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赫林-韦纳-武德连综合征合并膀胱阴道瘘:一例罕见病例报告。

Herlyn-Werner-Wunderlich Syndrome Complicated with Vesicovaginal Fistula: A Rare Case Report.

机构信息

Division of Nephrology, Department of Internal Medicine, Taoyuan Armed Forces General Hospital, Taoyuan 325, Taiwan.

Division of Nephrology, Department of Internal Medicine, Tri-Service General Hospital, National Defense Medical Center, Taipei 114, Taiwan.

出版信息

Medicina (Kaunas). 2024 Jul 1;60(7):1081. doi: 10.3390/medicina60071081.

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

赫尔林-韦纳-温德希(HWW)综合征的特征为单侧阴道梗阻和同侧肾脏异常,是一种罕见的先天性泌尿生殖系统畸形,由与苗勒氏管异常相关的尿路畸形引起。HWW 的初始症状常在初潮后出现,且可能无特异性,导致诊断延迟。我们报告了一例 19 岁女性,其因血尿和腹痛就诊 3 年。最终诊断为 HWW 综合征伴罕见的阴道膀胱瘘。HWW 综合征的治疗通常需要手术干预。主要治疗方法是切除或移除阴道隔。该患者接受了宫腔镜下阴道隔切除术和阴道重建术,以及阴道膀胱瘘修补术。患者术后恢复良好,3 个月后无后遗症,完全康复。此外,单侧肾发育不全是肾脏和泌尿道先天性异常之一,也是儿童慢性肾脏病(CKD)最常见的原因之一。本报告描述了一例 HWW 综合征患者,其罕见地合并阴道膀胱瘘,强调了早期识别和管理以预防相关并发症的重要性。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d317/11278804/7f7701867ff0/medicina-60-01081-g001.jpg

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