Disorders of Sex Development Clinical Trial
Official title:
Evaluation of the Outcomes of Feminizing Genitoplasty in Children With Disorders of Sex Development
Evaluate the short term outcomes of feminizing genitoplasty including vaginoplasty,clitroplasty, labioplasty and urethroplasty in children with disorders of sex development as regarding cosmetic and functional aspects.
Disorders of sex development (DSD) are a wide range of congenital conditions characterized by incongruent development of the components involved in sex differentiation including chromosomes, gonads, brain and anatomic sex.Sex assignment is a critical aspect in management of DSD and it is determined by investigations, genital appearance, fertility potential and cultural practices and pressurs. It is recommended that genital surgery for a child raised as a female considered only in cases of severe virilization (Prader 3-5) and that surgery for the clitoris not be performed for reasons of cosmesis only.Feminizing genital reconstruction has evolved considerably over the past decades as a result of improved understanding of anatomy , modification of surgical procedures and audit of outcomes. For patients with congenital adrenal hyperplasia (CAH) with severe virilization raised as a female , it is recommended that clitorial and perineal reconstruction be considered in infancy and those with a low vaginal confluence undergo vaginoplasty at an early age , the appropriate timing for those with higher vaginal confluence is still less certain. Cosmetic outcomes after genitoplasty are variable. Most studies have detected only functional and cosmetic outcomes rated by the physician, but satisfaction ratings may differ between physicians and patients or their parents . The present prospective , interventional study aimed to describe the frequency of sex assignment and types of surgery performed in a cohort of patients with moderate to severe genital ambiguity and also evaluate surgical outcomes of feminizing genitoplasty in these patients . ;
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