EC Gynaecology

OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly), also called Herlyn-Werner-Wunderlich syndrome, is defined by the triad of a duplicated uterus, an obstructing longitudinal vaginal septum, and ipsilateral renal agenesis; it reflects the coupled embryologic development of the Müllerian and Wolffian systems in early gestation [1,2]. During weeks ~6-12, the mesonephric (Wolffian) duct both induces the ureteric bud (future kidney/ureter) and serves as a guide for lateral migration/fusion of the paramesonephric (Müllerian) ducts; ipsilateral mesonephric duct maldevelopment can therefore produce renal agenesis and simultaneously disrupt Müllerian fusion, yielding a complete bicorporeal uterus, while failure of canalization or resorption of the distal Müllerian/vaginal segment leaves a longitudinal septum that may be obstructing [1,2,9]. In contemporary reporting, the uterine, cervical, and vaginal compartments are coded separately using the ESHRE/ESGE system; the classic OHVIRA configuration corresponds to U3b C2 V2 (complete bicorporeal uterus with double cervix and an obstructing longitudinal vaginal septum) [3]. Obstruction on one side leads to retained menstrual blood (hematocolpos/±hematometra), cyclical pain, and-when diagnosis is delayed-retrograde spill with an increased risk of endometriosis and infection, phenomena repeatedly described in reviews and series [1,4,10].

Keywords: Uterus Didelphys; MRI; Müllerian Duct

1. Moufawad G., et al. “Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome: systematic review”. Gynecol Minim Invasive Ther 3 (2023): 123-129.
2. Herlyn–Werner–Wunderlich (OHVIRA) syndrome (2025).
3. Grimbizis GF., et al. “ESHRE/ESGE consensus on classification of female genital tract congenital anomalies”. Human Reproduction8 (2013): 2032-2044.
4. Kudela G., et al. “Multiple variants of OHVIRA: single-center series and systematic review of 734 cases”. Journal of Pediatric Urology 5 (2021): 653.e1-653.e9.
5. Borges AL., et al. “OHVIRA/Herlyn-Werner-Wunderlich syndrome: review”. Radiology Case Reports 8 (2023): 2771-2784.
6. Tanaka YO., et al. “Uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis: MR findings in seven cases”. Abdominal Radiology 4 (1998): 437-441.
7. Cho YH., et al. “MRI features of OHVIRA and clinical implications”. Investigative Magnetic Resonance Imaging 3 (2015): 196-199.
8. Paudel S., et al. “OHVIRA imaging case series: uterine/vaginal mapping and urinary variants”. Annals of Medicine and Surgery (London)6 (2023): 3149-3154.
9. Del Vescovo R., et al. “Herlyn–Werner–Wunderlich syndrome: MRI findings and radiologic roadmap”. BMC Medical Imaging 12 (2012): 4.
10. Martini N., et al. “Management outcomes in OHVIRA: risks of delayed diagnosis and post-treatment prognosis” (2023).