Outcomes of one-stage feminizing genitoplasty in children with congenital adrenal hyperplasia and severe virilization.
Pediatr Surg Int
; 40(1): 72, 2024 Mar 06.
Article
in En
| MEDLINE
| ID: mdl-38446278
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.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Adrenal Hyperplasia, Congenital
Limits:
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
Language:
En
Journal:
Pediatr Surg Int
/
Pediatr. surg. int
/
Pediatric surgery international
Year:
2024
Document type:
Article