Herlyn-Werner-Wünderlich 综合征:两例报告

Pub Date : 2024-08-01 Epub Date: 2024-01-18 DOI:10.5546/aap.2023-10138.eng
Victoria Fontana, Gabriela S Aboud, Liliana Sabbaj
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引用次数: 0

摘要

Herlyn-Werner-Wunderlich综合征,又称血道阻塞和同侧肾脏异常(OHVIRA),是一种罕见的先天性穆勒氏管异常,其特征是子宫中隔、血道阻塞和同侧肾脏发育不良。最常见的临床表现是继发于血性结肠炎、疼痛和痛经的腹部肿块。它与不孕症、子宫内膜异位症、月经和产科改变有关。超声波是初步评估的首选技术,而磁共振成像仍然是最准确的诊断方法。阴道隔膜切除术是推荐的治疗方法。在此,我们描述了两个临床病例,以强调早期诊断对预防未来潜在并发症的重要性。
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Herlyn-Werner-Wünderlich syndrome: Two case report.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

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