What is Hereditary Lymphedema?

Often, we discuss lymphedema as a problem that occurs secondary to medical treatment that removes or alters lymph nodes. However, secondary lymphedema is only one type. Some people experience what is called primary lymphedema. This is also known as hereditary lymphedema, symptoms that occur unrelated to another health problem. Here, we discuss what hereditary (primary) lymphedema is and how it may be treated.

Lymphedema, in general, is a condition in which certain parts of the body develop significant swelling. This swelling relates to an accumulation of lymphatic fluid subcutaneous tissue. In a healthy lymphatic process, the protein-rich lymph fluid and blood cells travel through a network of nodes, ducts, and vessels throughout the body. Primary lymphedema occurs when various lymphatic vessels are underdeveloped, malformed, or obstructed.

There are three types of hereditary lymphedema. Each may be treated in similar ways. At this time, there is no medication that can prevent or decrease lymph accumulation and swelling. Patients with hereditary lymphedema may initially be encouraged to practice reduction strategies in an effort to manage swelling and decrease complications such as infection in subcutaneous tissue. These include exercise, wearing well-fitting compression garments, and taking excellent care of the skin in the affected extremity. Conditioning exercises may also be beneficial.

In some cases, lymphatic backup requires medical intervention. Some practitioners administer manual lymphatic massage, but this may not provide sufficient or long-lasting relief. In such cases, microsurgery may be performed to create new pathways through which lymph can circulate. The technically meticulous procedure locates obstructed lymph vessels and attaches them to blood vessels of the same size. Able to flow more readily through these new channels, lymph does not back up as quickly as it would otherwise.

Lymphedema reconstruction has demonstrated outstanding outcomes in patients who have previously been told there was no way to treat their swelling. The procedure can quickly reduce swelling for many patients. However, it is not a cure for lymphedema. Patients are advised to continue managing their condition with strategies that prevent unnecessary fluid retention, such as limiting salt intake and avoiding certain medications.

There is hope for those who struggle with hereditary lymphedema. To learn more about treatment options, schedule a consultation with Dr. Patel in our friendly, professional office.

Posted in: Lymphedema Surgery

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