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Gender reversal in 46XX congenital virilizing adrenal hyperplasia
Author(s) -
SRIPATHI V.,
AHMED S.,
SAKATI N.,
ALASHWAL A.
Publication year - 1997
Publication title -
british journal of urology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.773
H-Index - 148
eISSN - 1464-410X
pISSN - 0007-1331
DOI - 10.1046/j.1464-410x.1997.00127.x
Subject(s) - congenital adrenal hyperplasia , hyperplasia , endocrinology , medicine , disorders of sex development
Objective To review the results of gender reversal in six patients with 46XX congenital virilizing adrenal hyperplasia (CVAH). Patients and 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. Conclusion 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.