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 mullerian 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 mullerian system. Errors of formation or premature atrophy of the wolffian duct, or intrinsic mullerian organizers lead to the array of anomalies in this syndrome. Vaginal agenesis was found to be associated with mullerian, renal or ovarian defects in numerous embryological combinations. We propose a mullerian classfication, and describe the current diagnostic modalities and techniques of surgery.
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