일측성질폐쇄 및 동측신장무형성을 동반한 중복자궁의 진단과 치료
Diagnosis and treatment of uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis
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초록
선천성 자궁기형은 태생기때 뮬러관의 융합 장애로 일어나며, 그 발생 빈도는 0.001~10%로 다양하게 보고되고 있다. 또한 다른 신체 기관의 기형을 동반하기도 하는데 신장 무형성과 같은 비뇨기계 기형이 가장 흔하다. 편측의 질폐쇄 및 동측 신장 무형성을 동반하는 중복자궁의 경우 흔한 증상으로 초경 후 발생하는 골반통과 생리통, 골반 종괴의 소견을 보이며 진단이 지연될 경우 심한 자궁내막증이나 불임을 유발할 수 있다. 따라서 정확한 조기 진단이 중요하며 높은 민감도와 특이도를 가진 초음파와 자기공명영상이 이용될 수 있고 치료로는 질중격절제술과 조대술을 시행하여 증상 완화와 생식력 보존에 성공적인 치료효과를 기대할 수 있다. 본 저자들은 일측성 질폐쇄와 동측 신장 무형성을 동반한 중복자궁 7예를 경험하였기에 간단한 문헌고찰과 함께 보고하는 바이다.
Abstract
Uterine malformations consist of a group of miscellaneous congenital anomalies of the female genital system, and these anomalies are the result of major disturbances in the development, such as formation or fusion of the Mullerian or paramesonephric ducts during fetal life. The Mullerian anomalies have been estimated to occur in up to 0.001~10%, and these anomalies may be associated with numerous congenital anomalies, and ipsilateral renal anomalies especially are the most common. The uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis clinically may result in dysmenorrhea, low abdominal pain, endometriosis or pelvic mass after menarche. Furthemore the delayed diagnosis causes infertility and poor pregnancy outcome due to continued retrograde menstruation which leads to endometriosis and distorted pelvic anatomy. These anomalies could be diagnosed with ultrasonography and MRI that have the high sensitivity and specificity. Excision of vaginal septum and marsupialization are appropriate treatments to relieve symptoms and reserve fertility. We report 7 cases of uterine didelphys with obstructed hemivagina and ipsi-unilateral renal agenesis with a brief review of relevant literatures to help understand these anomalies
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