What is lymphedema?

It is localized swelling of the body caused by an abnormal accumulation of lymph. Which is a colourless fluid (ultrafiltrate) containing white blood cells, which bathes the tissues and drains through the lymphatic system into the bloodstream.

What are the causes of lymphedema?

Primary lymphedema developmental abnormality of the lymphatic system is termed primary lymphedema. They are of three types

• Congenital lymphedema is clinically evident at birth and accounts for 10% to 25% of all primary lymphedemas
• Lymphedema praecox is the most common form of primary lymphedema accounting for 65% to 80% beginning after birth and before 35 years of age. These patients usually present with unilateral limb (70%) oedema beginning after birth and before 35 years of age. Presentation at puberty is the most common age and females are affected four times as frequently as males.
• Lymphedema tarda is a primary form of lymphedema that manifests clinically after the age of 35 years and accounts for (10%) of primary lymphedema. It most commonly affects the lower extremity and occurs more commonly in women.

Secondary lymphedema is the acquired dysfunction of the otherwise normal functioning lymphatics. The causes for which may be -

• Traumatic lymphedema occurs as a consequence of scarring or injury following trauma, cancer surgery (breast and gynaecological cancer), burn injury, or radiation exposure. It can also occur after extensive skin resection performed for a variety of conditions including massive weight loss.
• Infective lymphedema is caused by invasion and subsequent destruction of the lymphatic vessels with a foreign organism. This is usually the filarial worm.  But is associated with tuberculosis bacteria or other infective bacteria and fungi. 
• Malignant lymphedema is caused as a result of infiltration and obstruction of the lymphatic system with malignant cells.
• Post-venous thrombosis lymphedema can occur after ligation or thrombosis of a major extremity vein

What are the symptoms of lymphedema?

• Limb swelling 
• Tightness in the skin
• Heaviness of the limb, fatigue, and difficulty moving a joint 
• Recurrent infections.

How is lymphedema diagnosed?

• Lymphoscintigraphy
• Indocyanine green dye scan 
• MRI

What is the staging of lymphedema?

Treatment depends on the stage of the lymphedema

How is lymphedema treated?

Non-surgical treatment is advocated in the early stages

• Complex decongestive therapy (CDT) is the mainstay of lymphedema management and aims to decrease the amount of fluid in lymphedematous tissues. CDT is comprised of multiple therapies that include manual lymphatic drainage (MLD), skincare, compression wraps with short-stretch bandages, and light exercises. 
• Compression therapy includes a wide range of treatments, including multilayer bandaging, self-adherent wraps, and custom-made pressure garments.
• Intermittent pneumatic compression where is a pneumatic cuff is connected to a pump that simulates the natural pump effect of muscular contraction on the peripheral lymphatic system. There is currently no consensus on the use of these devices.

Surgical treatment

Lymph venous bypass

The lymphatic vessels (green) are anastomosed to the veins (blue).

Lymph node transfer

Lymph nodes from the neck or the groin are transferred to the affected limb.

Lymphedema reduction surgeries

These are done is an extensive disease with severe skin changes

Charles procedure wherein the involved affected skin is excised up to the fascia and grafting with healthy skin is performed.

Sistruck procedure wherein the limb size is reduced by debulking and primarily suturing.

A combination of the above surgical procedures may be required in multiple stages for the treatment of the patient depending on the presentation and response to the initial treatment.