In adults, usually after surgery or radiotherapy. But can be primary, appearing in childhood (but not necessarily in early childhood). Secondary causes include Klippel-Tranaunay, Noonans, Turners, other venous malformation.
The incidence of primary lymphoedema is approximately 1 in 6,000 births. Approximately 10 children are born each year with primary lymphoedema in Scotland.
Lymphoedema, particularly if not well controlled, “carries significant human, personal, financial and societal costs”. Apart from cosmetic issues, pain is often a significant problem (underestimated), plus risk of cellulitis. Inadequate treatment increases risk of complications:
- fibrosis,
- papillomatosis (warty growths consisting of dilated lymphatics and fibrous tissue)
- lymphorrhoea (leakage of lymph fluid through the skin),
- functional limitations
- psychological morbidity, social isolation and limitation of life choices, including employment opportunities
So early diagnosis and control important.
Examination
Deep pressure for at least 30 seconds! If pits, then lymphatics ok.
Positive Stemmer sign= the inability to pick up a fold of skin at the base of the second toe, indicating thickening of the skin. This is useful in differentiating lymphoedema from other forms of oedema. [http://www.woundsinternational.com/ Journal of Lymphoedema, 2009, Vol 4, No 2]
Investigations
Consider secondary causes, as above.
Consider LFTs, thyroid function (impaired lymphatic drainage seen in hypothyroidism, hence oedema and even effusions).
Imaging to assess venous blood flow eg USS with dopplers.
MRI can show hypertrophy of fat, as in lipoedema (see below), or other tissues (viz Klippel Trenaunay).
Lymphoscintigraphy is gold standard but not readily available.
Differential is Lipoedema – almost exclusively females, presents at or after puberty, symmetrical lower limb enlargement. Often a history of easy bruising and tenderness in the affected limbs. Lymphoedema can later develop as a secondary complication.
Further advice from http://www.thebls.com/patients/index.php?bls_user=9f0a85c93b84f4e36a2629f6d17c38e7
Patient support from http://www.lymphoedema.org/