Pellerin D, Nihoul-Fékété C, Lortat-Jacob S
Bull Acad Natl Med. 1989 May;173(5):555-62; discussion 563.
From 1955 to 1988, 298 children suffering of ambiguous genitalia were operated upon in the Department of Pediatric Surgery, Hospital Necker-Enfants Malades, Paris. 187 were female pseudo-hermaphroditism (of which 85% congenital adrenal hyperplasia). 53 gonadal dysgenesis and 58 male pseudohermaphroditism--Feminizing genitoplasty, including relocation of the clitoris, published by us, in 1961, was made in 238 cases. It was for a long time the best procedure used, whatever be the etiology of intersex, because the results of masculinizing procedures to be used were poor. Using free bladder mucosa graft is a progress which allows an efficient one stage masculinizing genitoplasty, we have made 31 cases of it. Early and precise diagnosis, early medical treatment and psychological approach of intersex by a multidisciplinary team and adequate precocious one stage surgical repair of ambiguous genitalia, have dramatically improved this condition. Prenatal diagnosis of C21 hydroxylase deficiency and preventive control of adrenal hyperplasia of the foetus, by the supply of gluco-corticoïds to the mother in "a risk situation", will in the future allow to prevent virilisation of female foetuses--Surgery will become useless.
1955年至1988年期间，巴黎内克尔儿童医院小儿外科为298例生殖器模糊的儿童实施了手术。其中187例为女性假两性畸形（其中85%为先天性肾上腺皮质增生），53例为性腺发育不全，58例为男性假两性畸形。1961年我们发表的包括阴蒂复位在内的女性化生殖器成形术应用于238例患者。长期以来，无论两性畸形的病因如何，该手术都是最佳术式，因为当时采用的男性化手术效果不佳。使用游离膀胱黏膜移植是一项进步，它可实现高效的一期男性化生殖器成形术，我们已开展了31例。早期精确诊断、早期药物治疗、多学科团队对两性畸形的心理干预以及对生殖器模糊进行充分的早期一期手术修复，显著改善了这种情况。通过在“高危情况”下给母亲补充糖皮质激素来进行21 - 羟化酶缺乏的产前诊断和胎儿肾上腺增生的预防性控制，未来将有可能预防女性胎儿男性化——手术将变得不再必要。
