Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos

Herlyn-Werner-Wunderlich syndrome: a rare presentation with pyocolpos DOI: https://doi.org/10.3941/jrcr.v6i3.877Keywords: Herlyn-Werner-Wunderlich syndrome, OHVIRA, Mullerian duct anomalies, didelphic uterusAbstract Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn-Werner-Wunderlich syndrome, is a rare syndrome with only a few hundred reported cases described since 1922. Only a handful of these cases have been associated with pyocolpos. Mullerian duct anomalies have an incidence of 2-3%. While OHVIRA constitutes 0.16-10% of these Mullerian duct anomalies. Symptoms usually present shortly after menarche when hematocolpos develops during menstruation resulting in dysmenorrhea and a pelvic mass. The pelvic mass is the collection of blood products within the obstructed hemivagina. The first study in the diagnostic work-up is usually ultrasonography, which typically demonstrates a pelvic fluid collection which can simulate other disease processes thus confounding the diagnosis. MRI findings of the pelvis reveal a didelphic uterus. Imaging of the abdomen reveals agenesis of the ipsilateral kidney. MRI is beneficial in characterizing the didelphic uterus and vaginal septum for pre-operative planning. Understanding the imaging findings, in conjunction with the clinical presentation, is critical for early diagnosis in attempting to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility. Downloads Published Issue Section License The publisher holds the copyright to the published articles and contents. However, the articles in this journal are open-access articles distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 License, which permits reproduction and distribution, provided the original work is properly cited. The publisher and author have the right to use the text, images and other multimedia contents from the submitted work for further usage in affiliated programs. Commercial use and derivative works are not permitted, unless explicitly allowed by the publisher.