Lymphedema

What is Lymphedema?

Lymphedema is a long-term health condition where excess lymphatic fluid builds up in the body, leading to swelling and changes in the skin and tissue. This happens when the amount of protein-rich fluid exceeds what the lymphatic system, which is the body’s drainage system, can handle. This swelling can happen anywhere, such as the arms, legs, face, neck, chest and even the oral cavity. It’s essential to understand that getting a diagnosis of lymphedema can lead to psychological, physical, and social consequences. Lymphedema is typically classified as primary, which is genetic, or secondary, which is acquired.

The lymphatic system, made up of vessels, carries lymph, a fluid containing white blood cells, fats, bacteria, cell waste, water, and protein. This fluid’s composition is similar to that of blood plasma. The system is quite complex, featuring lymph capillaries (the tiniest lymph vessels), pre-collectors, collectors, lymphatic trunks, and lymph nodes. We can categorize this system into superficial (lies just beneath the skin), which drains the skin and immediate under-the-skin areas, and deep (lies beneath a layer of connective tissue called fascia), which drains muscles, joints, tendons, and nerves. There are also connecting vessels between these systems, moving lymph fluid from deeper tissue layers to the surface.

If you have lymphedema, you can develop symptoms like swelling in the extremities (such as the arms, hands, legs, feet), and, less commonly, the breast, chest, shoulder, pelvic area, groin, genitals, and tissues inside the mouth. Additionally, you may experience limited movement in the joints because of the swelling and tissue changes, changes in skin color, pain and altered sensation, a feeling of heaviness in the limb, and challenges with clothing fit due to swelling.

What Causes Lymphedema?

Primary lymphedema is a condition that a person is born with or inherits due to abnormal development of the system in the body responsible for carrying lymph, a type of fluid. This is often the result of a genetic mutation. Primary lymphedema is split into three groups: 1) Congenital lymphedema, where symptoms are evident at birth or within the first two years of a child’s life; 2) lymphedema praecox, noticeable around puberty or during early adulthood; and 3) lymphedema tarda, which begins after the age of 35.

There is another kind of lymphedema known as secondary lymphedema. This happens when the lymph system gets damaged or blocked. Globally, the most common cause is a disease caused by filariasis, an infection by a type of worm known as Wuchereria bancrofti. However, in developed countries, secondary lymphedema is mostly due to the development of cancer, or its various forms of treatment. These treatments can include the surgical removal of lymph nodes, radiation therapy, or medications. Among all types of cancer, breast cancer is the one most commonly associated with secondary lymphedema in developed countries.

Risk Factors and Frequency for Lymphedema

Primary lymphedema is a rare disease, impacting about 1 in 100,000 people. However, secondary lymphedema is much more common, affecting around 1 in 1,000 Americans. Although lymphedema is pervasive, we don’t fully understand its prevalence because there are not enough well-designed studies on this topic. This means that there are likely more instances of lymphedema than reported.

Lymphedema is most commonly studied in patients with cancer. For example, 1 in 5 women who survive breast cancer will develop lymphedema. Additionally, those with head and neck cancer can develop issues with their lymph system and soft tissues within 18 months of treatment, with over 90% of these patients experiencing some type of lymphedema. Furthermore, more than half of these patients will develop a condition called fibrosis.

A recent study showed that 37% of women treated for gynecological cancer showed signs of lymphedema within a year of their treatment. Potential risk factors for lymphedema in women treated for gynecological cancer include extensive lymph node surgery, chemotherapy and radiation treatments, higher body mass index, low physical activity, a diagnosis of vulvar/vaginal cancer, and the presence of lymphedema before treatment.

Signs and Symptoms of Lymphedema

Primary and secondary lymphedema are conditions that cause swelling in various parts of the body due to lymph fluid buildup. The two conditions can often present similarly, which is why a detailed medical history and physical examination are musts. Primary lymphedema is a disorder you’re born with, while secondary lymphedema tends to result from issues like injury, illness, or blockages. The medical history should cover aspects like duration and distribution of swelling, infections, foreign travel, history of cancer, liver or heart disease, and any prior surgeries, especially those involving lymph node removal.

In the early stages, lymphedema can often be mistaken for regular swelling or edema. It’s typically characterized by an unusually swollen area that won’t reduce in size with elevation or through the use of diuretics (water pills). Early on, the skin may look indented when pressed, and elevation helps reduce swelling. However, as it gets worse, these signs stop being visible—the skin hardens and swelling doesn’t improve with elevation. Lymphedema can cause discomfort and emotional distress, and if it’s secondary, symptoms may only show years after the underlying cause.

Checking the family history is crucial if primary lymphedema is suspected. Evaluating if family members from earlier generations also had swollen feet, ankles, or legs from an “unknown cause” is important.
Possible triggers of lymphedema include cancer, injuries, or severe burns.
Signs and symptoms to look out for include swelling (edema), especially in limbs, skin thickening (hyperkeratosis), formation of small skin bubbles and bumps (lymphangiomas), and leaking of lymph fluid from skin (lymphorrhea).

During the physical exam, several signs are checked:

Skin: Looking for dryness, increased thickness, hyperkeratosis, lymphangiomas, unusually warm or dark skin, and any nodules. A cobblestone appearance of the skin (papillomatosis) might also be visible due to dilated and distended lymph vessels enveloped in fibrotic tissue.
Examining the extremity: If the limb size difference is less than 20%, it’s considered a mild or moderate case. More than 20% difference signifies a severe case.

A positive Stemmer’s sign, which is when it’s impossible to pinch a fold of skin at the root of the second toe, can further indicate lymphedema.

Testing for Lymphedema

Lymphedema is often mixed up with other conditions that cause swelling and enlargement of the limbs. Knowing the risk factors and signs of lymphedema can help doctors diagnose the condition accurately about 90% of the time. It’s essential to correctly identify lymphedema so that patients can get the right treatment. Doctors often suspect lymphedema by reviewing the patient’s medical history and conducting a physical examination. They then typically confirm the diagnosis using a specific test called lymphoscintigraphy.

There’s typically no need for blood, urine, or tissue tests to diagnose lymphedema. However, these tests might help determine the underlying causes of limb swelling when the reason is unclear. For example, if doctors think that kidney or liver issues might be causing the swelling, they would check liver function and blood urea nitrogen (or BUN)/creatinine levels, and analyze the patient’s urine. If cancer is suspected, tests for cancer markers may be performed. If an infection is possible, a complete blood count (or CBC) would be checked.

While imaging isn’t necessary to diagnose lymphedema, it can help confirm the diagnosis, determine the extent of the condition, and assist in deciding the best treatment approach.

Lymphoscintigraphy is a procedure where a small amount of radioactive protein-dye is injected into the web space between the first and second digits of the swollen limb. The limb is then scanned with a gamma camera to track the dye’s movement through the lymphatic system, which is a network of tissues and organs that help rid the body of toxins, waste, and other unwanted materials. Images revealing dye outside the lymphatic structures suggest lymphedema.

Some newer technologies include 3-dimensional magnetic resonance imaging (MRI), computerized tomography (CT), ultrasound and bioelectrical impedance analysis. Ultrasound can be used to rule out other causes of limb swelling, like deep vein thrombosis (DVT) or poor vein function, and it can also spot tissue changes and masses that might be compressing the lymphatic system. CT and MRI scans can detect swelling of soft tissues with good sensitivity and specificity, but they are relatively more expensive.

Treatment Options for Lymphedema

Lymphedema is a disease that worsens over time, leading to swelling in the body. Diagnosing and treating it early is very important to slow down its progression and prevent it from becoming a severe, long-term issue. Therefore, it’s important to diagnose and promptly treat this condition, even when it just starts appearing or seems mild. If you have this disease, it is crucial to see a specialist trained in the treatment and management of lymphedema. This specialist could be a doctor or a therapist who specializes in physical or occupational therapy.

Treatment for lymphedema involves different methods. Here’s a brief overview:

Decongestive Lymphedema Therapy (DLT): This is the main treatment for lymphedema that has become serious. It involves procedures to help lymph fluid circulation and soften hard, thick tissue.
Manual Lymph Drainage (MLD): This is a gentle massage technique that aims to improve the flow of lymph fluid.
Compression: This involves wrapping the affected area to encourage fluid drainage, but it must be done correctly as incorrect application can lead to skin irritation and increase the risk of infection.
Skincare: Since lymphedema predisposes the skin to infections, it is necessary to take good care of the skin to prevent secondary infections.
Exercise: Light exercise can help drain lymph and absorb proteins through muscle contraction.

Medication can also be used as part of the treatment plan, primarily to manage pain or treat secondary infections. However, in some severe cases, surgery might be necessary.

There are several surgical options for lymphedema:

Debulking, though it is often ineffective,
Microsurgical techniques such as Vascularized Lymph Node Transfer (VLNT) and Lymphaticovenous Anastomoses (LVA). These surgeries are designed to improve the natural drainage of lymph fluid and can even eliminate the need for compression garments in some patients. These procedures typically work better if the patient’s lymphatic system is not heavily damaged,
Suction-Assisted Protein Lipectomy (SAPL) is more effective in later stages of lymphedema, as it removes the solid and fatty deposits that are not typically treatable with standard lymphedema therapy or VLNT or LVA surgeries.

Treating lymphedema can be a multi-step process, but with the right treatment and specialist guidance, it can be managed effectively.

The following conditions can cause lower limb swelling:

Congestive heart failure
Glomerulonephritis – a type of kidney disease
Nephrotic syndrome – another kidney disorder
Hypoproteinemia – unusually low levels of protein in the blood
Drug reactions
Cirrhosis of the liver
Pretibial myxedema – skin condition often linked to thyroid problems
Constrictive pericarditis – a type of heart disease
Lower limb dependency syndrome
Lipedema – a fat distribution disorder
Bilateral chronic venous insufficiency – a condition affecting blood flow from the legs
Malignancy
DVT – short for deep vein thrombosis, a condition where blood clots form in deep veins
Malignant lymphedema – swelling related to cancer
Postoperative complications following surgery
Cellulitis – a skin infection
Baker cyst – a fluid-filled cyst that can cause knee swelling
Cyclical and idiopathic edema – unspecified swelling that comes and goes
Arthritis in children – it’s known to be associated with lower limb swelling, but the reason for this is still uncertain.

This information is intended to help better understand what might be the cause of lower limb swelling. Always consult a healthcare professional for accurate diagnosis.

What to expect with Lymphedema

Once lymphedema, a swelling caused by a blockage in the lymphatic system, develops, it’s often hard to fully cure. However, diligent treatment and preventative steps can help reduce symptoms, slow down or halt the disease’s progression, and avoid complications. It’s important to note that patients who have been dealing with chronic lymphedema for ten years have a 10% risk of developing a form of cancer known as lymphangiosarcoma.

Lymphangiosarcoma is a particularly aggressive tumor that usually requires drastic treatments such as the amputation of the affected limb. Moreover, it presents with a grim outlook as the five-year survival rate is less than 10%. Thus, proactive management of lymphedema is crucial to avoid such severe outcomes.

Possible Complications When Diagnosed with Lymphedema

Lymphedema can lead to several complications, including:

Recurrent cellulitis: which is frequent infection of the skin and tissues beneath it
Lymphangitis: inflammation of the lymphatic vessels
Superficial bacterial and fungal infections: infections of the skin caused by bacteria or fungi
Lymphangio-adenitis: inflammation of lymph vessels and glands
Deep vein thrombosis (DVT): a blood clot in a deep vein, usually in the leg
Severe functional impairment: significant limitation in doing routine activities
Psychosocial dysfunction: mental and social challenges caused by illness
Cosmetic embarrassment: feeling self-conscious about physical appearance
Amputation: surgical removal of a body part

There can also be complications after surgery, such as:

Partial wound separation: part of the surgical wound reopens
Seroma: a collection of fluid under the skin at the surgical site
Hematoma: a collection of blood outside of a blood vessel, essentially a bruise
Skin necrosis: skin tissue death due to insufficient blood supply

Preventing Lymphedema

Taking care of yourself entails several key steps, including:

– Practicing Self Manual Lymphatic Drainage (MLD), a therapy that involves using your hands to move and drain the lymph fluid in your body to decrease swelling.
– Precautions to prevent infections since they can worsen lymphedema.
– Regular exercise to maintain a healthy body weight and promote lymph circulation.
– Managing your weight to lessen stress on your lower body.
– Wearing compression garments to squeeze affected areas and reduce swelling.
– Avoiding blood draws, intravenous lines or shots in the swollen arm or leg.
– Avoiding any unnecessary pressure on the affected limb. For example, do not get blood pressure reading on this arm or leg.

Support is critical. You can find numerous support groups and resources on the National Lymphedema Network website, a dedicated platform which can provide a lot of helpful information on handling this health condition.

Frequently asked questions

Lymphedema is a long-term health condition where excess lymphatic fluid builds up in the body, leading to swelling and changes in the skin and tissue.

Lymphedema is much more common than primary lymphedema, affecting around 1 in 1,000 Americans.

Signs and symptoms of lymphedema include: - Swelling (edema), especially in the limbs - Skin thickening (hyperkeratosis) - Formation of small skin bubbles and bumps (lymphangiomas) - Leaking of lymph fluid from the skin (lymphorrhea) During a physical exam, the following signs are checked: - Skin dryness - Increased thickness of the skin - Hyperkeratosis - Lymphangiomas - Unusually warm or dark skin - Presence of nodules - Cobblestone appearance of the skin (papillomatosis) due to dilated and distended lymph vessels enveloped in fibrotic tissue Additionally, a positive Stemmer's sign, which is when it's impossible to pinch a fold of skin at the root of the second toe, can further indicate lymphedema.

Lymphedema can be acquired through various means such as genetic mutations, abnormal development of the lymph system, damage or blockage to the lymph system, cancer, injuries, severe burns, and certain infections like filariasis.

The doctor needs to rule out the following conditions when diagnosing Lymphedema: - Congestive heart failure - Glomerulonephritis - a type of kidney disease - Nephrotic syndrome - another kidney disorder - Hypoproteinemia - unusually low levels of protein in the blood - Drug reactions - Cirrhosis of the liver - Pretibial myxedema - skin condition often linked to thyroid problems - Constrictive pericarditis - a type of heart disease - Lower limb dependency syndrome - Lipedema - a fat distribution disorder - Bilateral chronic venous insufficiency - a condition affecting blood flow from the legs - Malignancy - DVT - short for deep vein thrombosis, a condition where blood clots form in deep veins - Malignant lymphedema - swelling related to cancer - Postoperative complications following surgery - Cellulitis - a skin infection - Baker cyst - a fluid-filled cyst that can cause knee swelling - Cyclical and idiopathic edema - unspecified swelling that comes and goes - Arthritis in children - it's known to be associated with lower limb swelling, but the reason for this is still uncertain.

The types of tests that may be needed for diagnosing lymphedema include: - Lymphoscintigraphy: This specific test is used to confirm the diagnosis of lymphedema. It involves injecting a small amount of radioactive protein-dye into the web space between the digits of the swollen limb and then scanning the limb with a gamma camera to track the dye's movement through the lymphatic system. - Blood tests: These tests may be ordered to determine the underlying causes of limb swelling when the reason is unclear. For example, liver function tests and blood urea nitrogen (BUN)/creatinine levels may be checked if kidney or liver issues are suspected. Additionally, tests for cancer markers may be performed if cancer is suspected, and a complete blood count (CBC) may be checked if an infection is possible. - Imaging tests: While not necessary for diagnosing lymphedema, imaging tests such as ultrasound, CT scans, and MRI scans can help confirm the diagnosis, determine the extent of the condition, and assist in deciding the best treatment approach. Ultrasound can be used to rule out other causes of limb swelling, while CT and MRI scans can detect swelling of soft tissues. It's important to note that lymphedema can often be diagnosed accurately based on the patient's medical history and a physical examination, with lymphoscintigraphy being the specific test used to confirm the diagnosis. Other tests may be ordered to determine underlying causes or rule out other conditions.

Lymphedema is treated through various methods. The main treatment is Decongestive Lymphedema Therapy (DLT), which helps with lymph fluid circulation and softening of tissue. Manual Lymph Drainage (MLD) is a gentle massage technique that improves lymph fluid flow. Compression is used to wrap the affected area and encourage fluid drainage, while skincare is important to prevent secondary infections. Light exercise helps drain lymph, and medication can be used for pain management or treating infections. In severe cases, surgery may be necessary, with options including debulking, Vascularized Lymph Node Transfer (VLNT), Lymphaticovenous Anastomoses (LVA), and Suction-Assisted Protein Lipectomy (SAPL). With the right treatment and specialist guidance, lymphedema can be effectively managed.

The side effects when treating Lymphedema can include: - Recurrent cellulitis: frequent infection of the skin and tissues beneath it - Lymphangitis: inflammation of the lymphatic vessels - Superficial bacterial and fungal infections: infections of the skin caused by bacteria or fungi - Lymphangio-adenitis: inflammation of lymph vessels and glands - Deep vein thrombosis (DVT): a blood clot in a deep vein, usually in the leg - Severe functional impairment: significant limitation in doing routine activities - Psychosocial dysfunction: mental and social challenges caused by illness - Cosmetic embarrassment: feeling self-conscious about physical appearance - Amputation: surgical removal of a body part Complications after surgery can include: - Partial wound separation: part of the surgical wound reopens - Seroma: a collection of fluid under the skin at the surgical site - Hematoma: a collection of blood outside of a blood vessel, essentially a bruise - Skin necrosis: skin tissue death due to insufficient blood supply

The prognosis for lymphedema can vary depending on the individual and the severity of the condition. While lymphedema is typically a chronic condition that cannot be fully cured, diligent treatment and preventative measures can help reduce symptoms, slow down or halt the progression of the disease, and avoid complications. However, it's important to note that patients who have been dealing with chronic lymphedema for ten years have a 10% risk of developing a form of cancer known as lymphangiosarcoma, which has a five-year survival rate of less than 10%.

A specialist trained in the treatment and management of lymphedema, such as a doctor or a therapist specializing in physical or occupational therapy.

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