Zhu Lan, Zhao Zichen, Di Spiezio Sardo Attilio, Acién Maribel, Naftalin Joel, Van den Bosch Thierry, Cheung Charleen Sze-Yan, Xu Dabao, Huang Xiaowu, Grimbizis Grigoris
Department of Obstetrics and Gynecology, National Clinical Research Center for Obstetric and Gynecological Diseases, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Department of Obstetrics and Gynecology, Università degli Studi di Napoli “Federico II”, Naples, Italy
Facts Views Vis Obgyn. 2025 Jun 27;17(2):157-169. doi: 10.52054/FVVO.2025.62. Epub 2025 Jun 11.
Accessory cavitated uterine malformations (ACUMs) are a rare obstructive uterine anomaly that remains poorly understood, posing challenges for clinical management. The aetiopathogenesis is hypothesised to involve the duplication and persistence of ductal Müllerian tissue usually near the round ligament attachment, potentially related to gubernaculum dysfunction. ACUM is specifically classified by Acién's system, though rare variants necessitate continued international research to refine classification frameworks.
This consensus aims to develop good clinical practice recommendations for the pathophysiology, terminology, clinical presentation, diagnosis, and treatment of ACUM.
A working group consisted of Chinese and European experts, after approval from the European Society for Gynaecological Endoscopy, developed recommendations based on the best available evidence and experts' opinion.
Patients with ACUM present with typical symptoms such as dysmenorrhea and dyspareunia, and atypical symptoms, including gastrointestinal and generalised pelvic pain. Diagnostic criteria include isolated cavitated lesions in the anterolateral myometrium near the round ligament, lined by endometrial tissue and filled with haemorrhagic fluid, surrounded by a myometrial mantle with concentric orientation of myometrial fibres, and typically associated with a normal uterine cavity. Diagnosis is most accurately made through ultrasound and magnetic resonance imaging. Surgical excision of the ACUM is considered the definitive treatment offering near-complete symptom resolution, and minimally invasive approach should be preferred when possible. The timing of surgery and the interval before attempting pregnancy remain unclear. The mode of delivery post-surgery is individualised based on the degree of myometrial involvement.
The current consensus summarises the existing evidence on ACUM providing good clinical practice recommendations for their management. Existing gaps in the understanding and management of ACUMs, highlight the need for further research to guide clinical decision-making.
WHAT IS NEW?: Good clinical practice recommendations for ACUM aiming to understand and optimise their management.
附属性空化子宫畸形(ACUMs)是一种罕见的阻塞性子宫异常,目前仍了解不足,给临床管理带来挑战。其发病机制据推测涉及通常在圆韧带附着处附近的苗勒管组织的重复和持续存在,可能与 gubernaculum 功能障碍有关。ACUM 具体按照阿西恩系统进行分类,不过罕见变体需要持续的国际研究来完善分类框架。
本共识旨在为 ACUM 的病理生理学、术语、临床表现、诊断和治疗制定良好临床实践建议。
一个由中国和欧洲专家组成的工作组在获得欧洲妇科内镜学会批准后,基于现有最佳证据和专家意见制定了建议。
ACUM 患者表现出痛经和性交困难等典型症状,以及包括胃肠道和广泛性盆腔疼痛在内的非典型症状。诊断标准包括在圆韧带附近前外侧肌层中孤立的空化病变,内衬子宫内膜组织并充满血性液体,周围有一层肌层,肌层纤维呈同心排列,且通常与正常子宫腔相关。通过超声和磁共振成像能最准确地做出诊断。ACUM 的手术切除被认为是能使症状几乎完全缓解的确定性治疗方法,可能的情况下应首选微创方法。手术时机和尝试怀孕前的间隔时间仍不明确。术后分娩方式根据肌层受累程度个体化确定。
当前共识总结了关于 ACUM 的现有证据,为其管理提供了良好临床实践建议。ACUM 在理解和管理方面存在的现有差距凸显了进一步研究以指导临床决策的必要性。
新内容是什么?:针对 ACUM 的良好临床实践建议,旨在理解并优化其管理。