Yilmaz Sukriye, Yildiz Adalet Elcin, Fitoz Suat
Department of Radiology, Ankara Child Health, Haematology, Oncology Education and Research Hospital, Ankara, Turkey.
Department of Paediatric Radiology, Faculty of Medicine, Ankara University, Ankara, Turkey.
Pol J Radiol. 2017 Apr 16;82:216-219. doi: 10.12659/PJR.899889. eCollection 2017.
Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. Children usually have progressive pelvic pain after menarche, palpable mass due to hemihaemato(metro)colpos or pelvic inflammatory disease. The diagnosis usually requires a suspicion of this rare genitourinary syndrome.
We present ultrasonography and MR imaging findings of this rare anomaly in two cases.
Early recognition of this rare syndrome can lead to an immediate, proper surgical intervention and is necessary to prevent complications and preserve future fertility. Ultrasound and MR imaging findings can collectively delineate uterine morphology, indicate the absence of ipsilateral kidney and show obstructed hemivagina.
赫林-韦纳-温德利希综合征是一种罕见的先天性泌尿生殖系统异常，其特征为双子宫伴盲端半阴道和同侧肾缺如。儿童通常在初潮后出现进行性盆腔疼痛，因半阴道积血（子宫积血）或盆腔炎可触及肿块。该诊断通常需要怀疑存在这种罕见的泌尿生殖系统综合征。
我们展示了两例该罕见异常的超声和磁共振成像表现。
早期识别这种罕见综合征可促使立即进行恰当的手术干预，对于预防并发症和保留未来生育能力很有必要。超声和磁共振成像表现可共同描绘子宫形态，提示同侧肾脏缺如并显示梗阻性半阴道。
