PDF(Pubmed)
Abstract：
Herlyn-Werner-Wunderlich (HWW) syndrome is characterized by obstructed hemivagina and ipsilateral renal anomaly, a rare congenital anomaly of the genitourinary tract, resulting from malformations of the renal tract associated with Müllerian duct anomalies. The initial symptoms of HWW frequently present after menarche and may be nonspecific, leading to a delayed diagnosis. We presented a 19-year-old female with 3-year hematuria and abdominal pain. The final diagnosis of HWW syndrome with a rare vesicovaginal fistula was made. The treatment of HWW syndrome typically involves surgical intervention. The primary treatment is resection or removal of the obstructed vaginal septum. The patient underwent excision of vaginal septum and vaginal reconstruction via hysteroscopy, as well as repair of the vesicovaginal fistula. The patient improved well after surgery and fully recovered without sequelae after 3 months. In addition, unilateral renal agenesis is one of congenital abnormalities of the kidney and urinary tract, which are the most frequent cause of chronic kidney disease (CKD) in children. This report describes a patient of HWW syndrome with rarely combined vesicovaginal fistula, and highlights the importance of early recognition and management to prevent associated complications.
摘要：
Herlyn-Werner-Wunderlich（HWW）综合征的特征是半阴道阻塞和同侧肾脏异常，一种罕见的先天性泌尿生殖道异常，由与苗勒管异常相关的肾道畸形引起。HWW的初始症状在初潮后经常出现，可能是非特异性的，导致延迟诊断。我们介绍了一名19岁的女性，患有3年血尿和腹痛。最终诊断为HWW综合征伴罕见膀胱阴道瘘。HWW综合征的治疗通常涉及手术干预。主要治疗是切除或去除阻塞的阴道隔。患者行阴道隔切除术和宫腔镜阴道重建术，以及膀胱阴道瘘的修复。患者术后好转良好，3个月后完全恢复，无后遗症。此外,单侧肾脏发育不全是肾脏和泌尿道的先天性异常之一，这是儿童慢性肾病（CKD）的最常见原因。该报告描述了HWW综合征患者，很少合并膀胱阴道瘘，并强调了早期识别和管理对预防相关并发症的重要性。
