Department of Radiology and Imaging, Bharatpur Hospital, Chitwan, Nepal.
Department of Obstetrics and Gynaecology, Bharatpur Hospital, Chitwan, Nepal.
JNMA J Nepal Med Assoc. 2022 Jun 1;60(250):562-564. doi: 10.31729/jnma.7444.
Obstructed hemivagina and ipsilateral renal anomaly syndrome also known as Herlyn-Werner-Wunderlich syndrome is a rare congenital urogenital anomaly characterised by Mullerian duct anomalies associated with mesonephric duct anomalies. A 10-year old female presented with acute lower abdominal pain, urinary retention and scanty menstrual flow during her first menstruation. Ultrasonography and contrast computed tomography showed uterine didelphys, hematocolpos, obstructed hemivagina and left renal agenesis. Hemivaginal septal resection and drainage of the hematocolpos were done and operative findings also confirmed the final diagnosis. She was discharged and followed up after 2 weeks and her symptoms had resolved completely. Being a rare entity many clinicians and radiologists are unaware of this disease so this may lead to misdiagnosis whenever these cases present. So strong suspicion and knowledge of this disease entity are essential for a precise diagnosis.
case reports; hematocolpos; mullerian ducts; unilateral renal agenesis.
中文译文：
单侧阴道闭锁和同侧肾发育不全综合征，也称为赫尔林-韦纳-武德利希综合征，是一种罕见的先天性泌尿生殖系统畸形，其特征为苗勒管畸形合并中肾管畸形。一名 10 岁女性因急性下腹痛、尿潴留和初潮时月经流量少而就诊。超声和对比增强 CT 显示双子宫、阴道积血、单侧阴道闭锁和左肾发育不全。行阴道斜隔切除术和阴道积血引流术，手术发现也证实了最终诊断。术后 2 周出院，症状完全缓解。由于这种疾病较为罕见，许多临床医生和放射科医生都不了解，因此，当这些病例出现时，可能会导致误诊。因此，强烈怀疑和了解这种疾病对于准确诊断至关重要。
关键词：病例报告；阴道积血；苗勒管；单侧肾发育不全
