Lymphedema is a chronic disease marked by the increased collection of lymphatic fluid in the body, causing swelling, which can lead to skin and tissue changes. The chronic, progressive accumulation of protein-rich fluid within the interstitium and the fibro-adipose tissue exceeds the capacity of the lymphatic system to transport the fluid. Swelling associated with lymphedema can occur anywhere in the body, including the arms, legs, genitals, face, neck, chest wall, and oral cavity. There are many psychological, physical, and social sequelae related to a diagnosis of lymphedema. This activity reviews the presentation of lymphedema and highlights the role of the interprofessional team in its management.
Lymphedema refers to tissue swelling caused by an accumulation of protein-rich fluid that’s usually drained through the body’s lymphatic system. It most commonly affects the arms or legs, but can also occur in the chest wall, abdomen, neck and genitals.
Lymphedema Stages
Stage 0 (Latency stage)
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The patient is considered “at-risk” for lymphedema development due to injury to the lymphatic vessels but does not present with outward signs of edema.
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Includes patients with breast cancer who have undergone sentinel lymph node biopsy and or radiation but have not yet developed swelling.
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Lymphatic transport capacity has been reduced, which predisposes the patient to lymphatic overload and resultant edema.
Stage 1 (Spontaneous)
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Reversible
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Has pitting edema
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Swelling at this stage is soft and may respond to elevation
Stage 2 (Spontaneously irreversible)
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Has tissue fibrosis/induration
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Swelling does not respond to elevation
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Skin and tissue thickening occurs as the limb volume increases
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Pitting may be present but may be difficult to assess due to tissue and or skin fibrosis
Stage 3 (Lymphostatic elephantiasis)
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Show the following:
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Pitting edema
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Fibrosis
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Skin changes
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During this stage, papillomas may form, infections/cellulitis may occur, and the skin becomes dry
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The Stemmer sign may not be present in Stages 1 or 2.
Types of Lymphedema:
- Primary Lymphedema: This occurs when there’s a problem with the lymphatic system that’s present from birth.
- Secondary Lymphedema: This happens when something damages the lymphatic system later in life, such as surgery, infection, or radiation therapy.
Pathophysiology
Primary lymphedema is associated with dysplasia of the lymphatic system and can also develop with conditions of other vascular abnormalities, including Klippel-Trenaunay-Weber syndrome,[rx], and Turner syndrome.[rx] Primary lymphedema is marked by hyperplasia, hypoplasia, or aplasia of the lymphatic vessels.
Secondary lymphedema develops due to damage or dysfunction of the normally functioning lymphatic system. Although cancer treatments, including oncologic surgical procedures such as axillary lymph node dissection and excision in breast cancer and radiation treatment, are the most common cause of lymphedema in the United States, filariasis is the most common cause of secondary lymphedema globally.[rx] Filariasis is the direct infestation of lymph nodes by the parasite, Wuchereria bancrofti. The spread of the parasite by mosquitos affects millions of people in the tropic and subtropic regions of Asia, Africa, the Western Pacific, and Central and South America.
Oncologic surgical procedures such as sentinel lymph node biopsy and radical dissection that require excision of regional lymph nodes or vessels can lead to the development of secondary lymphedema. Other surgical procedures linked to secondary lymphedema development include peripheral vascular surgery, burn scar excision, vein stripping, and lipectomy.
Nonsurgical causes of lymphedema include recurrent tumors or malignancy that have metastasized to the lymph nodes; obstructive lesions within the lymphatic system; infected and/or traumatized lymphatic vessels; scar tissue obliterating the lumen of the lymphatic vessels. Edema from deep venous thrombosis (DVT) or nonobstructive causes of chronic venous insufficiency at the extremities may lead to secondary lymphedema.
Although there is no definitive cure for lymphedema, with proper diagnosis and management, its progression and potential complications can successfully be managed.[rx]
Causes of Lymphedema:
- Surgery: Especially when lymph nodes are removed or damaged during surgery, like in cancer treatment.
- Radiation Therapy: Used to treat cancer, but it can also damage the lymphatic system.
- Infection: Infections like cellulitis can block lymphatic vessels.
- Injury: Trauma to the lymphatic system can cause swelling.
- Obesity: Excess weight can put pressure on lymphatic vessels.
- Genetics: Some people may inherit a predisposition to lymphedema.
- Filariasis: A parasitic infection transmitted by mosquitoes, common in tropical regions.
- Deep Vein Thrombosis (DVT): Blood clots in the veins can obstruct lymphatic flow.
- Inflammatory Conditions: Conditions like rheumatoid arthritis or lupus can lead to inflammation that affects the lymphatic system.
- Cancer: Both the disease and its treatments can cause lymphedema.
- Chronic Venous Insufficiency: When veins have trouble sending blood back to the heart, it can affect the lymphatic system too.
- Immobility: Lack of movement can impair lymphatic drainage.
- Burns: Severe burns can damage lymphatic vessels.
- Scar Tissue: Scar tissue from surgery or injury can block lymphatic flow.
- Varicose Veins: Enlarged veins can impede lymphatic drainage.
- Pregnancy: Hormonal changes and increased pressure on the pelvis can lead to swelling.
- Certain Medications: Some medications may cause fluid retention, leading to swelling.
- Insect Bites: Severe reactions to insect bites can cause lymphedema.
- Chronic Liver Disease: Liver problems can lead to fluid buildup in the abdomen, affecting lymphatic flow.
- Chronic Kidney Disease: Impaired kidney function can cause fluid retention and swelling.
Symptoms of Lymphedema:
- Swelling: Often in the arms or legs, but can occur in other parts of the body too.
- Feeling of Heaviness or Tightness: The affected area may feel heavy or tight.
- Limited Range of Motion: Swelling can make it difficult to move the affected limb.
- Aching or Discomfort: The swollen area may feel achy or uncomfortable.
- Thickening of the Skin: The skin over the swollen area may become thicker or harder.
- Infections: The swollen area may be more prone to infections like cellulitis.
- Decreased Flexibility: Swelling can limit the flexibility of the affected limb.
- Clothing or Jewelry Tightness: Clothes or jewelry may feel tighter than usual due to swelling.
- Difficulty Fitting into Shoes: Swelling in the feet or ankles may make it hard to put on shoes.
- Pitting Edema: Pressing on the swollen area may leave an indentation (pit) that takes time to go away.
- Redness or Warmth: The skin over the swollen area may be red or warm to the touch.
- Loss of Appetite: Severe cases of lymphedema can cause loss of appetite or nausea.
- Fever: Infections associated with lymphedema can cause fever.
- Fatigue: The body may feel tired due to the strain of dealing with swelling.
- Lymph Fluid Leaking: In severe cases, lymph fluid may leak through the skin.
- Thickened Nails: Nails may become thickened or distorted in severe lymphedema cases.
- Emotional Impact: Dealing with a chronic condition like lymphedema can cause emotional stress.
- Difficulty Sleeping: Discomfort from swelling may make it hard to sleep.
- Difficulty Finding Clothes: Swelling can make it challenging to find clothes that fit comfortably.
- Anxiety or Depression: Living with lymphedema can take a toll on mental health.
Diagnostic Tests for Lymphedema:
History
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Thorough family history is essential if primary lymphedema is suspected. It is important to evaluate if other family members, usually from an earlier generation, suffer or have suffered from swollen feet, ankles, and legs due to an “unknown cause.”
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Cancer
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Injuries
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Severe burns
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Edema, especially of an extremity
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Hyperkeratosis: skin becomes scaly and thickens
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Lymphangioma: small blisters and bumps develop on the skin
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Lymphorrhea: lymph fluid leaks from the skin
Physical Exam
Skin
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Dryness, increased thickness, hyperkeratosis, lymphangiomas (blisters containing lymph fluid), abnormally warm or hot, unusually dark skin, and any nodules are all lymphedema indicators.
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Papillomatosis, a cobblestone appearance of the skin, may also be present; it results from dilated and distended lymph vessels enveloped in fibrotic tissue.[rx]
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Hyperkeratosis
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Lymphangioma
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Lymphorrhea
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Positive Stemmer’s sign: inability to pinch a fold of skin at the root of the second toe suggests lymphedema[rx]
Extremity
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Size: Less than a 20% difference in the affected extremity is considered mild or moderate, and greater than 20% is considered severe.[rx]
- Lymphoscintigraphy: A special imaging test that tracks the movement of a radioactive dye through the lymphatic system.
- MRI (Magnetic Resonance Imaging): This imaging test can provide detailed pictures of the lymphatic system.
- CT (Computed Tomography) Scan: Another imaging test that can help visualize the lymphatic system.
- Ultrasound: This test uses sound waves to create images of the lymph nodes and vessels.
- Lymphangiography: A procedure where dye is injected into the lymphatic vessels to make them visible on X-rays.
- Circumferential Measurements: Measuring the circumference of limbs can help track changes in swelling over time.
- Blood Tests: These tests can rule out other conditions that may cause swelling.
- Lymph Fluid Analysis: Analyzing the composition of lymph fluid can provide clues about the underlying cause of lymphedema.
- Biopsy: Removing a small sample of tissue for examination under a microscope can help diagnose certain types of lymphedema.
- Physical Examination: A thorough physical exam can help identify signs of lymphedema and its underlying cause.
- Lymph Node Mapping: Mapping the location and function of lymph nodes can help diagnose lymphatic disorders.
- Doppler Ultrasound: This test can assess blood flow and detect blood clots that may contribute to lymphedema.
- Indocyanine Green Lymphography: A dye is injected into the lymphatic vessels to visualize their function.
- Plethysmography: This test measures changes in limb volume to assess lymphatic function.
- Skin Biopsy: Removing a small sample of skin for examination can help diagnose certain skin-related lymphedema.
- Lymph Node Aspiration: Removing fluid from a swollen lymph node for analysis can help diagnose infections or cancer.
- Lymphatic Pump Testing: This test measures the effectiveness of lymphatic drainage.
- Contrast-Enhanced Ultrasound: Ultrasound imaging with contrast dye can help visualize lymphatic vessels.
- Immunohistochemistry: A technique used to study the distribution of proteins in tissue samples, helpful in diagnosing lymphedema.
- Electromyography (EMG): This test measures electrical activity in muscles and nerves, useful in evaluating muscle function in lymphedema cases.
Non-Pharmacological Treatments for Lymphedema:
- Manual Lymphatic Drainage (MLD): Gentle massage techniques to stimulate lymphatic flow.
- Compression Therapy: Using compression garments or bandages to reduce swelling.
- Exercise: Gentle exercises to promote lymphatic drainage and improve muscle tone.
- Complete Decongestive Therapy (CDT): A comprehensive treatment approach that includes MLD, compression therapy, exercise, and skin care.
- Pneumatic Compression Devices: Devices that use air pressure to massage the limbs and promote lymphatic drainage.
- Compression Pump Therapy: Similar to pneumatic compression devices, but with a pump that inflates and deflates sleeves around the affected limb.
- Skin Care: Keeping the skin clean and moisturized to prevent infections.
- Elevating the Affected Limb: Keeping the swollen limb elevated to reduce swelling.
- Diet and Nutrition: Eating a balanced diet to maintain a healthy weight and reduce fluid retention.
- Hydration: Drinking plenty of water to help flush toxins from the body.
- Smoking Cessation: Quitting smoking can improve circulation and overall health.
- Weight Management: Maintaining a healthy weight to reduce pressure on the lymphatic system.
- Avoiding Tight Clothing: Wearing loose-fitting clothes to prevent constriction of lymphatic flow.
- Avoiding Extreme Temperatures: Both hot and cold temperatures can worsen swelling, so it’s important to stay comfortable.
- Stress Management: Stress can exacerbate lymphedema symptoms, so finding ways to manage stress is important.
- Lymphatic Yoga: Gentle yoga poses designed to promote lymphatic drainage.
- Hydrotherapy: Using water therapy techniques like hot and cold packs to reduce swelling.
- Acupuncture: Some people find acupuncture helpful in managing lymphedema symptoms.
- Biofeedback: Learning to control certain bodily functions like heart rate and blood pressure through mental techniques.
- Support Groups: Connecting with others who have lymphedema can provide emotional support and practical tips for managing the condition.
Drugs Used in the Treatment of Lymphedema:
- Diuretics: Medications that help the body get rid of excess fluid through increased urine output.
- Antibiotics: Used to treat infections that may occur in swollen areas.
- Anti-Inflammatory Drugs: Medications that reduce inflammation and swelling.
- Anticoagulants: Blood-thinning medications used to prevent blood clots that can contribute to lymphedema.
- Analgesics: Pain-relieving medications for discomfort associated with swelling.
- Antihistamines: Medications that can help reduce itching and allergic reactions.
- Proteolytic Enzymes: Supplements that may help reduce tissue swelling and inflammation.
- Pentoxifylline: A medication that may improve blood flow and reduce swelling.
- Benzopyrones: Drugs that may help reduce tissue swelling and improve lymphatic function.
- Vitamin E: An antioxidant that may help reduce tissue inflammation and promote healing.
Surgeries for Lymphedema:
- Lymph Node Transfer: Surgical transfer of healthy lymph nodes to areas affected by lymphedema.
- Vascularized Lymph Node Transfer: Similar to lymph node transfer, but with the addition of blood vessels to improve blood supply.
- Lymphaticovenous Anastomosis (LVA): Surgery to create connections between lymphatic vessels and nearby veins to improve drainage.
- Suction-Assisted Lipectomy: Surgical removal of excess fat and tissue to reduce swelling.
- Vascularized Lymph Node Transplantation: Transplanting lymph nodes along with their blood vessels to improve lymphatic function.
- Lymphatic Bypass Surgery: Creating new pathways for lymphatic fluid to bypass blocked or damaged vessels.
- Liposuction: Removing excess fat deposits to reduce swelling and improve mobility.
- Excisional Procedures: Removing fibrous tissue or excess skin to relieve pressure and improve function.
- Microsurgical Lymphatic Reconstruction: Using microsurgery techniques to repair or bypass damaged lymphatic vessels.
- Lymphovenous Anastomosis: Creating connections between lymphatic vessels and veins to improve drainage.
Prevention of Lymphedema:
- Maintain a Healthy Weight: Excess weight can put pressure on the lymphatic system, so aim for a healthy weight.
- Avoid Infections: Take steps to prevent infections, such as proper wound care and avoiding contact with known sources of infection.
- Protect Against Injury: Be careful to avoid injury to the limbs, especially if you’ve had surgery or radiation therapy.
- Gradual Exercise: Ease into exercise gradually to avoid overexertion that can strain the lymphatic system.
- Compression Garments: If you’re at risk for lymphedema, wearing compression garments during air travel or strenuous activity can help prevent swelling.
- Skin Care: Keep your skin clean and moisturized to prevent dryness and cracking that can lead to infection.
- Avoid Tight Clothing: Wear loose-fitting clothes and jewelry to avoid constriction of lymphatic flow.
- Elevate Limbs: Elevate your arms or legs whenever possible to encourage lymphatic drainage.
- Gradual Temperature Changes: Avoid sudden temperature changes, which can exacerbate swelling.
- Follow Medical Advice: If you’ve had surgery or radiation therapy, follow your healthcare provider’s instructions for preventing lymphedema.
When to See a Doctor:
- Persistent Swelling: If you notice swelling that doesn’t go away, especially in the arms or legs, it’s important to see a doctor.
- Skin Changes: Redness, warmth, or thickening of the skin over swollen areas should be evaluated by a healthcare provider.
- Difficulty Moving Limbs: If swelling is causing difficulty with movement or range of motion, seek medical attention.
- Signs of Infection: Symptoms like fever, chills, or increased pain in the swollen area could indicate an infection that needs prompt treatment.
- Worsening Symptoms: If your symptoms are getting worse over time, don’t hesitate to contact your doctor.
- History of Cancer Treatment: If you’ve had surgery or radiation therapy for cancer, be vigilant for signs of lymphedema and discuss prevention strategies with your healthcare provider.
- Emotional Impact: If lymphedema is causing emotional distress or affecting your quality of life, talk to your doctor about support options.
- Unexplained Symptoms: If you’re experiencing symptoms like fatigue, loss of appetite, or difficulty sleeping along with swelling, it’s important to get evaluated by a healthcare professional.
- Changes in Skin Appearance: Any changes in the appearance of the skin over swollen areas, such as thickening or discoloration, should be checked out by a doctor.
- Concerns About Lymphedema Risk: If you’re concerned about your risk of developing lymphedema, talk to your healthcare provider about prevention strategies and early detection.
Conclusion:
Lymphedema can be a challenging condition to live with, but with early detection, proper treatment, and lifestyle modifications, many people can manage their symptoms and lead full, active lives. By understanding the causes, symptoms, diagnosis, treatment options, and prevention strategies for lymphedema, you can take proactive steps to protect your health and well-being. If you have any concerns about lymphedema or notice any symptoms, don’t hesitate to reach out to your healthcare provider for guidance and support.
Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, previous medical history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. Thank you for giving your valuable time to read the article.
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