Case

A 12-year-old girl, with an irrelevant medical history, was admitted to the pediatric emergency department with pollakiuria and abdominal and genital pain for two weeks, with worsening in the day before admission. There was a reference to menarche in the six months before, with scarce catamenia and intense dysmenorrhea refractory to NSAIDs. Last catamenia had been two weeks prior to the admission. On physical examination, the lower abdominal quadrants were rigid and painful on palpation and decompression, mainly in the right iliac fossa. An analytical study, urinalysis and pregnancy test were performed, all negative. Therefore, she did an abdominal ultrasound which suggested an hematocolpus (Fig. 1), but could not correctly evaluate the uterus. To better characterization, an abdominal and pelvic computized tomography was carried out which revealed the absence of the right kidney and retroaortic left renal vein, duplication of the uterus, cervix, and vagina, and marked distension of the right hemivagina and endometrial cavity, with hematocolpos, suggesting vaginal obstruction (Fig. 2). Afterwards, she did a renal scintigraphy for a renal morphofunctional assessment, which confirmed a right renal agenesis (Fig. 3). These findings allowed the diagnosis of an OHVIRA syndrome.

Figure 1: Abdominal ultrassound: hematocolpos. 

Figure 2: Abdominal and pelvic computed tomography: right kidney and retroaortic left renal vein agenesis; duplication of the uterus, cervix, and vagina; hematocolpos.  

Figure 3: Renal scintigraphy: right renal agenesis. 

Discussion

OHVIRA syndrome is a rare congenital female anomaly, that results from an abnormal embryological development of the Mullerian and Wolffian ducts.^1,2,3 It is characterized by the triad: uterus didelphys, unilaterally obstructed hemivagina and ipsilateral renal agenesis.^1,2,3 This syndrome should be considered as a differential diagnosis in female adolescents with dysmenorrhea, abdominal pain, pelvic mass and/or inpatient retention, since it is the typical presention.^1,2,3 Also, in female patients diagnosed with renal agenesis, these anomalies should be investigated, even in pre-pubertal stages.³ The prompt diagnosis and surgical treatment allow a clear improvement in the clinical and reproductive prognosis, avoiding late complications, such as endometriosis, pelvic adhesions, and infertility.^1,2,3 Thus, we emphasize the importance of the knowledge and suspicion of this condition, allowing the diagnosis, referral, and early approach.