Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review

Objective: To investigate if an asymmetry exists in the lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys. Design: All English-language medical papers on uterus didelphys, obstructed hemivagina, and associated renal agenesis published from 1980 to 2005 and identified by Embase, Medline, and Pubmed database searches were retrieved. In addition, 41 institutional cases are described. We considered articles in which the presence of a uterus didelphys, obstructed hemivagina, and renal agenesis was assessed as well as the affected side. Data were stratified based on surgical confirmation or imaging evidence of the specific müllerian anomaly. Two authors abstracted data independently on standardized forms, and the combined frequency of right- and left-side malformation subtype was computed. Result(s): Thirty-six reports including 138 subjects were selected. Unilateral hemato- or pyocolpos was on the right side in 91 patients (66%). Among the 125 cases with surgical demonstration of the müllerian malformation subtype, 81 (65%) had the anomaly on the right side. In the institutional series, lesions were on the right side in 25 cases (61%). Combining the above figures, the observed proportion of right-sided anomalies (116/179) was 65% (95% CI 57% to 72%). Conclusion(s): Left-right asymmetry may be induced before organogenesis, establishing differences in morphogenesis on the left and right sides of the embryo.