Spontaneous Urinary Bladder Rupture Secondary to OHVIRA Syndrome

As a rare mesonephric duct-induced Mullerian anomaly, OHVIRA syndrome is characterised by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly. Equally rare is the spontaneous rupture of the urinary bladder (SRUB), a condition that constitutes a life-threatening surgical emergency. This case represents an unprecedented presentation of OHVIRA syndrome involving SRUB. A 19-year-old female presented with acute abdomen superimposed on a long-standing progressive dysmenorrhea and recent dysuria. Diagnosis of OHVIRA syndrome was confirmed by imaging; however, SRUB was only discovered intraoperatively during a pre-procedure cystoscopy, just prior to the planned vaginoscopic septal resection. An emergency laparoscopic bladder repair was performed to treat the intraperitoneal bladder rupture. Identification of both OHVIRA and SRUB is often missed or significantly delayed. To overcome this, adolescents presenting with progressive dysmenorrhea since menarche should be investigated for unilateral obstructive Mullerian anomalies including OHVIRA syndrome. Furthermore, a new onset of urinary symptoms and acute abdominal pain suggestive of peritonitis should raise the suspicion of SRUB. In the era of minimally invasive surgery, combined vaginoscopic septoplasty and laparoscopic bladder repair should be considered the primary, least traumatic and most effective management.