Herlyn–Werner–Wunderlich (obstructed hemivagina and ipsilateral renal anomaly) syndrome presenting in adolescence and adulthood: a comparative case report of two patients with uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly

Abstract Background Herlyn–Werner–Wunderlich (HWW) syndrome, also termed obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare Müllerian-mesonephric malformation defined by the triad of uterus didelphys, unilateral obstructed hemivagina caused by an oblique vaginal septum, and an ipsilateral renal anomaly (most often renal agenesis, but encompassing a spectrum of ipsilateral renal and ureteral anomalies). It classically becomes symptomatic in adolescence, soon after menarche, with cyclic pelvic pain and hematocolpos arising from the obstructed hemivagina, but it may also be recognized only in adulthood after years of nonspecific complaints, endometriosis, infertility, or adverse obstetric outcomes. Direct, side-by-side comparisons of adolescent and adult presentations within the same clinical setting are rarely reported. Case presentation We report two female patients of Brazilian origin with Herlyn–Werner–Wunderlich (OHVIRA) syndrome diagnosed at different stages of life and followed at a single private gynecology service in São Paulo, Brazil, between 2013 and 2025. The first was a 13-year-old adolescent who presented with primary amenorrhea and a 5-month history of severe cyclic pelvic pain. Pelvic magnetic resonance imaging (MRI) demonstrated uterus didelphys with an obstructed left hemivagina due to an imperforate oblique vaginal septum, ipsilateral hematocolpos and hematometra, and ipsilateral (left) renal agenesis. She underwent vaginoscopic resection of the oblique vaginal septum with drainage of the retained blood and complete resolution of symptoms; oral micronized progesterone was started to prevent re-stenosis. The second patient was referred at 27 years of age and diagnosed at 39, with chronic pelvic pain, dysmenorrhea, dyspareunia, two preterm deliveries at 32 weeks gestation (each in a separate uterine horn) and a previously corrected obstructed right hemivagina. MRI showed two uterine bodies, initially interpreted as a bicornuate uterus, with adenomyosis, deep infiltrating endometriosis, and a solitary left ureter, with the right ureter not visualized on imaging, indicating an ipsilateral (right) urinary tract anomaly. She underwent total laparoscopic hysterectomy, bilateral salpingectomy and excision of endometriotic lesions; macroscopic and histopathological examination of the surgical specimen confirmed uterus didelphys. Combined with the previously treated obstructed hemivagina and the ipsilateral urinary tract anomaly, the findings established the diagnosis of Herlyn–Werner–Wunderlich (OHVIRA) syndrome. Postoperatively, she reported substantial improvement in pain and quality of life. Conclusions These two cases highlight the broad phenotypic spectrum and life-stage-dependent presentation of Herlyn–Werner–Wunderlich (OHVIRA) syndrome, the diagnostic limitations of MRI in distinguishing uterus didelphys from bicornuate uterus, and the value of the ipsilateral renal anomaly as a diagnostic clue to the underlying Müllerian-mesonephric defect. Early imaging diagnosis, prompt relief of the vaginal obstruction, individualized surgical planning and longitudinal follow-up were associated with favorable outcomes in both patients, although two cases cannot establish practice standards.