Overview

Lymphoedema is a chronic disease showcased by lymphatic fluid building up in the body causing swelling, which can cause changes in the skin and tissue. Swelling can appear in the legs, arms, face, genitals, neck, and chest wall.¹

This lymphatic fluid is called lymph and lymph circulates throughout the lymphatic system. Lymph is transported around the body by lymphatic vessels and is filtered through lymph nodes. A blockage of the lymphatic tract can cause an issue in drainage which causes lymph to accumulate between cells, causing swelling, resulting in the condition known as lymphoedema.²

Types of lymphoedema

There are two types of lymphoedema: primary (genetic) and secondary (acquired).¹

Primary lymphoedema is an inherited condition that causes an abnormality of the lymphatic system due to genetic mutations.¹ It is subdivided into 3 categories: congenital lymphoedema, lymphoedema praecox, and lymphoedema tarda.^1,2

Congenital lymphoedema also known as Milroy’s disease is the second most common type of primary lymphoedema. It occurs in the first 2 years of life.²

Lymphoedema praecox is also known as Meige’s disease and is the most common form of primary lymphoedema. It occurs more in women with an incidence of 1:100,000 in the population with a 4:1 female/male ratio. This type of lymphoedema presents itself during puberty. Primary lymphoedema can be linked to some abnormalities, including hearing loss, vertebral and cerebrovascular deformities, and distichiasis, or having two rows of eyelashes.²

Lymphoedema tarda is the rarest form of primary lymphoedema and occurs later in adulthood, > 35 years of age.²

Secondary lymphoedema occurs due to injury or obstruction of the lymphatic system¹ as well as from cancer, infection, or surgery.² Secondary lymphoedema is the more common form of the two types of lymphoedema.³ Breast cancer is the most common type of cancer associated with secondary lymphoedema, with 1 in 5 women who survived breast cancer developing secondary lymphoedema.¹

Causes of lymphoedema

Primary lymphoedema is linked to lymphatic dysplasia and other disorders such as Turner syndrome and Klippel-Trenaunay-Weber syndrome. The lymphatic vessels show hyperplasia, hypoplasia, or aplasia in primary lymphoedema.¹ Around 20 genes have been associated with irregularities in the lymphatic system, which lead to lymphatic structures being underdeveloped or the lymphatic outflow capacity being inadequate.³

Secondary lymphoedema results from damage or obstruction to the lymphatic system. The most common cause of secondary lymphoedema worldwide is Filariasis. Filariasis results from parasites, Wuchereria bancrofti­, infesting in the lymph nodes, thus causing infection and resulting in secondary lymphoedema. Millions of people who are affected by this are residing in the regions of Asia, Africa, Central and South America, and the Western Pacific.¹

Secondary lymphoedema can also be caused by surgical procedures used for cancer - biopsies of lymph nodes and radical dissection, which include removing lymph nodes or arteries. Other surgical procedures such as excisions of burn scars, lipectomy, and vein stripping can be a cause of secondary lymphoedema.¹

Non-surgical causes of lymphoedema include:¹

• Tumours or malignancies that have metastasized to the lymph nodes
• Obstructive lesions in the lymphatic system
• Lymphatic vessels that have become infected/traumatised
• Lumen of lymphatic vessels being destroyed by scar tissue
• Oedema from DVT (deep vein thrombosis)

Staging of lymphoedema

There are 4 stages of lymphoedema – Latency stage (0), Spontaneous (1), Spontaneous irreversible (2), and Lymphostatic elephantiasis (3).¹

Stage 0 - Latency stage

• Considered ‘at risk’ for lymphoedema development due to injury to the lymphatic vessel but oedema is not present
• Breast cancer patients are included – patients who have undergone biopsies and radiation but have not developed swelling in the body
• Reduction in the capacity for lymphatic transport – predisposes the patient to lymphatic overload and can result in oedema¹

Stage 1 – Spontaneous

• It is reversible
• Pitting oedema
• Swelling is soft and may go down with limb elevation¹

Stage 2 – Spontaneously irreversible

• Tissue fibrosis/induration
• Limb elevation does not help to swell
• As limb volume increases the skin and tissues continue to thicken
• Pitting may be present, but due to fibrosis of skin or tissue it may be hard to assess¹

Stage 3 – Lymphostatic elephantiasis

• Pitting oedema
• Skin changes
• Fibrosis
• Papillomas may form due
• Infections/cellulitis may occur
• Dry skin¹

Signs and symptoms of lymphoedema

• Swelling in extremities – hands, legs, arms, feet
• Swelling proximally – chest, pelvis, groin, breast, shoulder, genitals, face
• Restricted range of motion due to swelling
• Discolouration of skin
• Heavy limbs
• Pain and altered sensation
• Lymphangioma – blisters and bumps on the skin
• Lymphorrhea – fluid leaking from the skin
• Positive Stemmer’s signs – unable to pinch the skin at the base of the second toe

Management and treatment for lymphoedema

Complex decongestive therapy

Complex decongestive therapy (CDT) consists of several components to reduce swelling, improve the flow of lymph, and relieve pressure. The main components consist of:⁴

• Manual lymphatic drainage (MLD) – massage technique promoting lymph flow and helping reduce swelling
• Exercise therapy (ET) – exercises to support lymph flow and activate muscles
• Skincare – taking care of the skin reduces infections and any skin issues, which are common in people with lymphoedema. The skin should be kept clean and moisturised to avoid any infections occurring
• Compression therapy (CT) – compression bandages or garments are used on the affected area to increase pressure. This reduces swelling and promotes the flow of lymph
• Self-management – continuing therapy by applying bandages, self-massages, and exercises

Surgical interventions

• Vascularised lymph node transfer (VLNT) - Using a microsurgical approach, a free lymph node flap is harvested from outside the affected area, implanted into the diseased limb, and anastomosed to recipient vessels (without connecting the lymphatic tract)²
• Liposuction – a cannula is inserted and fatty tissue is suctioned out by negative pressure²
• Lymphatic Venous Anastomosis (LVA) – creates connections between the lymphatic and venous systems in the affected area. Superficial or deep lymphatics are anastomosed to nearby veins⁵

Diagnosis

A diagnosis of lymphoedema is made by carrying out a medical history and physical examination of the patient. During the physical examination any signs of skin dryness, increased skin thickness, blisters, pigmentation, and skin being abnormally warm/hot are key indicators of lymphoedema.¹

Lymphoscintigraphy is a procedure that confirms the diagnosis of lymphoedema. It is a procedure that injects a small amount of radioactive protein dye in the webbed area between the first and second digits of the affected limb. Gamma cameras are used to image the limb and observe the dye moving through the lymphatic system. Any dye shown to be outside the lymphatic structures suggests an oedema.¹

MRIs, CTs, bioelectrical impedance analysis, and ultrasounds are other ways to aid in the diagnosis of lymphoedema.¹

Risk factors

Risks of developing lymphoedema include:²

• Older age
• Obesity
• Cancer
• Cancer and post-cancer treatment
• Surgery

Complications

Complications of lymphoedema include:¹

• Cellulitis
• Infections – superficial bacterial and fungal
• Deep vein thrombosis (DVT)
• Lymphangitis
• Lymphangioadenitis
• Impaired functional mobility

FAQs

How can I prevent lymphoedema

Ways to avoid lymphoedema:

• Prevent infections – keep skin clean, use antibacterial cream to treat cuts or breaks in skin
• Do not constrict limbs with anything tight such as jewellery, tight clothes, tight bands
• Try to maintain a healthy weight for your height⁶

How common is lymphoedema

Primary lymphoedema affects 1 in 100,000 individuals and is the rarer form of the two. The most common type of lymphoedema is secondary lymphoedema.¹

What are the stages of lymphoedema?

There are 4 stages of lymphoedema:¹

Stage 0 – latency stage

• At risk of developing lymphoedema

Stage 1 – Spontaneous

• Early lymphoedema
• At a reversible stage
• Improves with elevating limbs

Stage 1 – Spontaneously irreversible

• Possible pitting oedema
• Elevation does not help with swelling

Stage 3 – Lymphostatic elephantiasis

• Skin changes
• Pitting oedema 

What can I expect if I have lymphoedema

A few things you could expect:¹

• Swelling in extremities – hands, feet, legs, arms
• Skin changes – increased thickness, pigmentation, dryness
• Pain
• Limbs feeling heavy
• Restricted motion
• A few complications could arise depending on the severity of the disease

When should I see a doctor

If you showcase any symptoms/signs of lymphoedema then it may be best to see a doctor soon. Early diagnosis allows healthcare professionals to be made aware of the lymphoedema and promptly start managing and caring. Lymphoedema is a progressive disease so early diagnosis and treatment are important.¹

Summary

Lymphoedema is a condition where the buildup of lymphatic fluid causes swelling in parts of the body. There are two types of lymphoedema: Primary (genetic) or secondary (acquired). Causes vary from genetic mutations to surgery or cancer. The disease advances through four stages 0 - 3.

Swelling, restricted movement, darker skin, and pain are key signs of lymphoedema. Diagnosis includes a physical exam, history, and procedures like lymphoscintigraphy. Risk factors include older age, obesity, cancer, and specific medical treatments. Complications may lead to cellulitis, infections, and impaired mobility.

Managing lymphoedema can involve complex decongestive therapy (CDT), integrating manual lymphatic drainage (MLD), exercise, skincare, and compression therapy (CT). Surgical interventions like vascularised lymph node transfer and liposuction are available as treatment. Prevention focuses on maintaining a healthy weight, preventing infections, and avoiding anything constricting on limbs and extremities.

Early diagnosis and treatment are crucial for effective management, with emphasis on the importance of seeking medical attention if symptoms arise.