedites the absorption and propulsion fluid from the limb.
A specific technique is required so it is important you consult your therapist.
MLD can be done while ICG Fluoroscopy to visualize fluid moving away from the limb.

Compression Bandaging

CLT’s apply a short stretch bandage around foam padding.
The foam is compressed to remove air.
As the limb shrinks, the foam expands.

Therapeutic Exercise

Exercise and movement is essential while in compression bandaging.
This improves lymphatic flow and the effectiveness of compression bandaging.

Skin Care

It is very important to keep the skin clean and healthy to reduce the likelihood of infection.

Long-Term Management

The goal is to keep the limb in a decongestive state.
Do not put swollen limbs in compression garments.
Shrink the limb first then place compression garments.
Work with your clinicians and make it fun for your child using toys or snacks.

Compression Bandaging Tips

Place bandaging distal to proximal (toes or fingers first, then up the limb).
Foam “fries” can be used over web space and in between fingers and toes.
For hands, coban can be put over bandaging to make it more durable. Kinesio tape should be placed between alternating fingers so the fingers do not get stuck together.
Ask your clinician to make videos for reference.

Advanced Air Pumps

These are used to mimic manual lymphatic drainage.
Some examples include Lymphapress and Tactile but note that neither make pediatric sizes.

Advocacy

Support is necessary in managing your lymphedema or your child’s lymphedema. If you feel your lymphedema needs management, do not wait for it to get worse or get an infection. Trust your instincts and find a CLT who will guide you through the process.

These websites can be used to find a CLT near you:

Affording Treatment

The Lymphedema Treatment Act states that Medicare is mandated to pay for 3 items per limb every 6 months. Most other insurances follow this. Lymphedema therapy is billed as a PT/OT visit and not a speciality visit. It is important that your CLT documents the diagnosis and functional limitations using correct CPT codes to help with coverage and approval.

Some examples of companies that make custom and off the shelf compression garments for adults include:

Luna
Compression Care
Sun Med
Verse Medical

Comfiwave makes pediatric garments off the shelf, including toe caps. There are not many pediatric size garments and custom garments are usually the way to go.

Research into lymphedema and Turner syndrome is crucial. With what we know now, Complete Decongestive Therapy (CDT) is evidence based and the gold standard for managing lymphedema. Early recognition and intervention is key to decreasing the chance of infection and progression of swelling. Trust yourself and find a CLT to guide you through your journey.

To learn more about Lymphedema & TS, watch the free webinar on our website.