PT  - JOURNAL ARTICLE
AU  - Gabriela E Jones
AU  - Sahar Mansour
TI  - An approach to familial lymphoedema
AID  - 10.7861/clinmedicine.17-6-552
DP  - 2017 Dec 01
TA  - Clinical Medicine
PG  - 552--557
VI  - 17
IP  - 6
4099  - http://www.rcpjournals.org/content/17/6/552.short
4100  - http://www.rcpjournals.org/content/17/6/552.full
SO  - Clin Med2017 Dec 01; 17
AB  - Lymphoedema is the build-up of lymphatic fluid leading to swelling in the tissues. Most commonly it affects the peripheries. Diagnosis is based on clinical assessment and imaging with lymphoscintigraphy. Treatment is supportive with compression garments, massage, good skin hygiene and prompt use of antibiotics to avoid the complication of cellulitis. Most commonly, lymphoedema occurs as a result of damage to the lymphatic system following surgery, trauma, radiation or infection. However, it can be primary, often associated with a genetic defect that causes disruption to the development of the lymphatic system. Common genetic conditions associated with lymphoedema include Turner syndrome and Noonan syndrome; however, there are numerous others that can be classified based on their clinical presentation and associated features. Herein we discuss how to diagnose and classify the known primary lymphoedema conditions and how best to investigate and manage this group of patients.