腹部腫瘤により発見されたHerlyn-Werner-Wunderlich 症候群の1 例 [in Japanese] Herlyn-Werner-Wunderlich Syndrome With a Lower Abdominal Mass in an Adolescent Girl [in Japanese]
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11 歳女児．初潮以降3 回の月経周期に伴う右下腹部痛，腹部腫瘤および同部位圧痛を主訴に近医を受診したところ，腹部超音波にて骨盤内腫瘤を認め，精査目的に紹介となった．腹部CT にて重複子宮・膣と右膣閉鎖による右子宮膣留血腫を認めた．また右腎は無形成であった．Herlyn-Werner-Wunderlich 症候群（HWWS）と術前診断し，待機的手術を施行した．右膣閉鎖と膣壁膨隆を認め，膣中隔切開・内容液ドレナージ後，膣壁形成を施行した．以降，下腹部痛と腹部腫瘤は消失し，術後経過，月経発来も順調である．HWWS は，見かけ上，月経が正常に起こるため他の女性性器奇形（処女膜閉鎖，膣閉鎖）よりも診断が遅れることがある．治療は膣中隔切除と留血腫ドレナージで良好な成績を得られる．下腹部腫瘤と片側腎無形成を伴う二次性徴期女性の急性腹症に対しては，HWWS を考慮に入れて診断と治療を行うべきである．
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.
- Journal of the Japanese Society of Pediatric Surgeons
Journal of the Japanese Society of Pediatric Surgeons 50(1), 76-80, 2014
The Japanese Society of Pediatric Surgeons