Herlyn-Werner-Wunderlich Syndrome With a Lower Abdominal Mass in an Adolescent Girl

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  • Takeuchi Yuki
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Higuchi Koji
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Sakai Kohei
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Fumino Shigehisa
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Aoi Shigeyoshi
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Furukawa Taizo
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Kimura Osamu
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine
  • Tajiri Tatsuro
    Department of Pediatric Surgery, Kyoto Prefectural University of Medicine

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Other Title
  • 腹部腫瘤により発見されたHerlyn-Werner-Wunderlich 症候群の1 例
  • フクブ シュリュウ ニ ヨリ ハッケン サレタ Herlyn-Werner-Wunderlich ショウコウグン ノ 1レイ

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Abstract

An 11-year-old girl, complaining of an abdominal mass with tenderness in the right lower quadrant, was admitted urgently to our center. She noticed that the size of an abdominal mass had gradually increased in the previous 3 months. Torsion of ovarian tumor was suspected initially. However, abdominal ultrasonography revealed that the abdominal mass was a multilocular cyst, although her right kidney was not examined then. Subsequent enhanced computed tomography demonstrated uterus didelphys, obstructed hemivagina with unilateral hematocolpos and ipsilateral renal agenesis. She was diagnosed as having the Herlyn-Werner-Wunderlich syndrome (HWWS). Partial excision and marsupialization of the vaginal septum was performed electively. Hematometrocolpos of the right half of the uterus didelphys was drained. She had an uneventful recovery and was free of symptoms 1 year after operation.<br>HWWS is a rare mullerian anomaly consisting of uterus didelphys, unilateral obstructed hemivagina with and ipsilateral renal agenesis. Most cases show symptoms related to these gynecologic anomalies within the first few periods after menarche. However, almost all cases of HWWS, including our present case, are difficult to diagnose accurately on initial examination. The vagina remained unilaterally open in HWWS patients to present regular menstruations, and this normal-like menorrhea may delay accurate diagnosis. HWWS should be suspected in adolescent females presenting a pelvic mass and renal agenesis. Early and accurate diagnosis with appropriate corrective operation, including excision of vaginal septum and drainage of hematometrocolpos, may preserve fertility on HWWS patients.

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