Xu Xiaotong, Tian Yanpeng, Zhou Jingwen, Li Zhongkang, Meng Li, Huang Xianghua, Zhang Mingle
Department of Gynecology, The Second Hospital of Hebei Medical University, Shijiazhuang, Hebei, 050000, PR China.
Department of Gynecology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, 450052, PR China.
Heliyon. 2024 Jun 25;10(13):e33558. doi: 10.1016/j.heliyon.2024.e33558. eCollection 2024 Jul 15.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital genitourinary abnormality defined by uterine didelphys, obstructed hemivagina, and ipsilateral urological anomalies. Accurate diagnosis and prompt commencement of therapy can be difficult owing to heterogeneous genitourinary malformation among different patients. This is a case report of a patient with rare HWWS with uterine didelphys, obstructed hemivagina, vagina-ureteral remnant fistula (Gartner's duct cyst), and ipsilateral kidney dysgenesis who complained of intermittent abdominal pain during menstruation. The right ureteral remnant of the patient was distinctive, with three portions. The upper section was connected to the right dysplastic kidney, the lower section formed the fistulous tract with the vagina and bladder, while the middle section communicated with Gartner's Duct Cyst, which merged with the vagina and opened to the posterior cavity of hemivagina. The lower section of the right ureter was excised and ligated during laparoscopic surgery, while the upper section was excised. The patient recovered after surgery. We presented this rare case and conducted a literature review to provide a more comprehensive understanding of HWWS. This could help gynecologists effectively reduce misdiagnosis and missed diagnosis, especially when combined with complicated urinary malformation.
赫林-韦纳-温德利希综合征(HWWS)是一种罕见的先天性泌尿生殖系统异常,其特征为双子宫、阴道半段梗阻以及同侧泌尿系统异常。由于不同患者的泌尿生殖系统畸形具有异质性,准确诊断并及时开始治疗可能具有挑战性。本文报告了一例罕见的HWWS患者,该患者存在双子宫、阴道半段梗阻、阴道-输尿管残余瘘(加特纳管囊肿)以及同侧肾发育不全,患者在月经期间出现间歇性腹痛。该患者的右侧输尿管残余部分较为独特,分为三个部分。上段与右侧发育不良的肾脏相连,下段与阴道和膀胱形成瘘管,而中段与加特纳管囊肿相通,该囊肿与阴道融合并开口于阴道半段的后腔。在腹腔镜手术中切除并结扎了右侧输尿管的下段,同时切除了上段。患者术后康复。我们展示了这一罕见病例并进行了文献综述,以更全面地了解HWWS。这有助于妇科医生有效减少误诊和漏诊,尤其是在合并复杂泌尿系统畸形的情况下。