Mahler O, Milstein A, Pollack A, Hauben D
Eye Dept., Kaplan Medical Center, Rehovot.
Harefuah. 2000 Dec;139(11-12):434-7, 494.
In some muscular dystrophies there is ocular involvement characterized by blepharoptosis and ophthalmoplegia. These conditions occur in chronic progressive external ophthalmoplegia, oculopharyngeal muscular dystrophy, mitochondrial myopathy, myotonic dystrophy, and ocular myasthenia, among others. Although they differ in their systemic clinical manifestations and in genetic inheritance, ocular involvement is common to all of them. Manifestations include bilateral progressive blepharoptosis with or without extraocular muscle malfunction. During surgical repair of the ptotic eyelid, consideration must be given to eyeball movements, in addition to maximal eyelid elevation, and to avoiding overcorrection and consequent corneal overexposure, leading to dryness and visual impairment. With these muscular dystrophic disorders, resection of the levator muscle or blepharoplasty alone does not suffice. Follow-up shows that most patients need a secondary repair after a short while. Operative correction uses a frontalis sling for eyelid elevation and support. A series of 8 patients with these diseases, operated on by various surgical techniques during the past 7 years, is presented.
在某些肌肉营养不良症中,存在以睑下垂和眼肌麻痹为特征的眼部受累情况。这些病症见于慢性进行性外眼肌麻痹、眼咽型肌营养不良症、线粒体肌病、强直性肌营养不良症以及眼肌型重症肌无力等。尽管它们在全身临床表现和遗传方式上有所不同,但眼部受累在所有这些病症中都很常见。表现包括伴有或不伴有眼外肌功能障碍的双侧进行性睑下垂。在对下垂眼睑进行手术修复时,除了要使眼睑最大限度上抬外,还必须考虑眼球运动,并避免过度矫正以及由此导致的角膜过度暴露,进而引起干燥和视力损害。对于这些肌肉营养不良性疾病,单纯切除提上睑肌或进行睑成形术是不够的。随访显示,大多数患者在短时间后需要二次修复。手术矫正采用额肌悬吊术来提升和支撑眼睑。本文介绍了过去7年中采用各种手术技术对8例患有这些疾病的患者进行手术的情况。