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.

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.