Ten-day-old female referred to a pediatric urology consultation due to prenatal diagnosis of left renal agenesis and a pelvic cystic image, which raised the suspicion of a nephro-urologic malformation.

At birth, analytically with normal values of Urea (34 mg/dl) and Creatinine (1.38 mg/dl). Ultrasound in the first week of life confirmed a hydrohematocolpos, showing a pelvic, non-pure, cystic image (Fig. 1), identified two hemi-uterus suggesting a didelphys uterus and a tubular image with bladder insertion adjacent to the left uterus, interpreted as a ureteral remnant.

Figure 1: Pelvic ultrasound (longitudinal) showing the blader (B), the left hemi-uterus (U) and a hematocolpos of heterogenous content in the obstructed hemi-vagina (H). 

Hydrohematocolpos was drained to prevent infection. Magnetic resonance imaging was performed at 7 months of age, confirming left renal agenesis, a left ureteral remnant with ureterocele (Fig. 2) and a didelphys uterus (Fig. 3).

Figure 2: Coronal T2-weighted image demonstrating the presence of a right kidney (blue circle), absence of the left kidney (white circle), a left ureteral remnant (yellow arrow) and an ureterocele (red arrow). 

Figure 3: Axial T2-weighted image showing two hemi-uterus (arrows). 

Currently at 2 years-old, she remains asymptomatic and maintaining regular follow-ups with urologic pediatricians. OHVIRA (Obstructed hemivagina and ipsilateral renal anomaly) syndrome, also known by Herlyn-Werner- Wunderlich syndrome, is a congenital anomaly of the female urogenital tract resulting from an anomaly of the Mullerian and mesonephric ducts. It is characterized by a didelphys uterus with an obstructed/blind hemi-vagina and ipsilateral renal agenesis¹ accounting for 0.16-10% of Mullerian duct malformations (MDM).

Patients are usually asymptomatic until puberty, when they begin to have dysmenorrhea and cyclic pelvic pain due to hematometrocolpos, as a consequence of obstructed hemi- vagina. In the postnatal period and early infancy, the same symptoms may occur due to the influence of maternal hormones.

Acute complications include pyohematocolpos and pyosalpinx, and long-term complications include endometriosis, pelvic inflammatory disease, and infertility.¹ Differential diagnosis includes others MDM (bicornuate uterus, septate uterus), imperforate hymen and transverse vaginal septum.

Treatment is symptomatic, with vaginal septotomy for hematocolpos drainage. Hemi-hysterectomy is not indicated.²,³

As there is a strong association of female genital tract and renal anomalies, when the patient presents with one of these, a screening for associated anomalies should be performed.