Herlyn-Werner-Wunderlich 증후군 1례: 12세 여자 환자의 드문 선천성 비뇨생식계 기형
A case of Herlyn-Werner-Wunderlich syndrome: a rare, congenital genitourinary anomaly in a 12-year-old girl
박중완, 정재윤,
소속 상세정보
박중완 ( Pakr Joong-Wan )
서울대학교 의과대학 응급의학교실
정재윤 ( Jung Jae-Yun )
분당서울대학교병원 응급의학과
KMID : 1037620160030010032
Abstract
Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Mullerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.
키워드
Hematocolpos; Hereditary Renal Agenesis; Mullerian Ducts; Uterine Anomalies; Wolffian Ducts
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