Ludwig K S
Department of Anatomy, University of Basel.
Arch Gynecol Obstet. 1998;262(1-2):1-26. doi: 10.1007/s004040050224.
The histological appearance of the rudimentary uterus, endometrium, uterine tube, Gartner's duct, round ligament, vagina and ovary is described in ten case of the MRK syndrome. When the layout of the pelvic contents of patients with this syndrome is compared with that of a normal embryo of 20 mm CRL (stage 20), the following conclusions may be drawn. The MRK syndrome can be regarded as resulting from the cessation of development of the Müllerian duct, which in this condition extends only as far as its attachment to the caudal mesonephric ligament, the round ligament. The adjacent structures beyond this point, which include the connecting strand and smooth muscle bundles dorsal to the bladder and vaginal rudiment, are derivatives of the Wolffian duct or Gartner's duct respectively. Our findings suggest that this syndrome is due to a deficiency of the estrogen and gestagen receptors. This deficiency may inhibit the further development of the embryonic Müllerian duct and account for the subsequent faulty differentiation of its existing elements. It is still undecided why, in cases of the MRK syndrome, development of the Müllerian duct ceases at the attachment of the caudal mesonephric ligament (later the round ligament). Further research is necessary to answer this question.
本文描述了10例苗勒管发育不全综合征（MRK综合征）患者的始基子宫、子宫内膜、输卵管、加特内管、圆韧带、阴道及卵巢的组织学表现。将该综合征患者盆腔脏器的布局与头臀长20mm（第20期）正常胚胎的布局进行比较后，可得出以下结论。MRK综合征可被视为苗勒管发育停止所致，在此情况下，苗勒管仅延伸至其与尾侧中肾韧带（即圆韧带）的附着处。该附着点以外的相邻结构，包括连接索以及膀胱和阴道始基背侧的平滑肌束，分别是中肾管或加特内管的衍生物。我们的研究结果表明，该综合征是由于雌激素和孕激素受体缺乏所致。这种缺乏可能会抑制胚胎苗勒管的进一步发育，并导致其现有结构随后出现分化异常。目前仍不清楚为何在MRK综合征病例中，苗勒管的发育会在尾侧中肾韧带（即后来的圆韧带）的附着处停止。需要进一步研究来回答这个问题。
