Rekonstrukcja zewnętrznych narządów płciowych w kierunku żeńskim u dzieci z zaburzeniami różnicowania płci / Feminizing genitoplasty in children with disorders of sex development
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Abstract:
In the group of children with disorders of sex development (DSD) feminizing genitoplasty is mainly performed in patients with congenital adrenal hyperplasia
(CAH). Feminizing genitoplasty is rarely performed in other forms of DSD like ovotesticular DSD or DSD with mosaic kariotype. In patiens with CAH virilization
of external genitalia is caused by enzymatic block in steroidogenesis resulting in androgen overproduction. After birth it is crucial to stabilize the
patient and to start hormonal treatment. Feminizing genitoplasty is performed between 6-12 month of life. Reconstruction procedure consists of: urethroplasty,
vaginoplasty, clitoroplasty and labioplasty. Cosmetic and functional results are good but some patients may require reoperation of a narrow vaginal
outlet at pubertal age.
Standardy Medyczne/Pediatria 2021 T. 18 484-489
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