关键词: Complete obstruction Congenital malformation Herlyn-Werner-Wunderlich syndrome (HWWS) Incomplete obstruction Obstructed hemivagina

来  源:   DOI:10.1007/s43032-024-01628-2

Abstract:
OBJECTIVE: To describe the clinical and imaging characteristics of Herlyn-Werner-Wunderlich syndrome (HWWS).
METHODS: This study presented an observational case series involving consecutive patients diagnosed with HWWS, whose medical records were retrospectively reviewed. From June 2012 to December 2022, there were a total of 85 patients with HWWS enrolled in our study. We obtained the medical history, including demographic characteristics, clinical presentation, treatment, complications, and radiologic examinations performed. Patients > 18 years of age (n = 58) were recontacted.
RESULTS: In our analysis, 27 patients were categorised as having complete obstruction, and 58 were categorised as having incomplete obstruction. The mean age at the onset of symptoms and diagnosis of complete obstruction was significantly younger than incomplete obstruction (P < 0.05). For complete obstruction, the median time between menarche and the onset of symptoms was 2.1 years, while for incomplete obstruction, it was 5.3 years. There was a significantly lower incidence of intermittent mucopurulent discharge, irregular vaginal haemorrhage, and occasional examination findings of complete obstruction than incomplete obstruction (P < 0.05). Complete obstruction was significantly associated with dysmenorrhea and pelvic endometriosis compared with incomplete obstruction (P < 0.05).
CONCLUSIONS: There are distinct clinical differences between patients with complete obstruction of the hemivagina and those with incomplete obstruction. HWWS can manifest as various combinations of uterine anomalies, communications anomalies, and renal anomalies. Early recognition and treatment can avoid complications and preserve fertility.
UNASSIGNED: Herlyn-Werner-Wunderlich syndrome (HWWS); complete obstruction; incomplete obstruction; obstructed hemivagina; congenital malformation.
摘要:
目的:描述Herlyn-Werner-Wunderlich综合征(HWWS)的临床和影像学特征。
方法:本研究提供了一系列观察性病例,包括连续诊断为HWWS的患者,对其医疗记录进行了回顾性审查。从2012年6月到2022年12月,共有85名HWWS患者参加了我们的研究。我们拿到了病史,包括人口特征,临床表现,治疗,并发症,和放射学检查。年龄>18岁的患者(n=58)被重新联系。
结果:在我们的分析中,27例患者被归类为完全梗阻,58人被归类为不完全阻塞。出现症状和诊断完全梗阻的平均年龄明显小于不完全梗阻(P<0.05)。对于完全阻塞,初潮和症状发作之间的中位时间为2.1年,而对于不完整的阻塞,这是5.3年。间歇性粘液脓性分泌物的发生率明显降低,不规则阴道出血,且偶尔检查发现完全梗阻优于不完全梗阻(P<0.05)。与不完全梗阻相比,完全梗阻与痛经和盆腔子宫内膜异位症显著相关(P<0.05)。
结论:半阴道完全梗阻患者和不完全梗阻患者之间存在明显的临床差异。HWWS可以表现为子宫异常的各种组合,通信异常,和肾脏异常.早期识别和治疗可以避免并发症并保持生育能力。
Herlyn-Werner-Wunderlich综合征(HWWS);完全梗阻;不完全性梗阻;半阴道梗阻;先天性畸形。
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