Fedele Luigi, Bianchi Stefano, Frontino Giada, Ciappina Nevio, Fontana Eleonora, Borruto Franco
Department of Obstetrics, Gynecology and Neonatology, Fondazione Policlinico-Mangiagalli-Regina Elena, University of Milano, Milan, Italy.
Obstet Gynecol. 2007 May;109(5):1111-5. doi: 10.1097/01.AOG.0000260872.28368.46.
To describe analytically the anatomic variety and laparoscopic findings observed in patients with Rokitansky syndrome throughout an 11-year span.
We analyzed the laparoscopic and chart records of 106 consecutive patients who underwent surgery for the creation of a neovagina, according to the modified laparoscopic Vecchietti procedure.
A hypoplastic vagina was observed in 61 women. Müllerian remnants, laterally displaced in the pelvis, were identified in 92 cases; 42 (25.9%, 95% confidence interval [CI] 19.2-33.3) of 162 müllerian remnants were cavitated and contained endometrial mucosa. Ovaries were extrapelvic in 17 (16.0%, 95% CI 9.6-24.4) cases. Anomalies of the urinary tract were identified in 32 (30.2%, 95% CI 21.7-39.9) patients; unilateral renal agenesis was the most frequent finding (18 cases; 18%, 95%CI 10.4-25.5).
Rokitansky syndrome has a wide variability of anatomic presentations, and, as the do other congenital anomalies of the female genital tract, it represents a continuum of embryonic malformations, which occur at different stages of development.
分析11年间观察到的罗基坦斯基综合征患者的解剖学变异和腹腔镜检查结果。
我们分析了106例连续接受手术以创建新阴道的患者的腹腔镜检查记录和病历，手术采用改良的腹腔镜韦基耶蒂手术。
61名女性存在阴道发育不全。92例患者在盆腔内发现了向侧面移位的苗勒氏管残余；162个苗勒氏管残余中有42个（25.9%，95%置信区间[CI]19.2 - 33.3）呈空洞状并含有子宫内膜黏膜。17例（16.0%，95%CI 9.6 - 24.4）患者的卵巢位于盆腔外。32例（30.2%，95%CI 21.7 - 39.9）患者发现泌尿系统异常；最常见的是单侧肾缺如（18例；18%，95%CI 10.4 - 25.5）。
罗基坦斯基综合征的解剖学表现具有广泛的变异性，与女性生殖道的其他先天性异常一样，它代表了一系列在不同发育阶段发生的胚胎畸形。
