Summary
Introduction
Little data exist about the surgical interventions taking place for children with
disorders of sex development (DSD). Most studies that have evaluated cosmetic outcomes
after genitoplasty have included retrospective ratings by a physician at a single
center.
Objective
The present study aimed to: 1) describe frequency of sex assignment, and types of
surgery performed in a cohort of patients with moderate-to-severe genital ambiguity;
and 2) prospectively determine cosmesis ratings by parents and surgeons before and
after genital surgery.
Study design
This prospective, observational study included children aged <2 years of age, with
no prior genitoplasty at the time of enrollment, moderate-to-severe genital atypia,
and being treated at one of 11 children's hospitals in the United States of America
(USA). Clinical information was collected, including type of surgery performed. Parents
and the local pediatric urologist rated the cosmetic appearance of the child's genitalia
prior to and 6 months after genitoplasty.
Results
Of the 37 children meeting eligibility criteria, 20 (54%) had a 46,XX karyotype, 15
(40%) had a 46,XY karyotype, and two (5%) had sex chromosome mosaicism. The most common
diagnosis overall was congenital adrenal hyperplasia (54%). Thirty-five children had
surgery; 21 received feminizing genitoplasty, and 14 had masculinizing genitoplasty.
Two families decided against surgery. At baseline, 22 mothers (63%), 14 fathers (48%),
and 35 surgeons (100%) stated that they were dissatisfied or very dissatisfied with
the appearance of the child's genitalia. Surgeons rated the appearance of the genitalia
significantly worse than mothers (P < 0.001) and fathers (P ≤ 0.001) at baseline. At the 6-month postoperative visit, cosmesis ratings improved
significantly for all groups (P < 0.001 for all groups). Thirty-two mothers (94%), 26 fathers (92%), and 31 surgeons
(88%) reported either a good outcome, or they were satisfied (see Summary Figure);
there were no significant between-group differences in ratings.
Discussion
This multicenter, observational study showed surgical interventions being performed
at DSD centers in the USA. While parent and surgeon ratings were discordant pre-operatively,
they were generally concordant postoperatively. Satisfaction with postoperative cosmesis
does not necessarily equate with satisfaction with the functional outcome later in
life.
Conclusion
Graphical Abstractpostoperative cosmesis ratings at baseline and 6 months.
Keywords
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Article Info
Publication History
Published online: October 08, 2016
Accepted:
August 31,
2016
Received:
May 18,
2016
Identification
Copyright
© 2016 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.
