Congenital lymphedema of genitalia and extremities1
Kenneth J. Tomecki, M.D.
Jacob W. E. Dijkstra, M.D.
Wilma F. Bergfeld, M.D.
Robert Kay, M.D.
A boy presented at birth with congenital lymphedema of the genitalia. During the following two years, lymphedematous swelling of the right arm and lymphangiectasias, as shown by microscopic examination of noninvolved skin of the other extremities, developed. These generalized lymphatic malformations are rare. The clinical and histopathological changes are detailed, and the possible consequences of further-developed systematized lymphedema and/or lymphangiosarcoma are discussed. Conservative management, including compression therapy, was ineffective. The initial results of surgical reconstruction were satisfactory.