Abstract :
Introduction: Lymphoedema is a chronic, progressive debilitating disorder of lymphoreticular system, characterized by abnormal collection of excessive proteins, oedema, chronic inflammation and fibrosis.
Classification: Primary lymphoedema, associated with underlying, but inherent malfunction of lymphatic channels, unilateral or bilateral. It can be further subdivided in to congenital lymphoedema, lymphoedema praecox and lymphoedema tarda. Depends on the age of onset or manifestation. Another genetic disorder of lymphatic dysfunction is Milroy disease. It is an autosomal dominant condition due to VEGFR3 mutation characterised by lymphatic hypoplasia. Secondary lymphoedemas are more frequent than primary to dysfunction of lymphatics. Mostly localised, follwing recurrent infections, trauma, surgery, obesity and malignancy.
Pathophysiology: Inadequate drainage of lymphatics, leads to accumulation of lymph, interstitial fluid and proteins in skin and subcutaneous tissue. Elastic tissue disruption, fibroblast stimulation occurs and the resulting hypoxia causes chronic inflammation and fibrosis. Later adipocyte remodelling occurs and proinflammatory cytokines secreted.
Clinical Features: Starts as pitting non tender pedal oedema, later skin thickening and fibrosis which results in non-pitting oedema. Squared off appearance and Kaposi Stemmer sign are characteristic features. May develop lymphorrhoea and secondary infections may supervene.
Confusions: Chronic venous
insufficiency, lipoedema, myxedema.
Complications: Vicious cycle of infections and lymphedema.
Diagnosis: Lymphoscintigraphy, lymphoflouroscopy- gold standard.
Treatment: Depends on severity. Pneumatic compression garments, Low level light therapy (LLLT), Extracorporeal shock wave therapy (ESWT). Advanced stages may require surgical debulking, lymphatic reconstruction. But the results are mostly unsatisfactory, recurrences are inevitable. Combined multidisciplinary approaches will be helpful and that should be continued throughout their life.
Keyword :
Primary congenital, Lymphoedema, Lymphoscintigraphy, Lipoedema.