[Adrenogenital syndrome: feminizing genital reconstruction].
Adrenogenital syndrome, or so called congenital adrenal hyperplasia, is caused by a congenital insufficiency of the enzyme 21-hydroxylase, which is responsible for converting cortisol into cholesterol. Because of virilizing effect of androgens overproduction girls develop clitoral hypertrophy and persistent urogenital sinus (common channel for urethra and vagina). Surgical treatment is recommended in order to repair those developmental faults. The aim of this study was to employ the contemporary surgical techniques and to evaluate the postoperative results. Forty-seven patients affected by adrenogenital syndrome were investigated and treated at two institutions: Departments of Pediatric Surgery of Copenhagen University Hospital and Kaunas Medical University Hospital. Forty-three patients have been operated and underwent genitoplasty. Surgical method was chosen individually depending on the height of the urogenital sinus. In a case of low sinus a simple cut-back procedure was performed. In a case of high sinus the more complex procedure such as total urogenital mobilization or vaginal pull through would be involved. All patients underwent vaginal dilatations for 6-12 months postoperatively. Twenty-eight patients underwent clitoroplasty while the glans and the neurovascular bundle were preserved and clitoral skin used for plasty of the labia minora. Postoperatively the patients were observed for 0.5-5 years, the close results showed to be good. There were 4 cases of vaginal stenosis and 2 cases of urethrovaginal fistula (all successfully repaired later). Early one staged genitoplasty and postoperative vaginal dilatations for the period of 6-12 months is recommended.
Zaparackaite
Indre
I
Kauno medicinos universiteto Vaiku chirurgijos klinika, Eiveniu 2, 3007 Kaunas.
Barauskas
Vidmantas
V
Nielsen
Ole Henrik
OH
Jokela
Riitta
R
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery*; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D002987:Clitoris / Q000601:surgery; D004106:Dilatation; D005260:Female; D005500:Follow-Up Studies; D005836:Genitalia, Female / Q000601:surgery*; D006801:Humans; D007223:Infant; D011182:Postoperative Care; D011446:Prospective Studies; D019651:Plastic Surgery Procedures; D013997:Time Factors; D014566:Urogenital System / Q000196:embryology; D014621:Vagina / Q000601:surgery