Outcomes of one-stage feminizing genitoplasty in children with congenital adrenal hyperplasia and severe virilization
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In brief
In brief
This study reports outcomes of one-stage feminizing reconstruction in 14 CAH patients with severe virilization (Prader IV-V) using combined total urogenital mobilization and modified pull-through vaginoplasty. At median 4-year follow-up, 78.5% achieved good cosmetic results with normal urinary continence, demonstrating this approach effectively addresses high vaginal confluence with short urethral length.
Written by the GCMD Library team from the article.
Abstract
Purpose
To present our surgical experience and outcomes in congenital adrenal hyperplasia (CAH) patients with severe virilization using a combined technique of total urogenital mobilization (TUM) and a modified pull-through vaginoplasty to perform a safe and effective one-stage feminizing genital reconstruction for these children.
Methods
Fourteen CAH patients with severe virilization, defined by a Prader IV and V rating of the external genitalia, underwent TUM followed by a limited vaginal pull-through procedure from June 2016 to December 2020. Postoperative anatomical and cosmetic outcomes, and urinary continence, were evaluated.
Results
Out of the 14 cases in this study, 8 were classified as prader IV and 6 as Prader V. The median age at surgery was 11 months (range 6–36 months), and the mean urethral length was 1.4 cm (range 1.2–1.8 cm). The median follow-up period was 4 years. Our cosmetic outcomes were good in 11 (78.5%), satisfactory in 2, and poor in one case. All patients achieved age-appropriate toilet training without urinary incontinence.
Conclusion
Adopting our surgical approach of TUM with modified pull-through vaginoplasty has simplified feminizing surgical reconstruction in CAH cases with severe genital atypia and a very high vaginal confluence with short urethral length, yielding adequate introitus with good anatomical and cosmetic appearance and adequate urinary continence outcomes.