Herlyn-Werner-Wunderlich syndrome: merits of sonographic and magnetic resonance imaging for accurate diagnosis and patient management in 13 cases

Abstract

Study objective: To review the main sonographic and magnetic resonance imaging manifestations of Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis, and to discuss the value of techniques for its diagnosis and treatment.

Participants: Thirteen patients with Herlyn-Werner-Wunderlich syndrome referred to our department with acute abdomen were identified for the period from 2009-2012.

Design: Retrospective chart review.

Setting: Two tertiary academic centers.

Interventions: None.

Main outcome measures: The evaluation of the clinical features, ultrasonography and magnetic resonance imaging findings, and the treatment protocols.

Results: Ultrasonographic evaluation of didelphic or double uterus in 10 and bicornuate-bicollis uterus in 3 patients with hematocolpos or hematometrocolpos associated with agenesis of the ipsilateral kidney revealed the diagnosis. In 10 patients, magnetic resonance imaging provided extra information. In 1 patient, a 25-week pregnancy was additionally detected and follow-up was suggested. Seven patients underwent surgical excision of the septum and drainage of the obstructed vagina. Total hysterectomy and hemicolpectomy were performed in 1 patient with 2 children suffering from severe chronic pelvic pain due to pelvic inflammatory disease.

Conclusions: Despite its rarity, accurate diagnosis and morphologic description of Herlyn-Werner-Wunderlich syndrome with radiologic modalities are of importance in relieving the clinical complaints by enabling the suitable surgical treatment to be identified.

Keywords: Herlyn-Werner-Wunderlich syndrome; Magnetic resonance imaging; Obstructed hemivagina; Ultrasonography; Uterus didelphys.