Herlyn-Werner-Wunderlich syndrome: Why is early diagnosis important?

Background. Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) or Herlyn-Werner-Wunderlich (HWW) syndrome is an associative genitourinary tract malformation: uterus didelphys and unilateral aplasia of its lower two-thirds and the kidney. Despite the fact that the defect occurs in 0.16-10% of women with genital tract malformation from the detection of renal aplasia to the identification of genital malformation, is present throughout childhood and pre-puberty; that is, an average of 10-12 years. Moreover, lack of awareness about the possible association between genital and urinary tract defects causes girls to frequently undergo wrong surgical interventions. As a result, the common outcome of these interventions is the formation of strictures, fistulas, and suppurations of the closed vagina and cicatricial deformities of the adequate second vagina in every three patients. Case report. The paper describes three clinical cases of girls with HWW syndrome that was timely unestablished, which resulted in various complications. Conclusion. Detection of urinary tract malformation in a girl at any age requires that a pediatric and adolescent gynecologist should mandatorily examine in order to timely diagnose associated developmental abnormalities. Patients with genitourinary tract defects should be assisted at third-level specialized hospitals, which will be able to minimize the risk of subsequent complications.

OHVIRA, genitourinary tract malformation, girls, Herlyn-Werner-Wunderlich syndrome, OHVIRA, reconstructive gynecology