HWWS HWWS HWWS CA CA X free air

Transcription

1 pp doi: /jjsps.50.1_76 Herlyn-Werner-Wunderlich CT Herlyn-Werner-Wunderlich HWWS HWWS I Herlyn-Werner-Wunderlich HWWS obstructed hemivagina and ipsilateral renal anomary OHVIRA 1) 200 2) HWWS 11 II cm 42 kg 36.4 C CRP 0.68 CA CA X free air

2 CT A B 250 ml 3ABC cm 4 cm CT enhance 1AB HWWS 2 III 6 1 2/3 3)4) 1971 Gartner Herlyn-Werner 1976 Wunderlich Herlyn-Werner-Wunderlich

3 A B 250 ml C 2) HWWS American Fertility Society AFS 17 5)6) 1) 7) 8)9) CT MRI 8) 10) 11)12) CT 13) 14) 2) 12)15) 3

4 ) 2) 16) Cox D, Ching BH: Herlyn-Werner-Wunderlich syndrome: A rare presentation with pyocolpos. J Radiol Case Rep, 6: 9-15, Gholoum S, Puligandla PS, Hui T, et al: Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome). J Pediatr Surg, 41: , Sadler TW, Langman J: Langman s Medical Embryology (8th ed). pp , Lippincott Williams & Wilkins, Philadelphia, Skondras KG, Moutsouris CC, Vaos GC, et al: Uterus didelphys with an obstructed hemivagina and ipsilateral renal agenesis: A rare cause of acute abdomen in pubertal girls. J Pediatr Surg, 26: , Capito C, Echaieb A, Lortat-Jacob S, et al: Pitfalls in 79 the diagnosis and management of obstructive uterovaginal duplication: A series of 32 cases. Pediatrics, 122: e , The American Fertility Society: The American Fertility Society classification of adnexal adhesions, distal tubal occlusion secondary to tubal ligation, tubal pregnancies, Mullen anomaries and intrauterine adhesions. Fertil Steril, 49: , Herlyn-Werner- Wunderlich 1 115: , Wunderlich 1 23: 32-36, Herlyn-Werner- Wunderlich 4: : , : , : , Candiani GB, Fedele L, Candiani M: Double uterus, blind hemivagina, and ipsilateral renal agenesis: 36 cases and long-term follow-up. Obstet Gynecol, 90: 26-32, Watanabe Y, Etoh T, Nakai H: Adenocarcinoma of the lower female genital tract in patients with Herlyn- Werner-Wunderlich syndrome. Am J Obstet Gynecol, 207: e5-6, Wunderlich 4 53: , Olive DL, Henderson DY: Endometriosis and mullerian anomalies. Obstet Gynecol, 69: ,

5 Herlyn-Werner-Wunderlich Syndrome With a Lower Abdominal Mass in an Adolescent Girl Yuki Takeuchi 1, Koji Higuchi 1, Kohei Sakai 1, Shigehisa Fumino 1, Shigeyoshi Aoi 1, Taizo Furukawa 1, Osamu Kimura 1, and Tatsuro Tajiri 1 1 Department of Pediatric Surgery, Kyoto Prefectural University of Medicine 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 Key words: uterus didelphy, hemivaginal septum, ipsilateral renal agenesis, hematometrocolpos Correspondence to: Yuki Takeuchi, Department of Pediatric Surgery, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto, JAPAN 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. 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.