Background
Lymphoedema refers to the swelling of soft tissues which follows when the body’s lymphatic system is disrupted. Lymphoedema commonly affects the limbs and extremities, however can occur anywhere the lymphatic system is damaged.
Lymph (the contents of lymphatic vessels) is produced from blood plasma; it forms the liquid that leaks from capillaries and into interstitial spaces in body tissues. The majority of this fluid re-enters the capillaries and returns to the venous circulation while the remaining fluid moves into lymphatic vessels, to ultimately drain into major veins in the torso.
In lymphoedema, the disrupted lymphatics cannot drain the lymph effectively, causing tissue to swell as fluid accumulates (lymphostasis). Over time this leads to chronic inflammatory changes.
Signs & symptoms
- Gradual swelling of a limb or an extremity. The genitalia may also be affected.
- Initially pressure on the swelling will leave an indentation (pitting), but with time tissues will become firmer and non-compressible.
- Changes in sensation of the affected area (e.g. pins and needles, feeling of heaviness).
- Pain and difficulty with daily activities.
- Changes to appearance of overlying skin e.g. eczema/ dermatitis, hyperpigmentation, skin infections
Diagnosis
Lymphoedema is primarily a clinical diagnosis.
Primary lymphoedema: a rare developmental problem affecting the lymphatic system. This is usually present from birth, however may not become apparent until later in life, e.g. Milroy disease.
Secondary lymphoedema: damage occurs to an originally healthy lymphatic system. This is the most common type of lymphoedema in children and there are a multitude of causes:
- Cancer or cancer-related treatment (radiotherapy is the most common childhood cause)
- Surgery
- Infection
- Trauma
Management
Management is based around reducing symptoms and minimising disease progression. This requires a multidisciplinary team of doctors, therapists and nurses.
Conservative treatment: elevation, skin care, compression, exercise, weight management (in overweight patients).
Medical treatment: antibiotics, antiparasitics (depending on underlying cause).
Surgery can be considered in certain patient groups.
References
https://bestpractice.bmj.com/topics/en-gb/610
https://dermnetnz.org/topics/lymphoedema/
https://www.who.int/news-room/fact-sheets/detail/lymphatic-filariasis