Ludwig K S
Department of Anatomy, University of Basel.
Arch Gynecol Obstet. 1998;262(1-2):27-42. doi: 10.1007/s004040050225.
The Müllerian duct (MD; ductus paramesonephricus) develops independent of the coelomic epithelium above the mesonephros. This part of the duct gives rise to the infundibulum with its fimbriated ostium abdominale. The part of the duct which lies along the mesonephros as far as its caudal pole makes a contribution to the ampulla and less often the isthmus. In the area of the mesonephros the MD fuses with the Wolffian duct (WD; ductus mesonephricus). The WD gives rise to the ampulla and the isthmus. Below the caudal pole of the mesonephros, as well as beyond the attachment point of the inguinal ligament of the mesonephros, the later round ligament of the uterus, the MD develops as an outgrowth of the WD and no longer as an independent structure. The MRK syndrome is, in its formal genesis, a non-fusion of the MD with the WD. This explains the fact that in a classic case of MRK syndrome, the Fallopian tube with a very small part of the cornu uteri extends only as far as the connection with the round ligament of the uterus. Different possibilities for the origin of MRK syndrome are discussed. It is suggested that the cause of the development of MRK syndrome could be a deficiency of gestagen and/or oestrogen receptors. This would also explain the various forms of the rudimentary vagina.
苗勒管(MD;副中肾管)在中肾上方独立于体腔上皮发育。该管的这一部分产生带有具纤毛的腹侧开口的漏斗部。沿着中肾直至其尾极的那部分管道对壶腹部有贡献,对峡部的贡献较少。在中肾区域,苗勒管与沃尔夫管(WD;中肾管)融合。沃尔夫管产生壶腹部和峡部。在中肾尾极下方以及中肾腹股沟韧带附着点之外,即后来的子宫圆韧带处,苗勒管作为沃尔夫管的一个分支发育,不再是一个独立结构。苗勒管发育不全综合征在其形式发生上是苗勒管与沃尔夫管未融合。这解释了在典型的苗勒管发育不全综合征病例中,输卵管连同子宫角的一小部分仅延伸至与子宫圆韧带相连处这一事实。文中讨论了苗勒管发育不全综合征起源的不同可能性。有人提出,苗勒管发育不全综合征发生的原因可能是孕激素和/或雌激素受体缺乏。这也可以解释发育不全阴道的各种形式。