Chronic hereditary lymphedema (also known as Milroy disease) is a condition characterized by lower limb lymphedema. Patients typically present with pedal edema at or before birth or soon after. Occasionally, it develops later in life.
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Clinical presentation
The commonest presentation is bilateral asymmetric lymphedema in the lower limbs. However, all distal extremities can be affected. The edema tends to be progressive 4.
Complications can include:
- pleural effusion
- recurrent septic arthritis
- skin changes
- infections of the toes
- intestinal lymphangiectasia
Pathology
Genetics
Mutations in the FLT4 gene are thought to be present in some cases. It is usually inherited in an autosomal dominant pattern.
Treatment
Management is usually conservative, and involves limb elevation, skin care, compression and manual lymphatic drainage. Surgical bypass and debulking procedures are considered a last resort 4.
History and etymology
It is thought to have first been described by Letessier in 1865 1.
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