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A full-term infant born to a woman (gravida 1, para 1) with a history of gestational diabetes presented with edema of the right leg (Panel A). When the infant was one week of age, lymphoscintigraphy (Panel B) showed prompt migration of the radiopharmaceutical agent from the left foot to the inguinal lymph nodes, but there was no migration in the right leg. There was no evidence of a lymph channel. The findings on this scan suggested the presence of congenital aplasia of the lymph system in the right leg. Subsequent magnetic resonance imaging (Panel C) revealed some patchy infiltration of the subcutaneous fat in the posterior aspect of the right thigh, with nearly complete involvement of the right calf and without obvious muscular involvement. These findings supported the diagnosis of congenital lymphedema. When the infant was four and a half months of age, her right calf and foot were debulked with soft-tissue reconstruction (Panel D).
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