OHVIRA Syndrome with Deep Pelvic Endometriosis and Cervical Carcinoma in a Reproductive-Age Woman: A Rare Case Report

OHVIRA syndrome is a rare Mullerian duct anomaly characterised by uterus didelphys, obstructed hemivagina, and ipsilateral renal anomaly, usually diagnosed in adolescence because of dysmenorrhea, pelvic pain, or a pelvic mass. Delayed diagnosis may lead to retrograde menstruation, hematosalpinx, and endometriosis, while associated cervical malignancy is exceptionally uncommon. Case Presentation This report describes a 34-year-old woman who presented with brownish vaginal discharge and was found to have OHVIRA syndrome complicated by deep pelvic endometriosis (DPE) and squamous cell carcinoma of the cervix. Pelvic examination showed a tender left-sided vaginal bulge. Contrast-Enhanced Magnetic Resonance Imaging (CEMRI) demonstrated uterine duplication, left hematometrocolpos, bilateral hematosalpinx, sigmoid endometriotic deposit, cervical growth with parametrial extension, pelvic nodal disease, and an absent left kidney. Septal incision and drainage exposed a necrotic cervical lesion, and histopathology confirmed squamous cell carcinoma. Since pelvic lymphadenopathy indicated FIGO stage IIIC1 disease, the patient was referred for primary chemoradiotherapy instead of upfront surgery.

This case highlights the value of early MRI in obstructive Müllerian anomalies and the importance of evaluating for advanced endometriosis and rare coexisting malignancy. Similar content being viewed by others Data Availability Data sharing does not apply to this article, as no datasets were generated or analysed in the current study.

Tanase Y, Yoshida H, Naka T, Kitamura S, Natsume T, Kobayashi Kato M, et al. Clear cell carcinoma of the cervix with OHVIRA syndrome: a rare case report. World J Oncol. 2021;12:34–8. Takahashi N, Harada M, Kanatani M, Wada-Hiraike O, Hirota Y, Osuga Y. The association between endometriosis and obstructive müllerian anomalies. Biomedicines. 2024;12:651. Kriplani A, Dalal V, Kachhawa G, Mahey R, Yadav V, Kriplani I. Minimally invasive endoscopic approach for management of OHVIRA syndrome. J Obstet Gynaecol India. 2019;69:350–5. Lorusso F, Scioscia M, Rubini D, Stabile Ianora AA, Scardigno D, Leuci C, et al. Magnetic resonance imaging for deep infiltrating endometriosis: current concepts, imaging technique and key findings. Insights Imaging. 2021;12:105. Funding No funding was obtained for this study. Author information Authors and Affiliations Contributions All authors contributed to the study conception and design. All authors prepared the material, collected the data, and analysed the data. All authors contributed to writing the first draft of the manuscript and commented on previous versions. All authors read and approved the final manuscript. Corresponding author Ethics declarations Conflict of interest The authors declare no conflicts of interest. Consent to Participate Written informed consent was obtained from the patient. Consent to Publish Written informed consent for publication of their clinical details and/or clinical images was obtained from the patient. Ethical Approval IEC approval was not required. There are no ethical issues. Additional information Publisher's Note Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. Disclaimer: The views and opinions expressed in this article are those of the authors and are the product of professional research. They do not necessarily reflect the official policy or position of any affiliated institution, funder, agency, or the publisher. The authors are responsible for the results, findings, and content of this article. OHVIRA syndrome with deep pelvic endometriosis and cervical carcinoma in a reproductive-age woman: a rare case report. Ajith Kumar P is a Junior Resident; Rajlaxmi Mundhra is an Additional Professor; Priyanka Pawar is a Senior Resident; Poonam Gill is an Assistant Professor; and Rahul Dev is an Assistant Professor Rights and permissions Springer Nature or its licensor (e.g. a society or other partner) holds exclusive rights to this article under a publishing agreement with the author(s) or other rightsholder(s); author self-archiving of the accepted manuscript version of this article is solely governed by the terms of such publishing agreement and applicable law. About this article Cite this article P., A.K., Mundhra, R., Pawar, P. et al. OHVIRA Syndrome with Deep Pelvic Endometriosis and Cervical Carcinoma in a Reproductive-Age Woman: A Rare Case Report. J Obstet Gynecol India (2026). https://doi.org/10.1007/s13224-026-02417-4 Received: Accepted: Published: Version of record: DOI: https://doi.org/10.1007/s13224-026-02417-4