Schook CC, Kulungowski AM, Greene AK, Fishman SJ, et al.
Journal of pediatric surgery. Date of publication 2014 Nov 1;volume 49(11):1647-51.
1. J Pediatr Surg. 2014 Nov;49(11):1647-51. doi: 10.1016/j.jpedsurg.2014.05.031.
Epub 2014 Jul 1.
Male genital lymphedema: clinical features and management in 25 pediatric
patients.
Schook CC(1), Kulungowski AM(2), Greene AK(1), Fishman SJ(3).
Author information:
(1)Department of Plastic and Oral Surgery, Boston Children's Hospital, Boston, MA
02115, USA; Vascular Anomalies Center, Boston Children's Hospital, Harvard
Medical School, Boston, MA 02115, USA.
(2)Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA;
Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School,
Boston, MA 02115, USA.
(3)Department of Surgery, Boston Children's Hospital, Boston, MA 02115, USA;
Vascular Anomalies Center, Boston Children's Hospital, Harvard Medical School,
Boston, MA 02115, USA. Electronic address: steven.fishman@childrens.harvard.edu.
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.
Copyright © 2014 Elsevier Inc. All rights reserved.
DOI: 10.1016/j.jpedsurg.2014.05.031
PMID: 25475811 [Indexed for MEDLINE]
