Herlyn-Werner-Wunderlich 综合征和子宫内膜异位症患者成功足月妊娠(40 周零 6 天):病例报告。

IF 0.9 Q3 MEDICINE, GENERAL & INTERNAL Journal of Medical Case Reports Pub Date : 2024-08-03 DOI:10.1186/s13256-024-04695-w
Juliana Vieira Queiroz Almeida Oliveira, Chris Elizabeth Philip, Thayná Andreza Ribeiro Pereira, Gabriela Martins Perez Garcia, Quésia Tamara Mirante Ferreira Villamil
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引用次数: 0

摘要

简介Herlyn-Werner-Wunderlich综合征是一种罕见的Müllerian导管先天性疾病,其特征是双子宫、盲性半阴道和同侧肾缺如。通过超声波和磁共振成像可在幼年时确诊,预后良好。通常,并发症会演变为子宫内膜异位症和继发性盆腔炎:一名 40 岁的女性患者,巴西人,白人,初产妇,30 岁时超声诊断为无子宫,4 年后磁共振检查发现左侧卵巢子宫内膜异位症、多发性卵巢囊肿和左肾缺如。随后,由于排便困难和肿胀感,患者接受了肠道准备的经阴道超声波检查,发现了血性结肠炎,并怀疑患有 Herlyn-Werner-Wunderlich 综合征;确诊 10 年后,她计划怀孕。她在怀孕 15 周后出现频繁宫缩,所幸没有并发症或早产。分娩在 40 周零 6 天时进行,但没有进展,因此需要进行剖宫产手术,手术没有出现并发症。Herlyn-Werner-Wunderlich综合征常常不被人注意,导致治疗不当。Herlyn-Werner-Wunderlich综合征患者通常会面临生育问题,如高流产率(21%-33%)和产科并发症,如自然流产(40%的风险)、宫内发育受限、产后出血、胎儿死亡率增加、早产(21%-29%)和剖宫产率升高。此外,子宫内膜异位症的发病率也较高,尤其是伴有半阴道梗阻和盆腔粘连的患者:结论:早期诊断有助于及时治疗,从而减少并发症。尽管如此,如果不存在这些因素,仍有可能通过阴道分娩。与 Herlyn-Werner-Wunderlich 综合征相关的并发症的真实发病率和发生率仍是未知数。
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Patient with Herlyn-Werner-Wunderlich syndrome and endometriosis achieves successful full-term pregnancy (40 weeks and 6 days): a case report.

Introduction: Herlyn-Werner-Wunderlich syndrome , a rare Müllerian ducts congenital disease, is characterized by a diphtheritic uterus, blind hemivagina, and ipsilateral renal agenesis. Diagnosis is at young age by ultrasound and magnetic resonance imaging, and the prognosis is good. Usually, complications evolve endometriosis and secondary pelvic inflammation.

Case report: A 40-year-old female patient, Brazilian, white, primigravida, diagnosed at 30 years with a didelphic uterus on ultrasound, and 4 years later, with a left ovarian endometrioma, multiple ovarian cysts, and left renal agenesis on magnetic resonance imaging. Subsequently, due to dyspareunia and a feeling of swelling, the patient underwent transvaginal ultrasound with bowel preparation, and a hematocolpos was found and Herlyn-Werner-Wunderlich syndrome was suspected; 10 years after the diagnosis she had a planned pregnancy. She presented frequent contractions following the 15th week of pregnancy and fortunately there were no complications or premature labor. Labor was inducted at 40 weeks and 6 days without progress and a cesarean section was indicated and performed without complications. Herlyn-Werner-Wunderlich syndrome often goes unnoticed, leading to inadequate treatment. Individuals with Herlyn-Werner-Wunderlich syndrome commonly face fertility issues, such as high miscarriage rate (21-33%), and obstetric complications, such as spontaneous abortions (40% risk), intrauterine growth restriction, postpartum hemorrhage, increased fetal mortality, preterm delivery (21-29%), and elevated rates of cesarean sections. In addition, there is higher susceptibility of developing endometriosis, especially with hemivaginal obstruction, and pelvic adhesions.

Conclusion: Early diagnosis enables timely treatment and, consequently, fewer complications. Still, when these factors are absent, vaginal birth may still be possible. The true prevalence and incidence of complications related to Herlyn-Werner-Wunderlich syndrome are still unknown.

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来源期刊
Journal of Medical Case Reports
Journal of Medical Case Reports Medicine-Medicine (all)
CiteScore
1.50
自引率
0.00%
发文量
436
期刊介绍: JMCR is an open access, peer-reviewed online journal that will consider any original case report that expands the field of general medical knowledge. Reports should show one of the following: 1. Unreported or unusual side effects or adverse interactions involving medications 2. Unexpected or unusual presentations of a disease 3. New associations or variations in disease processes 4. Presentations, diagnoses and/or management of new and emerging diseases 5. An unexpected association between diseases or symptoms 6. An unexpected event in the course of observing or treating a patient 7. Findings that shed new light on the possible pathogenesis of a disease or an adverse effect
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