Herlyn-Werner 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 bicomuate-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.