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双子宫伴单侧宫颈和(或)阴道发育不全/发育不良(CVAD)的临床表现和处理:多中心病例系列。

Presentation and Management of Uterine Didelphys with Unilateral Cervicovaginal Agenesis/Dysgenesis (CVAD): A Multicenter Case Series.

机构信息

Department of Obstetrics & Gynecology, Baylor College of Medicine, Houston, Texas; Department of Obstetrics & Gynecology, University of Oklahoma College of Medicine, Oklahoma City, Oklahoma.

University of Oklahoma College of Medicine, Oklahoma City, Oklahoma; Department of Obstetrics & Gynecology, University of Southern California, Los Angeles, California.

出版信息

J Pediatr Adolesc Gynecol. 2024 Feb;37(1):72-77. doi: 10.1016/j.jpag.2023.08.001. Epub 2023 Aug 7.

DOI:10.1016/j.jpag.2023.08.001
PMID:37558159
Abstract

INTRODUCTION

There are several well-described presentations of uterine didelphys (UD): UD without vaginal septum, UD with non-obstructed longitudinal vaginal septum, or UD with duplicated vaginas and an obstructed hemivagina on one side with ipsilateral renal anomaly.

STUDY OBJECTIVE

To describe another variant of UD and compare the presentation and management across different institutions METHODS: This was a retrospective case series approved by the NASPAG Fellows Research Consortium. Participating institutions obtained IRB approval. Inclusion criteria included a diagnosis of UD and unilateral cervicovaginal agenesis/dysgenesis (CVAD). Descriptive statistics were used.

RESULTS

Five patients met the inclusion criteria, with ages ranging from 13 to 27 years. Presenting symptoms included dysmenorrhea (80%), irregular bleeding (40%), acute onset left lower quadrant pain (20%), and abdominal mass (20%). Three patients had additional known abnormalities, including solitary kidney and solitary adrenal gland. All patients underwent pelvic magnetic resonance imaging. Two cases were only suspicious for unilateral CVAD on imaging and required pathology review postoperatively to confirm diagnosis. Two cases required a 2-staged approach with an initial diagnostic surgery followed by a second definitive procedure. Three patients were noted to have endometriosis intraoperatively. Postoperative follow-up ranged from 2 months to 2 years, with 1 patient reporting chronic pelvic pain.

CONCLUSION

Diagnosis on the basis of pelvic imaging can be difficult, as this unique variant may mimic classic obstructed hemivagina with ipsilateral renal anomaly. In patients with UD with unilateral CVAD, standard management is removal of the obstructed uterine horn. This multicenter series stresses awareness about the clinical presentation, distinguishes cases of cervical agenesis from dysgenesis, and reviews approaches to management.

摘要

介绍

子宫双角(UD)有几种明确的表现形式:无阴道隔的 UD、非梗阻性纵向阴道隔的 UD、或双阴道伴单侧梗阻性半阴道和同侧肾异常的 UD。

研究目的

描述 UD 的另一种变体,并比较不同机构的表现和管理方法。

研究方法

这是一项由 NASPAG 研究员研究联合会批准的回顾性病例系列研究。参与机构获得了 IRB 的批准。纳入标准包括 UD 诊断和单侧宫颈阴道发育不全/发育不良(CVAD)。使用描述性统计。

结果

符合纳入标准的患者有 5 例,年龄从 13 岁到 27 岁不等。主要症状包括痛经(80%)、不规则出血(40%)、左下腹疼痛急性发作(20%)和腹部肿块(20%)。3 例患者还有其他已知异常,包括孤立肾和孤立肾上腺。所有患者均行盆腔磁共振成像检查。2 例仅在影像学上疑似单侧 CVAD,术后需行病理检查以明确诊断。2 例需采用 2 期手术方法,先进行诊断性手术,再进行第二次确定性手术。3 例术中发现子宫内膜异位症。术后随访时间为 2 个月至 2 年,1 例患者报告慢性盆腔痛。

结论

基于盆腔影像学的诊断可能较为困难,因为这种独特的变体可能与同侧肾异常的经典梗阻性半阴道相混淆。在 UD 合并单侧 CVAD 的患者中,标准治疗方法是切除梗阻的子宫角。本多中心研究强调了对临床表现的认识,区分了宫颈发育不全与发育不良,并回顾了治疗方法。

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