Tarry W F, Duckett J W, Stephens F D
J Urol. 1986 Sep;136(3):648-52. doi: 10.1016/s0022-5347(17)45004-x.
Agenesis of the vagina in karyotypic female subjects may be accompanied by other defects of the urogenital system. We describe 8 cases that exemplify nearly all variants in the group of müllerian and renal anomalies that we identify as the Mayer-Rokitansky syndrome. We trace the association of system defects to errors of formation of the wolffian body. This structure is the progenitor of the gonad and wolffian duct, which although temporary in the female subject, gives rise to the ureter and is the path finder of the müllerian system. Errors of formation or premature atrophy of the wolffian duct, or intrinsic müllerian organizers lead to the array of anomalies in this syndrome. Vaginal agenesis was found to be associated with müllerian, renal or ovarian defects in numerous embryological combinations. We propose a müllerian classification, and describe the current diagnostic modalities and techniques of surgery.
核型为女性的患者出现阴道发育不全时，可能伴有泌尿生殖系统的其他缺陷。我们描述了8例病例，这些病例几乎涵盖了我们认定为迈耶-罗基坦斯基综合征的苗勒管和肾脏异常组中的所有变异类型。我们追踪了系统缺陷与中肾管形成错误之间的关联。该结构是性腺和中肾管的祖细胞，尽管在女性患者中是暂时的，但它会发育成输尿管，并且是苗勒系统的路径引导者。中肾管形成错误或过早萎缩，或内在的苗勒管组织者异常，会导致该综合征出现一系列异常。研究发现，阴道发育不全与多种胚胎学组合中的苗勒管、肾脏或卵巢缺陷有关。我们提出了一种苗勒管分类方法，并描述了当前的诊断方式和手术技术。
