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针对线粒体细胞病——卡恩斯-塞尔综合征中的严重上睑下垂的双侧眼睑/眉提升手术。

Bilateral lid/brow elevation procedure for severe ptosis in Kearns-Sayre syndrome, a mitochondrial cytopathy.

作者信息

Sebastiá Roberto, Fallico Ester, Fallico Matteo, Fortuna Eduardo, Lessa Sergio, Neto Guilherme Herzog

机构信息

Department of Ophthalmology, Fluminense Federal University, Niterói, Brazil ; Oculoplastic Surgery Department, 38th Infirmary of the Santa Casa de Misericordia do Rio de Janeiro, Instituto Ivo Pitanguy, Pontifícia Universidade Catolica do Rio de Janeiro, Rio de Janeiro, Brazil.

Clinica Gretter, Università degli Studi di Catania, Catania, Italy.

出版信息

Clin Ophthalmol. 2014 Dec 22;9:25-31. doi: 10.2147/OPTH.S74179. eCollection 2015.

DOI:10.2147/OPTH.S74179
PMID:25565765
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC4278785/
Abstract

BACKGROUND

The purpose of this work was to determine the effectiveness and possible complications encountered with bilateral fascia lata lid suspension used to correct blepharoptosis in patients with Kearns-Sayre syndrome.

METHODS

This was a retrospective study of seven patients with Kearns-Sayre syndrome who had a minimum of 1 year of follow-up. A bilateral fascia lata sling was used to correct the ptosis. Preoperative and postoperative measurements of the vertical lid fissure width (VFW) and marginal reflex distance (MRD) were performed. The Student's t-test was used to analyze the results.

RESULTS

The mean preoperative VFW and MRD measurements were 4±2.45 mm and 0.14±0.92 mm, respectively. The mean postoperative VFW and MRD measurements were 7.71±1.85 mm, and 2.86±1.69 mm, respectively. All preoperative and postoperative values were considered to be statistically significant (P<0.01). Adequate elevation of the lids was obtained in all patients, both functionally and aesthetically. All of the patients showed a mild symmetric postoperative inferior version lagophthalmos, and one patient developed corneal ulceration and scarring due to corneal exposure and a weak Bell's phenomenon.

CONCLUSION

The surgical technique described to correct the blepharoptosis found in patients with Kearns-Sayre syndrome was found to be efficient and relatively safe. The correction should be conservative to decrease the risk of postoperative corneal damage that occurred in one patient.

摘要

背景

本研究旨在确定采用双侧阔筋膜眼睑悬吊术矫正凯-赛综合征患者上睑下垂的有效性及可能出现的并发症。

方法

这是一项对7例凯-赛综合征患者进行的回顾性研究,患者均至少随访1年。采用双侧阔筋膜吊带矫正上睑下垂。术前及术后测量垂直睑裂宽度(VFW)和边缘反射距离(MRD)。采用学生t检验分析结果。

结果

术前VFW和MRD的平均测量值分别为4±2.45mm和0.14±0.92mm。术后VFW和MRD的平均测量值分别为7.71±1.85mm和2.86±1.69mm。术前和术后的所有数值均具有统计学意义(P<0.01)。所有患者在功能和美学上均获得了足够的眼睑提升。所有患者术后均出现轻度对称性下睑外翻性兔眼,1例患者因角膜暴露和贝尔现象减弱而发生角膜溃疡和瘢痕形成。

结论

所描述的用于矫正凯-赛综合征患者上睑下垂的手术技术被发现是有效且相对安全的。矫正应保守进行,以降低1例患者术后发生角膜损伤的风险。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/01cba9f2056f/opth-9-025Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/3cdc43693edc/opth-9-025Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/a7f8b44d1799/opth-9-025Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/92fcf29a9d55/opth-9-025Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/04a32b76bf71/opth-9-025Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/01cba9f2056f/opth-9-025Fig5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/3cdc43693edc/opth-9-025Fig1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/a7f8b44d1799/opth-9-025Fig2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/92fcf29a9d55/opth-9-025Fig3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/04a32b76bf71/opth-9-025Fig4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/eec2/4278785/01cba9f2056f/opth-9-025Fig5.jpg

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Macular lesion resembling adult-onset vitelliform macular dystrophy in Kearns-Sayre syndrome with multiple mtDNA deletions.Kearns-Sayre 综合征伴多发性 mtDNA 缺失的黄斑病变类似于成人发病型卵黄样黄斑营养不良。
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