Surgical options in disorders of sex development (dsd) with ambiguous genitalia.
Disorders of sexual development (DSD) include three main groups of patients: (1) The virilised 46,XX DSD essentially represented by congenital adrenal hyperplasia (CAH) ; (2) The undervirilised 46,XY DSD essentially represented by hypospadias; and (3) the chromosomic jigsaws essentially represented by mixed gonadal dysgenesis. It is in this last group that gender assignment remains a difficult decision involving various indicators, which can be split into four categories: (1) the inside sex (i.e., genes, hormones and target tissues); (2) the outside sex (i.e., anatomy of genitalia including size of the genital tubercle, mullerian cavity and potential adult height of the patient); (3) the functional sex (i.e., potential sexuality and fertility); and (4) and the social sex (i.e., the cultural medium in which the child is brought up). The challenge is to outline the future individual identity of the child in the postnatal period using these indicators. Current evolutions of surgical techniques of 'feminisation' and 'masculinisation' are described as well as their outcomes.
Vidal
Isabelle
I
Department of Paediatric Urology and Surgery, Hôpital Mère-Enfants, and Claude-Bernard University, 69677 Bron, France.
Gorduza
Daniela Brindusa
DB
Haraux
Elodie
E
Gay
Claire-Lise
CL
Chatelain
Pierre
P
Nicolino
Marc
M
Mure
Pierre-Yves
PY
Mouriquand
Pierre
P
D000312:Adrenal Hyperplasia, Congenital / Q000601:surgery; D000328:Adult; D013734:Androgen-Insensitivity Syndrome / Q000188:drug therapy / Q000601:surgery; D002648:Child; D003456:Cryptorchidism / Q000601:surgery; D012734:Disorders of Sex Development / Q000601:surgery*; D005260:Female; D005835:Genitalia / Q000002:abnormalities* / Q000601:surgery; D023961:Gonadal Dysgenesis, 46,XX / Q000601:surgery; D006061:Gonadal Dysgenesis, 46,XY / Q000601:surgery; D006060:Gonadal Dysgenesis, Mixed / Q000601:surgery; D006801:Humans; D007021:Hypospadias / Q000601:surgery; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D010413:Penis / Q000601:surgery; D046468:Sexual Development; D014521:Urethra / Q000601:surgery