Endometriosis Coinciding with Uterus Didelphys and Renal Agenesis: A Literature Review

Abstract

Background/Objectives: Endometriosis and urogenital malformation with uterus didelphys and renal agenesis might occur concomitantly, and the question arises whether both entities are associated with each other. Methods: A literature search was conducted in PubMed and Web of Science, using the following search terms: “endometriosis and uterine malformation, endometriosis and Herlyn–Werner–Wunderlich syndrome”, “endometriosis and OHVIRA (Obstructed Hemivagina and Ipsilateral Renal Anomaly) syndrome” and “uterus didelphys, renal agenesis and endometriosis”. Results: We identified and examined 36 studies, comprising a total of 563 cases with coinciding endometriosis and OHVIRA. The most prevalent symptoms were dysmenorrhea and lower abdominal pain. Renal agenesis occurred more frequently on the right side. In the majority of cases, vaginal septum resection was performed to alleviate hematometrocolpos. Among the 97 cases necessitating abdominal exploration, endometriosis was identified in 61 patients (62.9%), although this figure is most likely an overestimation. However, a significantly heightened risk of endometriosis was evident. Conclusions: This literature review highlights the importance of considering the potential for urogenital malformation and endometriosis in cases of dysmenorrhea during adolescence. Ultrasound examination has proven to be a valuable diagnostic tool for identifying uterine abnormalities and guiding subsequent diagnostic and, if necessary, surgical interventions. Thorough assessment and appropriate management are imperative to mitigating the long-term consequences associated with deep infiltrating endometriosis.