Male genital lymphedema: clinical features and management in 25 pediatric patients

PURPOSE: Genital lymphedema in the pediatric population is poorly understood. The purpose of this study was to determine the epidemiology, morbidity, and treatment outcomes for males with genital lymphedema.

MATERIALS AND METHODS: Male patients with genital lymphedema evaluated at our vascular anomalies center between 1995 and 2011 were reviewed. Etiology, age-of-onset, location, morbidity, and treatment were analyzed.

RESULTS: Of the 3889 patients with vascular anomalies, 25 (0.6%) had genital lymphedema: 92% (23/25) with primary and 24.0% (6/25) with familial/syndromic lymphedema. For primary disease, the mean age-of-onset was 4.5 ± 6.3 years with 60.9% (14/23) presenting in infancy, 13.0% (3/23) in childhood, and 26.1% (6/23) in adolescence. Combined penoscrotal lymphedema was identified in 72.0% (18/25) of patients; 19 children (76.0%) had concomitant lower extremity involvement. The most common complication was cellulitis (24.0%). Surgical contouring was performed in 44.0% (11/25) of patients. Patients with operative intervention and follow-up (n=6) had sustained improvement after a median of 4.2 years (range: 0.3-11.0).

CONCLUSIONS: Lymphedema of the male genitalia is typically idiopathic. Most patients develop swelling in infancy but can present in adolescence and occasionally childhood. The penis and scrotum are usually both involved and concurrent lower-extremity swelling is common. Surgical debulking can improve symptoms and appearance.