PURPOSE: Congenital lymphedema is a rare disorder that may result in disfiguring edema of the male genitalia. We reviewed our experience with 5 cases to advance our understanding of this challenging problem. MATERIALS AND METHODS: Four boys with significant lymphedema underwent excision of the involved subcutaneous genital tissue and coverage with local skin flaps. Two boys in whom this approach failed later underwent complete excision of the involved subcutaneous tissue and skin, and coverage with split thickness skin grafts. The boy with minimal edema was observed. RESULTS: Two of the 4 boys who underwent subcutaneous genital tissue resection and coverage with local skin flaps are markedly improved, although 1 requires further revision. In the other 2 boys treatment failed, necessitating repeat genital tissue excision and grafting. While there have been no recurrences in the grafted areas, each patient has required additional operations to manage recurrent edema in adjacent tissues of the perineum and inguinal region, and in 1 significant contraction of the grafted skin developed. Mild genital lymphedema in the remaining patient has remained stable during 10 years of followup. CONCLUSIONS: Congenital lymphedema of the genitalia is a challenging problem. Recurrences requiring multiple operations are common. We recommend expectant management of mild cases. In more severe cases excision without grafting should be attempted. While skin grafting may be the most definitive solution, it does not prevent recurrence in adjacent regions, and it carries the risk of skin contraction. Skin grafts should only be used when other techniques have failed.
PURPOSE:Congenital lymphedema is a rare disorder that may result in disfiguring edema of the male genitalia. We reviewed our experience with 5 cases to advance our understanding of this challenging problem. MATERIALS AND METHODS: Four boys with significant lymphedema underwent excision of the involved subcutaneous genital tissue and coverage with local skin flaps. Two boys in whom this approach failed later underwent complete excision of the involved subcutaneous tissue and skin, and coverage with split thickness skin grafts. The boy with minimal edema was observed. RESULTS: Two of the 4 boys who underwent subcutaneous genital tissue resection and coverage with local skin flaps are markedly improved, although 1 requires further revision. In the other 2 boys treatment failed, necessitating repeat genital tissue excision and grafting. While there have been no recurrences in the grafted areas, each patient has required additional operations to manage recurrent edema in adjacent tissues of the perineum and inguinal region, and in 1 significant contraction of the grafted skin developed. Mild genital lymphedema in the remaining patient has remained stable during 10 years of followup. CONCLUSIONS:Congenital lymphedema of the genitalia is a challenging problem. Recurrences requiring multiple operations are common. We recommend expectant management of mild cases. In more severe cases excision without grafting should be attempted. While skin grafting may be the most definitive solution, it does not prevent recurrence in adjacent regions, and it carries the risk of skin contraction. Skin grafts should only be used when other techniques have failed.