Incomplete reduplication of internal genitalia and unilateral renal aplasia syndrome

Abstract

From 1973 through 1987, 19 girls with malformation of the urogenital drainage system were observed, treated, and followed-up. Each had a double uterus: one uterus emptied into a normally developed vagina; the other either ended blindly (hemihematometra, 8 cases) or led into an atretic vagina (hemihematocolpos, 11 cases). On the non-communicating side, the kidney and ureter were undeveloped, probably due to nondevelopment of the mesonephric (wolffian) duct on the affected side. The proper diagnosis (using x-ray, ultrasonography, and biopsy) is very important for surgical correction. When the hemihematocolpos was present, establishing a wide communication between the two vaginas was always sufficient. Extirpation of the uterus in these cases was not the treatment of choice. In hemihematometra, extirpation of the uterus on the atretic side was required in three cases. Several patients became pregnant and delivered healthy children.