Gender reversal in 46XX congenital virilizing adrenal hyperplasia.
OBJECTIVE
To review the results of gender reversal in six patients with 46XX congenital virilizing adrenal hyperplasia (CVAH).


METHODS
Fifty-one patients with 46XX CVAH were seen in an 8 year period; 45 were managed by conventional feminizing genitoplasty, but six underwent gender reversal and were managed as males. The clinical decision for gender reversal was made after appropriate counselling and was based primarily on parental choice, this being influenced significantly by a delayed diagnosis in four patients. Surgical management consisted of gonadectomy, excision of Müllerian structures and staged hypospadias repair/ chordee correction in four patients, and circumcision in two completely masculinized children.


RESULTS
All six boys are well adjusted to their gender of rearing, with ages ranging from 3 years to 16.5 years (mean 8.5) at the time of review. Two children have normal penises and four have a satisfactory result after two-stage repair of hypospadias/chordee.


CONCLUSIONS
Most patients with 46XX CVAH are preferably raised as females and require a feminizing genitoplasty. However, the clinical decision may be influenced by many factors, including delay in diagnosis, social bias and the premium on male rearing in certain communities. When male rearing is chosen, early gonadectomy and excision of Müllerian structures, together with staged hypospadias repair, gives satisfactory results.
Sripathi
V
V
Department of Surgery, King Faisal Specialist Hospital and Research Centre, Riyadh, Saudi Arabia.
Ahmed
S
S
Sakati
N
N
al-Ashwal
A
A
D000293:Adolescent; D000312:Adrenal Hyperplasia, Congenital / Q000235:genetics / Q000601:surgery*; D002648:Child; D002668:Child Rearing; D002675:Child, Preschool; D002755:Choice Behavior; D003376:Counseling; D003657:Decision Making; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D010375:Pedigree; D014770:Virilism / Q000235:genetics / Q000601:surgery*