주산기에 Herlyn-Werner-Wunderlich 증후군으로 진단된 2례
Two Cases of Herlyn-Werner-Wunderlich Syndrome Diagnosed in Perinatal Period
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초록
Herlyn-Werner-Wunderich 증후군은 뮬러관과 울피안관이 연루된 비뇨생식기계의 매우 드문 선천 기형으로 중복 자궁과 일측성 폐쇄질 및 동측의 신장 무형성을 특징으로 하며, 초경 이후 반복되는 골반통이나 복강내 종괴로부터 의심하여진단되는 경우가 많다. 이 증후군은 방치될 경우 화농자궁관 및 골반 협착, 불임 등이 합병될 수 있으나 합병증이 없는경우 간단한 수술적 치료만으로 예후가 양호하다. 따라서 산전 초음파 검사에서 일측 신장의 무형성이 의심되는 경우 동반될 수 있는 비뇨생식기계 기형을 의심한다면 신생아기에 이 증후군을 진단하여 추적 관찰하고 초경 전 수술적 치료를시행함으로써 환자의 고통과 합병증을 조기에 예방할 수 있을 것이다. 저자들은 산전초음파상 일측 신장 무형성이 의심되어 출생 후 평가를 통해 Herlyn-Werner-Wunderlich 증후군으로 진단된 2례를 경험하여 보고하는 바이다.
Abstract
Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.
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