비박형 사구체 기저막 질환이 동반된 중복 자궁 일측성 폐쇄질 및 동측 신장 무형성 증후군(Herlyn-Werner-Wunderlich syndrome) 1례
Thin Glomerular Basement Membrane Disease withHerlyn-Werner-Wunderlich Syndrome : Uterus Didelphys, BlindHemivagina and Ipsilateral Renal Agenesis
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HHW(Herlyn-Werner-Wunderlich) 증후군은 중복자궁, 일측성 폐쇄질 및 동측 신장 무형성을 보이는 비뇨생식기계의 선천성 기형으로 매우 드문 질환 중 하나이다. 대부분 초경 이후 발생하는 월경통이나 복강내 종물등으로 발견되지만, 본 증례는 소아에서 반복되는 요로감염과 현미경적 혈뇨로 인해 진단된 경우이다. 복부 초음파 검사에서 일측 신무형성이나 중복 자궁의 소견이 보일 때는 이러한 뮬러관 기형의 가능성을 염두에 두어야 보다 빠른 진단이 가능하고, 환자의 고통 또한 줄여줄 수 있을 것이다．특히 반복되는 요로 감염 시에도 단순한 감염 치료보다 정밀검사를 항상 염두에 두어야 할 것이다.
Abstract
Herlyn-Werner-Wunderlich syndrome(HWWs) is a rare variant of Mullerian ductal anomalies characterized by the presence of a hemivaginal septum, a didelphic uterus, and ipsilateral renal agenesis. It usually presents after menarche with progressive pelvic pain, and palpable mass due to hemihematocolpos. If a cystic mass is detected behind the urinary bladder in children, in association with the absence of a kidney, the diagnosis of uterus didelphys with imperforate vagina and hydrocolpos should be considered. When renal agenesis is found in asymptomatic children, the small size and the tubular shape of the uterus makes it almost impossible to evaluate uterine anomalies, so follow-up should be performed until the end of puberty. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedures and offer relief of symptoms. We report one case of didelphic uterus with blind hemivagina and ipsilateral renal agenesis with biopsy- proven thin glomerular basement membrane disease which is not related to the above syndrome.
참고문헌 (20)
- "Renal aplasia is the predominant cause of congenital solitary kidneys", 61, pp.1840~1844 (2002)
- "Urological anomalies in children with renal agenesis or multicystic dysplastic kidney", 47, pp.171~176 (2006)
- "Associated urologic anomalies in children with solitary kidney", 39, pp.85~87 (2004)
- "Genital anomalies associated with solitary kidney", pp.538~48
- "Simultaneous occurrence of an open Gartner-duct cyst a homolateral aplasia of the kidney and a double uterus as a typical syndrome of abnormalities", pp.340~7
- "Unusual form of genital malformation with aplasia of the right kidney", pp.559~62
- "A new embryologic classification for uro-gynecologic malformations the syndromes of mesonephric duct induced mullerian deformities", pp.265~7
- "Mullerian defects in women with normal reproductive outcome", pp.1192~3
- "Asymmetric lateral distribution of obstructed hemivagina and renal agenesis in women with uterus didelphys: institutional case series and a systematic literature review", 87, pp.719~724 (2007)
- "A Case of the Syndrome of Uterus Didelphys, Unilateral Obstructed Hemivagina, and Ipsilateral Renal Agenesis Associated with Ipsilateral Gartner's Duct Cyst and Contralateral Renal Cysts", 41, pp.689~693 (2000)
- "A Case of Communicating Double Uterus with Double Cervix, Blind Hemivagina and Ipsilateral Renal Agenesis", 23, pp.801~806 (2002)
- "Bicervical Bicornuate Uterus with Unilateral Cervial Atresia and Homolateral Renal Agenesis", 12, pp.2841~2845 (1999)
- "Herlyn-Werner-Wunderlich syndrome: uterus didelphys, blind hemivagina and ipsilateral renal agenesis. Sonographic and MR findings in 11 cases", 37, pp.657~665 (2007)
- "Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis(Herlyn-Werner-Wunderlich syndrome)", 41, pp.987~992 (2006)
- "The congenital and acquired solitary kidney", 5, pp.2~8 (2003)
- "Diagnosing neonatal female genital anomalies using saline-enhanced sonography", 177, pp.1041~1044 (2001)
- "Didelphic uterus and obstructed hemivagina with renal agenesis: case report and review of the literature", 17, pp.137~141 (2004)
- "MR imaging diagnosis of uterovaginal anomalies: current state of the art", 23(e13) (2003)
- "Magnetic resonance imaging in the evaluation of uterus didelphys with obstructed hemivagina and renal agenesis: a case report", 274, pp.246~247 (2006)
- "Analysis of renal biopsies performed in children with abnormal findings in urinary mass screening", 95, pp.849~853 (2006)