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口服西罗莫司治疗成人眼眶慢血流血管畸形的疗效:容积评估

Efficacy of Oral Sirolimus Therapy for Adult Orbital Slow-Flow Vascular Malformations: A Volumetric Evaluation.

作者信息

Nomura Tadashi, Satake Hiroshi, Hata Yuki, Sakakibara Shunsuke, Terashi Hiroto

机构信息

Department of Plastic Surgery, Kobe University Graduate School of Medicine, Kobe, JPN.

Department of Plastic Surgery, Japan Community Healthcare Organization (JCHO) Osaka Hospital, Osaka, JPN.

出版信息

Cureus. 2024 Oct 14;16(10):e71478. doi: 10.7759/cureus.71478. eCollection 2024 Oct.

DOI:10.7759/cureus.71478
PMID:39544547
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11560402/
Abstract

Orbital lymphatic malformations significantly impair visual function, with inflammation contributing to corneal dryness and diplopia due to exophthalmos. Disease progression may lead to blindness, and in severe cases, exenteration may be required. We treated two symptomatic cases of adult orbital slow-flow vascular malformations with oral sirolimus. Although the patients maintained good visual acuity before treatment, prominent exophthalmos posed a substantial risk to visual function. After medication, eyeball proptosis improved significantly in all cases, with magnetic resonance imaging volumetry analysis showing a reduction of 28.5% to 82.1%. This treatment demonstrated minimal side effects and may serve as an initial therapeutic option.

摘要

眼眶淋巴管畸形会显著损害视觉功能,炎症会导致角膜干燥以及因眼球突出引起复视。疾病进展可能导致失明,严重时可能需要进行眼眶内容剜除术。我们用口服西罗莫司治疗了两例有症状的成人眼眶缓慢血流性血管畸形。尽管患者在治疗前视力良好,但明显的眼球突出对视觉功能构成了重大风险。用药后,所有病例的眼球突出均有显著改善,磁共振成像容积分析显示减少了28.5%至82.1%。该治疗显示出极小的副作用,可作为初始治疗选择。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95db/11560402/bfe226f10788/cureus-0016-00000071478-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95db/11560402/b125fd7acc26/cureus-0016-00000071478-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95db/11560402/bfe226f10788/cureus-0016-00000071478-i02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95db/11560402/b125fd7acc26/cureus-0016-00000071478-i01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/95db/11560402/bfe226f10788/cureus-0016-00000071478-i02.jpg

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本文引用的文献

1
Intraorbital lymphatic-venous malformation in an adult patient: is sirolimus the key?成人眼眶内淋巴血管畸形:西罗莫司是关键吗?
Orbit. 2024 Aug;43(4):535-538. doi: 10.1080/01676830.2023.2214938. Epub 2023 Jun 1.
2
Sirolimus in the Treatment of Microcystic Lymphatic Malformations: A Systematic Review.西罗莫司治疗微囊型淋巴管畸形:系统评价。
Lymphat Res Biol. 2023 Apr;21(2):101-110. doi: 10.1089/lrb.2021.0103. Epub 2022 Jul 18.
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The Use of Sirolimus for Treatment of Orbital Lymphatic Malformations: A Systematic Review.
西罗莫司治疗眼眶淋巴管畸形的应用:系统评价。
Ophthalmic Plast Reconstr Surg. 2020 May/Jun;36(3):215-221. doi: 10.1097/IOP.0000000000001518.
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Efficacy and safety of sirolimus treatment for intractable lymphatic anomalies: A study protocol for an open-label, single-arm, multicenter, prospective study (SILA).西罗莫司治疗顽固性淋巴管畸形的疗效与安全性:一项开放标签、单臂、多中心前瞻性研究(SILA)的研究方案
Regen Ther. 2019 Jan 14;10:84-91. doi: 10.1016/j.reth.2018.12.001. eCollection 2019 Jun.
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Use of percutaneous bleomycin sclerotherapy for orbital lymphatic malformations.经皮注射博来霉素硬化疗法治疗眼眶淋巴管瘤
Orbit. 2019 Feb;38(1):30-36. doi: 10.1080/01676830.2018.1480636. Epub 2018 Jun 14.
6
Sirolimus-induced regression of a large orbital lymphangioma.西罗莫司诱导大型眼眶淋巴管瘤消退
Orbit. 2019 Feb;38(1):79-80. doi: 10.1080/01676830.2018.1436569. Epub 2018 Feb 6.
7
Sirolimus as treatment for 19 patients with refractory cervicofacial lymphatic malformation.西罗莫司治疗19例难治性头颈部淋巴管畸形患者。
Laryngoscope. 2018 Jan;128(1):269-276. doi: 10.1002/lary.26780. Epub 2017 Aug 7.
8
Role of intralesional bleomycin sclerotherapy as the sole or adjunct treatment of superficial ocular adnexal lymphatic malformations.腔内博来霉素硬化治疗作为眼附属器浅表淋巴管畸形的单一或辅助治疗手段的作用。
Eye (Lond). 2018 Jan;32(1):152-155. doi: 10.1038/eye.2017.154. Epub 2017 Aug 4.
9
Lymphatic Malformation in Newborns as the First Sign of Diffuse Lymphangiomatosis: Successful Treatment with Sirolimus.新生儿淋巴管畸形作为弥漫性淋巴管瘤病的首发症状:西罗莫司治疗成功
Neonatology. 2016;109(1):52-5. doi: 10.1159/000440939. Epub 2015 Oct 28.
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AKT hyper-phosphorylation associated with PI3K mutations in lymphatic endothelial cells from a patient with lymphatic malformation.来自一名淋巴管畸形患者的淋巴管内皮细胞中,AKT过度磷酸化与PI3K突变相关。
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