Colacurci N, Cardone A, De Franciscis P, Landolfi E, Venditto T, Sinisi A A
Institute of Gynaecology and Obstetrics, Second University of Naples, Italy.
Hum Reprod. 1997 Feb;12(2):272-4. doi: 10.1093/humrep/12.2.272.
We describe laparoscopic diagnosis and treatment for a case of dysgenetic male pseudohermaphroditism with persistent Mullerian ducts. The patient, a 32 year old man, with a history of surgery for hypospadias and cryptorchidism during childhood, was referred because of anejaculation. He was of short stature, with male external genitalia composed of a small penis and hypoplastic testis (1 ml right, 6 ml left side). Plasma follicle stimulating hormone (FSH) was high (17 mUI/ml), testosterone low (1.9 ng/ml), and his karyotype was 46,XY. Pelvic ultrasound, nuclear magnetic resonance (NMR) and genitography disclosed a uterine-like structure with cavity communicating with the urethra. Laparoscopy and urethrocystoscopy confirmed the presence of a 4 cm uterus, which was removed endoscopically at the same time. A biopsy of the left gonad was also performed. The uterus contained endometrial tissue and was fibrotic. Histology of the left gonad showed spermatocytic arrest. We diagnosed dysgenetic male pseudohermaphroditism. Laparoscopy, in our opinion, is an optimal tool to diagnose and treat abnormal sexual conditions.
我们描述了一例患有持续性苗勒管的发育不全性男性假两性畸形的腹腔镜诊断和治疗。患者为一名32岁男性，童年时有尿道下裂和隐睾手术史，因无射精前来就诊。他身材矮小，男性外生殖器由小阴茎和发育不全的睾丸组成（右侧1毫升，左侧6毫升）。血浆促卵泡激素（FSH）升高（17 mUI/ml），睾酮降低（1.9 ng/ml），其核型为46,XY。盆腔超声、核磁共振（NMR）和生殖系统造影显示一个类似子宫的结构，其腔与尿道相通。腹腔镜检查和尿道膀胱镜检查证实存在一个4厘米的子宫，同时在内镜下将其切除。还对左侧性腺进行了活检。子宫内含有子宫内膜组织且纤维化。左侧性腺组织学显示生精细胞停滞。我们诊断为发育不全性男性假两性畸形。我们认为，腹腔镜检查是诊断和治疗异常性状况的最佳工具。
