Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome
Preventing the O in OHVIRA (Obstructed Hemivagina Ipsilateral Renal Agenesis): Early Diagnosis and Management of Asymptomatic Herlyn-Werner-Wunderlich Syndrome
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DOI：
10.1016/j.jpedsurg.2019.06.006
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发表时间：
2020-07-01
影响因子：
2.4
通讯作者：
Ong, Caroline C. P.
中科院分区：
文献类型：
--
作者：
Tan, Yu Guang;Laksmi, Narasimhan K.;Ong, Caroline C. P.
前言：Herlyn-Werner-Wunderlich综合征（HWWS）是一种罕见的苗勒管畸形伴同侧肾发育不全的变异型。大多数患者在月经初潮后被诊断为子宫阴道梗阻、泌尿生殖道感染和盆腔粘连并发症。患者经常因误诊或治疗并发症而接受额外手术。我们的机构管理几个HWWS患者诊断前的症状，筛查产前诊断的肾发育不全。本研究旨在改善HWWS患者的症状前管理。方法：我们对2010年至2017年诊断为HWWS的患者进行了回顾性病例分析，包括患者人口统计学，症状，临床病程和手术管理，并总结了迄今为止发表的稀疏文献。结果：有8例HWWS患者，但只有2例有症状的患者急性阴道积血，需要紧急阴道手术。另外6例患者通过产后超声筛查得到早期诊断。没有病人需要进一步的手术诊断或并发症阻塞hemivagina.Conclusion：我们的病例系列和文献回顾表明，大多数青春期前的HWWS患者不需要早期妇科手术。我们建议，女婴与肾脏发育不全，应筛选HWWS综合征超声。早期诊断和症状前选择性手术可以预防导致生育和肾功能损害的泌尿妇科并发症。研究设计：病例系列，IV级证据。(C)2019 Elsevier Inc版权所有。
introduction: Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian ductal anomaly associated with ipsilateral renal agenesis. Most patients are diagnosed after menarche with complications of uterovaginal obstruction, genitourinary infection and pelvic adhesions. Patients often undergo additional operations for misdiagnosis or treatment of complications. Our institution manages several HWWS patients diagnosed before symptoms by screening for antenatally-cliagnosed renal agenesis. This study aims to improve the presymptomatic management of HWWS patients.Methods: We carried out retrospective case review of patients diagnosed with HWWS from 2010 to 2017 on patient demographics, symptoms, clinical course and operative management and summarize the sparse literature published to date.Results: There were 8 patients with HWWS but only 2 symptomatic patients presented acutely with hematocolpos requiring urgent vaginal surgery. The other six patients had early diagnosis through postnatal ultrasound screening. No patient required further operation for diagnosis or complications related to obstructed hemivagina.Conclusion: Our case series and literature review show that the majority of prepubertal patients with HWWS do not require early gynecological surgery. We recommend that female babies with renal agenesis should be screened for HWWS syndrome with ultrasound. Early diagnosis and presymptomatic elective surgery may prevent urogynecological complications that cause fertility and renal impairment.Study design: Case series, level IV evidence. (C) 2019 Elsevier Inc All rights reserved.