[Uterus Didelphys, obstructed hemivagina and ipsilateral renal agenesis as a presentation of a case of the Herlyn-Wemer-Wünderlich syndrome. Literature review]

Abstract

The Herlyn-Werner-Wünderlich syndrome is a rare congenital anomaly of the Müllerian ducts. Müllerian malformations result from defective fusion of the Müllerian ducts during development of the female reproductive system. These malformations are associated with an increased probability of adverse obstetric events. The syndrome is characterized by uterus didelphys with obstructed hemivagina, and ipsilateral renal agenesis. Patients with this syndrome are asymptomatic until menarche, and can follow other complications.

Case report: A 12 year-old female patient presented with a clinical picture of acute abdomen and a previous history of cyclical dysmenorrhea. A diagnosis of uterus didelphys associated with right hematometracolpos was made following an exploratory laparotomy. Resection of the right obstructed hemivagina was subsequently performed using a vaginal approach. A subsequent computed tomography seen identified renal agenesis ipsilateral to the obstructed hemivagina.

In conclusion: The Herlyn-Werner-Wünderlich syndrome is a rare Müllerian anomaly. The characteristics of the syndrome may result in missed diagnosis until detailed evaluation is carried out. We therefore advise clinicians to look for Müllerian duct anomalies whenever a renal malformation is discovered in a fetus or girl postnatal. Early diagnosis of the syndrome and its associated anomalies are essential to provide adequate therapy, and to reduce reproductive complications through radical treatments. Conservative treatment is effective and has a good prognosis for the patient's reproductive function.