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蓝色橡皮疱痣综合征孕妇的分娩管理:一例报告及文献综述

Delivery management of pregnant blue rubber bleb nevus syndrome patients: a case report and literature review.

作者信息

Nagahama Mie, Chigusa Yoshitsugu, Shiraki Atsuko, Takei Yui, Yamaguchi Ayaka, Mizota Toshiyuki, Komatsu Maya, Nomura Takashi, Egi Moritoki, Mandai Masaki, Mogami Haruta

机构信息

Department of Gynecology and Obstetrics, Graduate School of Medicine, Kyoto University, Kyoto, Japan.

Department of Anesthesia, Kyoto University Hospital, Kyoto, Japan.

出版信息

Nagoya J Med Sci. 2024 Aug;86(3):507-513. doi: 10.18999/nagjms.86.3.507.

Abstract

Blue rubber bleb nevus syndrome (BRBNS) is a rare systemic vascular disorder characterized by multifocal venous malformations (VMs). Little is known about the perinatal management of pregnant women with BRBNS owing to the limited number of reported cases. We present the case of a 36-year-old primigravida with BRBNS who underwent an uneventful cesarean section under spinal anesthesia for breech presentation. A thorough systemic examination revealed VMs in various organs, including the skin, conjunctiva, larynx, gastrointestinal tract, lungs, and vulva. Prior to spinal anesthesia, careful examination using imaging modalities was conducted to assess the spinal and epidural involvement of the VMs to avoid complications, including accidental puncture of the VMs, associated bleeding, and epidural hematoma. In pregnant women with BBNS, it is imperative to scrutinize the localization and distribution of VMs throughout the body to anticipate potential complications and select the appropriate delivery mode and anesthetic management.

摘要

蓝色橡皮疱痣综合征(BRBNS)是一种罕见的系统性血管疾病,其特征为多灶性静脉畸形(VMs)。由于报道的病例数量有限,关于患有BRBNS的孕妇的围产期管理知之甚少。我们报告一例36岁初产妇患有BRBNS,因臀位在脊髓麻醉下行剖宫产术,过程顺利。全面的系统检查发现其多个器官存在静脉畸形,包括皮肤、结膜、喉部、胃肠道、肺和外阴。在脊髓麻醉前,使用影像学检查手段进行仔细检查,以评估静脉畸形在脊柱和硬膜外的累及情况,避免出现包括意外穿刺静脉畸形、相关出血和硬膜外血肿等并发症。对于患有BRBNS的孕妇,必须仔细检查全身静脉畸形的定位和分布情况,以预测潜在并发症,并选择合适的分娩方式和麻醉管理方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/4c1e/11439602/bd92c3febb25/2186-3326-86-0507-g001.jpg

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