卡恩斯·塞尔综合征(KSS)上睑下垂的处理:1例报告及文献综述

IF 1.3 Q3 SURGERY Archives of Plastic Surgery-APS Pub Date : 2023-11-09 DOI:10.1055/a-2207-7587
MOULAY OMAR MOUSTAINE, Zakaria Azmour, Mohamed Frarrchi, Othman Benenda, Hicham Nassik, Mahdi Karkouri
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引用次数: 0

摘要

Kearns Sayre综合征(KSS)是一种罕见的线粒体疾病,影响年轻人,由于线粒体DNA的缺失,其特征为:发病年龄低于20岁,慢性进行性外眼麻痹和非典型色素视网膜病变。它的特点还包括其他内分泌,神经系统,尤其是心脏损伤在各种麻醉下的手术过程中心脏并发症的风险非常高。我们报告一例KSS表现为严重的双侧上睑下垂,并通过肌肉活检证实为“粗糙的红色纤维”。在局部麻醉“额神经阻滞”下,采用谨慎的额神经悬吊术治疗上睑下垂。通过这个案例,我们讨论了管理KSS患者所面临的挑战。
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Management of ptosis in Kearns Sayre syndrome (KSS): a case report and Literature review on Management of ptosis in KSS
Kearns Sayre syndrome (KSS) is a rare mitochondrial disease that affects young adults, due to a deletion of mitochondrial DNA and characterized by the triad: age of onset lower than 20 years, chronic progressive external ophthalmoplegia and an atypical pigmentary retinopathy. It’s also characterized by other endocrine, neurological and especially cardiac impairment with a very high risk of cardiac complications during surgical procedures under all types of anesthesia. We report a case of KSS revealed by sever bilateral ptosis and confirmed by a muscle biopsy with “ragged red fibers”. The ptosis was surgically managed by cautious Frontal suspension under local anesthesia “Frontal nerve block”. Through this case we discuss challenges in the management of KSS patients.
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来源期刊
CiteScore
2.10
自引率
6.70%
发文量
131
审稿时长
10 weeks
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