Surgical Treatment after Failed Primary Correction of Urogenital Sinus in Female Patients with Virilizing Congenital Adrenal Hyperplasia: Are Good Results Possible?
OBJECTIVE
Genital reconstruction in female patients with virilizing congenital adrenal hyperplasia (CAH) is very challenging. Our aim was to evaluate the techniques employed to treat complications after failure of primary urogenital sinus (UGS) surgery, as well as the result of these reoperations.


METHODS
Twenty girls with virilizing CAH who were previously submitted to genitoplasty in our service and elsewhere had recurrent UGS stenosis and vaginal introitus stenosis that required surgical treatment. The main symptoms were recurrent urinary tract infection (UTI) in nine, dyspareunia in six, and hematocolpos in three (two associated with sepsis). The anatomical findings were the persistence of UGS with stenosis in 17 patients and vaginal introitus stenosis in 3. The mean age at procedure was 15.2 years, averaging 13.1 years after the first surgery. The surgical techniques employed were isolated perineal flap in 17 patients and perineal flap with partial mobilization of UGS in 3. The mean follow-up after the procedure was 4.8 years (varying from 1 to 17 years).


RESULTS
Vaginal dilations were performed after surgery in 15 patients. Good functional and anatomical results were obtained in 15 patients, with vaginal introitus amenable to dilators of 3.0 cm in diameter. Five patients with high vaginal insertion had recurrent vaginal stenosis and required a surgical revision. No patients presented menstrual obstruction or UTI after surgery. Eight of the 15 adult patients are sexually active.


CONCLUSIONS
The reoperation to treat failed primary UGS treatment using Y-V flap and partial mobilization techniques associated with vaginal dilations, promoted good anatomical, and functional results with low morbidity in 75% of the patients.
Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil; Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo, São Paulo, Brazil.
Maria Helena Palma
MH
Sircili
Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo , São Paulo , Brazil.
Tania Sartori Sanchez
TS
Bachega
Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo , São Paulo , Brazil.
Guiomar
G
Madureira
Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo , São Paulo , Brazil.
Larissa
L
Gomes
Division of Endocrinology, Hospital das Clínicas, School of Medicine, University of São Paulo , São Paulo , Brazil.
Berenice Bilharinho
BB
Mendonca
Division of Urology, Hospital das Clínicas, School of Medicine, University of São Paulo , São Paulo , Brazil.
Francisco Tibor
FT
Dénes
congenital adrenal hyperplasia; failed feminizing genitoplasty; follow-up; persistent urogenital sinus; reoperation