Background and aims Müllerian anomalies are rare, causing malformations of the female reproductive system as the Herlyn-Werner-Wunderlich Syndrome (HWWS) and distal vaginal atresia. These patients are usually asymptomatic until menarche. There may be associated with renal and urinary tract anomalies. We describe two cases of young girls with occluded vagina associated with urethrovaginal fistula.
Cases presentation The first patient is a 3-years-old asymptomatic girl with prenatal diagnosis of left renal agenesis. Ultrasonography, performed 6 months prior to consultation, identified an anechoic cyst in hypogastrium. On genital examination, distal vagina occluded by a protruding membrane over the vaginal introitus. Magnetic resonance revealed a left paravesical formation suggestive of hydrocolpos. By the transmembrane puncture, urine was aspired. Laparoscopy confirms uterus didelphys. Membrane resection enable two hemivaginas. Based on the association of these anomalies, the diagnosis of HWWS was made. Girl present constant loss of urine and cystoureterography performed reveals urethrohemivaginal fistula. The second patient is a 4-years-old girl with occluded vagina suggesting distal atresia. Saline injected through the membrane eliminates by urethra. Cystoureterography confirms urethrovaginal fistula.
Conclusions The diagnoses of HWWS (uterus didelphys, unilateral low vaginal obstruction and ipsilateral renal agenesis) also known by OHVIRA and distal vaginal atresia in infancy and early childhood are unusual. The reported cases represent examples off premenarche diagnosis. In both patients, we detected urethrovaginal fistula, association not reported before in literature. Early diagnosis allows appropriate therapeutic management and prevents subsequent complications.
- Herlin-Werner-Wunderlich Syndrome
- Ipsilateral renal agenesis
- uterus didelphys
- urethrovaginal fistula.