Thursday, August 30, 2012

Primary upper-limb lymphedema.

Primary upper-limb lymphedema.

August 2012

Vignes S, Arrault M, Yannoutsos A, Blanchard M.


Department of Lymphology, Centre National de Référence des Maladies Vasculaires
Rares (lymphœdèmes primaires), Hôpital Cognacq-Jay, 75015 Paris, France.


Background: Lymphedema is a general term applied to designate pathological regional accumulation of protein-rich fluid. It can be either primary or secondary, mainly after cancer treatment.

Objective: To analyze clinical and lymphoscintigraphic characteristics of  primary upper-limb lymphedema. 

Method: All patients with upper-limb lymphedema (January 2007-December 2011) recruited in a single Department of Lymphology were included.

Results: Sixty patients (33 females, 27 males) were enrolled. For the 54 noncongenital lymphedemas, mean age at onset was 38.5 (3-82) years. Lymphedema was unilateral in 51 (85%) patients. It always affected the hand, but less often the forearm (55%) or upper arm (23%). Eleven (18%) patients developed cellulitis after lymphedema onset. Twenty-one (35%) patients had associated lower-limb lymphedema. Forty-six patients (with 49 lymphedematous limbs) underwent
lymphoscintigraphy: axillary lymph-node uptake was diminished in 18 (37%), absent in 24 (49%) and normal in 7 limbs (14%). Among the 43 patients with unilateral lymphedema and lymphoscintigraphy, 28 had epitrochlear node visualization, suggesting a re-routing through the deep lymphatic system, 15 only on the lymphedematous limb and 22 on the contralateral nonlymphedematous limb. Median follow-up was 103 months and 57/60 (95%) patients considered their lymphedema stable.

Conclusion: Primary upper-limb lymphedema appears later in life than lower-limb lymphedema without sex predominance. Infectious complications are rare and patients considered the lymphedema volume stable throughout life.