[Sex of rearing in 46,XY partial gonadal dysgenesis (case report)].
XY patients with gonadal dysgenesis present with a wide array of clinical pictures that is characterised by a variable incomplete virilisation of the external genital organs (female phenotype, posterior hypospadias, cryptorchidism) and the persistence of the internal müllerian duct structures. The birth of a child with this type of abnormality is a social phenomenon, which will probably have a physical and psychical impact. The choice of the sex depends on the organic anatomy, diagnosis age, risk of gonadal tumors and the development possibilities (mainly at puberty) of the child. The registration of the civil status can be made only after determining the sex through the aforementioned anatomical and functional study. Once this process is finished, the treatment must be considered. We report our experience, particularly complicated in the choice of the sex of child, with an XY karyotype and partial gonadal dysgenesis. Raised as a girl until her seventh day when her parents noticed the existence of a genital bud and decided to register their child as a boy in the civil status. He was referred to our hospital, at the age of 16 months, in order to explore a bilateral cryptorchidism and posterior hypospadias. Our patient was declared to be a boy based on an unanimous opinion of a multidisciplinary team.
Iraqi
N
N
Service d'endocrinologie diabétologie, maladies métaboliques et nutrition, CHU Rabat, Rabat, Morocco. nezhairaqi@gmail.com
Gaouzi
A
A
Bouhafs
M A
MA
D003456:Cryptorchidism / Q000473:pathology; D006061:Gonadal Dysgenesis, 46,XY / Q000175:diagnosis / Q000235:genetics / Q000473:pathology*; D006801:Humans; D007021:Hypospadias / Q000473:pathology; D007223:Infant; D008297:Male; D010413:Penis / Q000473:pathology; D010641:Phenotype; D012727:Sex Characteristics