Treatment management of childrens with sexual development disorders

Aim: The aim of this study is to evaluate the diagnostic and therapeutic applications of the patients with sexual development disorders, who were followed at Çukurova University Faculty of Medicine, Department of Pediatric Surgery, between 2002 and 2009.Patients and Methods: We analyzed the files of patients retrospectively by taking into account their age of admission, reason of application, findings of physical examination, diagnosis, laboratory results, chromosome analysis, ultrasonography, magnetic resonance imaging, genitogram, endoscopic findings, gonad biopsies, decisions of `gender research council? and surgical treatments.Results: The average age of 36 patients was 39 months. Of the patients 66.6% was younger than the age of two. The most frequent reason of applications was gender ambiguity and more than half of the patients were female pseudo hermaphrodite (61.1%) and others were male pseudo hermaphrodite (33,33%) and mixed gonadal dysgenesis (5.55%). Etiological cause was 21-OH deficiency at the 95.4% of female pseudo hermaphrodite patients. Etiology was unknown at the 58.3% of male pseudo hermaphrodite patients. Genitogram and endoscopical examinations were useful diagnostic methods. First name of the 22.2% of patients was changed. Average age of the female corrective surgery was 44.7 months. Of these patients 47% was operated before two-year-age and 12 of them (33.3%) were operated as single stage clitero-vaginoplasty. Due to high type of vaginal opening, among 27 patients whom their gender was decided as girl, five of them had two-stage procedure: Cliteroplasty was performed and vaginoplasty was left to adolescence.Conclusion: Gender of the patients with sexual development disorders is decided with a multidisciplinary series of laboratory and imaging study, in cooperation with the parents. Timing of surgery debatable but results of early surgery for short urogenital sinus and low vaginal opening is favorable.Keywords: Ambiguous genitale, disorders of sexual development, intersex, congenital adrenal hyperplasia, vaginoplasty.