Primary lymphoedema

Description 

A 32-year-old woman presented with a 20-year history of slowly progressive leg oedema with recurrent cellulitis. She was referred from her primary care physician. Her mother had the same condition. Physical examination revealed non-pitting bilateral leg oedema (figure 1). Stemmer's sign was positive. Lymphoscintigraphy showed non-visualisation of inguinal nodes 30 min postexercise and dermal backflow in the lower limbs (figure 2). Compression garments were used but failed. She underwent bilateral lower limb supermicrosurgical lymphaticovenular anastomosis (two times anastomosis and four times incisions) and her oedema markedly improved. Four years after the operation, she was well without relapse.

Figure 1

Non-pitting bilateral leg oedema.

Figure 2

Preoperative lymphoscintigraphy. Lymphoscintigraphy revealed no migration of radiopharmaceutical agent in the lower limbs.

Primary lymphoedema is due to a congenital and/or inherited condition associated with pathological development...

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