Anatomical and functional outcomes of feminizing genitoplasty for ambiguous genitalia in patients with virilizing congenital adrenal hyperplasia.
The ideal surgical technique and appropriate age for performing feminizing genitoplasty are debatable, and few long-term outcome studies have been reported.


OBJECTIVE
To report a retrospective study on anatomical and functional outcomes of feminizing genitoplasty in patients with virilizing congenital adrenal hyperplasia.


METHODS
We selected 34 patients (mean age = 3.4 +/- 2.5 yr) with genital ambiguity classified according to Prader stage. Follow-up ranged from 2 to 16 years. Clitoral length ranged from 1.9 to 5.0 cm; 28 patients had a single perineal orifice, and 6 had a double orifice. The surgical technique included clitorovaginoplasty in a single procedure and was carried out before 2 years of age in 18 patients. Clitoroplasty was performed with glans preservation in all patients. Blood supply was exclusively maintained by the frenular pedicle in 97% of the cases, whereas clitoral dorsal nerves and vessels were preserved in the remaining 3%. The opening of the urogenital sinus was performed using either the Y-V perineal flap procedure (25 patients) or the cut-back incision procedure (8 patients).


RESULTS
Good morphological and functional results were achieved in 68% of the patients; 21% of the patients had surgical complications, such as incision bleeding (2 cases), glans necrosis (1 girl with Prader V), and vaginal introitus stenosis (4 cases). Three of the latter underwent dilation with acrylic molds in the post-pubertal period with good functional results.


CONCLUSIONS
We conclude that single-stage feminizing genitoplasty consisting of vulvoplasty, clitoroplasty, and Y-V perineal flap produced good cosmetic and functional results in virilized girls with congenital adrenal hyperplasia, with few complications. In addition, this surgical approach prevented the need for neovaginaplasty even in patients with high vaginal insertion.
Sircili
Maria Helena Palma
MH
Endocrinology Unit, Hospital das Clínicas, São Paulo University Medical School, Brazil. mhps_msm@yahoo.com
de Mendonca
Berenice B
BB
Denes
Francisco Tibor
FT
Madureira
Guiomar
G
Bachega
Tânia Aparecida Sartori Sanchez
TA
e Silva
Frederico Arnaldo de Queiroz
FA
D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D013509:Gynecologic Surgical Procedures / Q000379:methods; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D016896:Treatment Outcome; D014621:Vagina / Q000601:surgery*; D014770:Virilism / Q000601:surgery*