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What Pediatricians Need to Know About Lymphedema in Pediatrics

What Pediatricians Need to Know About Lymphedema in Pediatrics 150 150 Abbie Miller

Lymphedema is chronic, progressive swelling caused by maldevelopment or disruption of the lymphatic system. Typically, patients present with an asymmetric swelling of an arm or leg that progressively worsens. Min-Jeong Cho, MD, a microsurgeon and assistant professor at The Ohio State University and Nationwide Children’s Hospital, says that lymphedema affects more children and teens than you might expect. In the Q&A below, she answers some common questions to help you identify and treat patients with lymphedema.

 

Q: What causes lymphedema in children and teens?

Dr. Cho: There are two types of lymphedema: primary and secondary lymphedema. Secondary lymphedema occurs due to disruption of the lymphatic system by an external cause such as trauma or surgery. It is the most common type of lymphedema and often affects people who have had cancer surgeries where lymph nodes were removed or sustained damages to lymphatic vessels.

Primary lymphedema is a congenital condition, and it can typically shows up during the teen years. Patients will often present at a primary care office with unilateral swelling of an extremity that progressively worsens and complaints of heaviness, aching, difficulty of doing activities, and enlarged extremity. These patients typically see many specialists due to difficulty with finding a specialist in lymphedema.

Q: How is lymphedema diagnosed?

Dr. Cho: It is critical to recognize patients with lymphedema and refer them to a specialist in a timely manner. The key clinical characteristics of lymphedema include unilateral limb swelling, heaviness, paresthesia, pain, aching, skin thickening, and a history of cellulitis. Patients who are suspected of having lymphedema should obtain lymphoscintigraphy.

In lymphoscintigraphy, a special dye is injected into the affected tissue to evaluate the status of the lymphatic system.

Another important tool for diagnosing and developing a treatment plan for lymphedema is ICG fluorescence mapping. ICG is a dye that is injected into the affected area. The movement of the lymph is then observed under fluorescence. This allows us to see what channels may be blocked or missing.

Q: How can you treat lymphedema?

Dr. Cho: Not that long ago, liposuction and compression were the only options for lymphedema treatment. But as micro- and supermicro-surgeries have advanced, we’re able to treat it earlier, restore lymphatic system, and prevent its progression.

While microsurgery refers to surgeries on vessels smaller than 2 mm, supermicrosurgeries involve vessels smaller than 1 mm.

Using a specialized microscope that magnifies my vision 40x, I use a suture that is thinner than a hair to perform lympho-venus bypass (LVB) surgeries to restore lymphatic flow and bypass the areas of lymphatic. If the lymphedema has progressed so far that LVB is not possible, we can still perform physiologic surgery such as vascularized lymph node transfer or debulking procedure such as liposuction to remove the accumulated fatty tissue.

Q: Can lymphedema be prevented?

Dr. Cho: In patients who are at high risk of developing lymphedema, I perform prophylactic lymphovenous bypass procedures. In patients who will undergo node dissection, there’s a 40 to 60% chance that the child will develop lymphedema. In these cases, we may opt for prophylactic LVB. This is an approach we commonly take for breast cancer patients at the Ohio State, but it can be applied to pediatric patients who are having surgeries were the lymph nodes may be removed or affected.

About the author

Abbie (Roth) Miller, MWC, is a passionate communicator of science. As the manager, medical and science content, at Nationwide Children’s Hospital, she shares stories about innovative research and discovery with audiences ranging from parents to preeminent researchers and leaders. Before coming to Nationwide Children’s, Abbie used her communication skills to engage audiences with a wide variety of science topics. She is a Medical Writer Certified®, credentialed by the American Medical Writers Association.