Lymphedema

Introduction and Facts

Lymphedema is swelling in various areas of the body that occurs when something affects the lymphatic system. The lymphatic system collects excess fluid, proteins, and toxins from the body's cells and tissues and returns them to the bloodstream.

The lymphatic system collects excess fluid, proteins, and toxins from the body's cells and tissues and returns them to the bloodstream. When the lymphatic system is not functioning properly, body fluids build up and begin to swell. Swelling usually occurs in the arms and legs, but can also affect other areas of the body. Lymphedema also increases the risk of developing infections that start in the skin.

Pathophysiology

There are two types of lymphedema, namely primary and secondary lymphedema. Primary lymphedema occurs due to a rare, inherited (congenital) condition that affects the development of the body's lymphatic system. Primary lymphedema is associated with dysplasia of the lymphatic system and may also coexist with other vascular disorders, including Klippel-Trenaunay-Weber syndrome, and Turner syndrome. Primary lymphedema is characterized by hyperplasia, hypoplasia, or aplasia of the lymphatic vessels.

Secondary lymphedema develops due to damage or dysfunction of the normal lymphatic system. Filariasis is the most common cause of secondary lymphedema globally. Filariasis is a direct infection of the lymph nodes by the parasite Wuchereria bancrofti. The spread of parasites via mosquitoes affects millions of people in tropical and subtropical regions of Asia, Africa, the West Pacific, and Central and South America.

Apart from this, oncological surgical procedures such as lymph node biopsy and dissection that require excision of lymph nodes or regional blood vessels can lead to the development of secondary lymphedema. Other surgical procedures associated with the development of secondary lymphedema include peripheral vascular surgery, excision of burn scars, vein stripping, and lipectomy.

Non-surgical causes of lymphedema include recurrent tumors or malignancies that have metastasized to the lymph nodes, obstructive lesions in the lymphatic system, infected and/or traumatized lymphatic vessels, scar tissue that obliterates the lumen of the lymphatic vessels. Edema due to deep vein thrombosis (DVT) or non-obstructive causes of chronic venous insufficiency in the extremities can cause secondary lymphedema.

Signs and Symptoms

The most common symptom of lymphedema is swelling. Swelling can develop slowly or suddenly. Other symptoms of lymphedema include:

• Cannot see or feel blood vessels or tendons in the hands and feet
• Arms or legs appear to be different sizes
• Joints feel very stiff or inflexible
• The skin appears swollen or red
• Swelling in the arms or legs or other areas of the body
• Your arms, legs, or other parts of your body feel heavy or full and uncomfortable
• There is a burning or itching sensation
• The skin becomes noticeably thicker

Diagnosis

Diagnosis is based on a thorough history and physical examination. It is very important to perform these examinations to differentiate primary and secondary lymphedema because they are very similar. Primary lymphedema is congenital, while secondary lymphedema occurs due to injury or blockage.

Lymphedema can be divided into several stages, namely:

• Stage 0: The affected area may feel swollen, tight, and heavy but without visible signs of swelling
• Stage 1: There is swelling that occasionally appears and then disappears when the leg is elevated
• Stage 2: The affected area is almost always swollen and the skin in the area may feel tighter than the surrounding area
• Stage 3: The affected area experiences significant swelling along with changes in the skin such as changes in color and texture.

After carrying out an history and physical examination, several tests that support the diagnosis of lymphedema can be performed, such as:

• Doppler Ultrasound (USG): This examination is carried out by paying attention to blood flow by reflecting high frequency sound waves (USG) from red blood cells. This Al helps find obstruction and rule out other possible causes of swelling, such as blood clots.
• Magnetic Resonance Imaging (MRI): This examination uses magnets, radio waves, and a computer to create a series of detailed 3D (three-dimensional) images inside the body. This is done to see if there is another disease, such as a tumor, that is putting pressure on the lymphatic system.
• Computed tomography (CT) scan: This examination uses X-rays to show detailed images of body structures. Like MRI, this examination is performed to check whether there are other causes of obstruction that are pressing on the lymphatic system.

Management dan Treatment

Lymphedema treatment aims to relieve symptoms and reduce swelling. Lymphedema treatment varies depending on the cause and stage. Treatment includes physical therapy or other medications to keep lymphatic fluid flowing and reduce swelling and pain. Apart from that, symptomatic medication such as pain relievers or antibiotics can also be given.

Some physical therapies for lymphedema are:

• Compression garments are special stockings that function to compress the arms or legs so that lymphatic fluid can flow
• Light exercise to stretch problematic muscles and help reduce accumulated lymphatic fluid
• Elevation by positioning the affected arm or leg higher than the heart when lying down to relieve pain or symptoms
• Manual lymphatic drainage, which is a manual massage technique carried out by medical personnel to improve the flow of the lymphatic system
• Pneumatic compression is a device that is wrapped around the arms and legs to periodically pump and apply pressure so that lymphatic fluid can flow smoothly.

If after carrying out the steps above, the condition does not improve or gets worse, it will be recommended to carry out surgery to remove excess fluid or to remove tissue. Several types of surgery that can be considered for lymphedema that does not improve or gets worse include:

• Lymphatic bypass procedure: This surgery involves connecting and rerouting the lymphatic vessels and veins, thereby avoiding blockage and allowing lymphatic fluid to flow into the venous system.
• Lymph node transfer: Surgeons replace damaged lymphatic nodes with healthy lymphatic nodes taken from another area of ​​the body, essentially creating a new lymphatic system for the area of ​​the body affected by lymphedema.
• Debulking: This surgery treats very severe forms of lymphedema. This procedure involves surgery to remove all the skin, fat and tissue in the affected area of ​​the body, then placing a skin graft in the area.

References:

1. Kementerian Kesehatan RI. Limfedema [Internet]. 2022. Available from: https://yankes.kemkes.go.id/view_artikel/1461/limfedema
2. Sleigh BC, Manna B. Lymphedema. National Library of Medicine [Internet]. 2023. Available from:https://www.ncbi.nlm.nih.gov/books/NBK537239/
3. Cleveland Clinic. Lymphedema [Internet]. 2022. Available from: https://my.clevelandclinic.org/health/diseases/8353-lymphedema