Strabismus and mitochondrial defects in chronic progressive external ophthalmoplegia

American Journal of Ophthalmology
J A SorkinS R Lambert

Abstract

To describe the results of strabismus surgery on three patients with chronic progressive external ophthalmoplegia, a group of rare disorders characterized by ptosis and slowly progressive ophthalmoparesis that has been shown to result from defects in mitochondrial DNA. Strabismus surgery using the adjustable suture technique was performed in three patients with strabismus and chronic progressive external ophthalmoplegia confirmed by clinical, biochemical, histopathologic, and genetic criteria. All three patients had mitochondrial DNA deletions. Two patients were exotropic; one patient was esotropic. Rectus muscle recessions were initially unsuccessful in correcting strabismus in one patient, although a subsequent procedure employing rectus muscle resections was successful in alleviating a significant head turn and improved ocular alignment. In the two other patients, a single procedure consisting of rectus muscle recessions combined with large rectus muscle resections successfully achieved good postoperative alignment. The amount of surgery performed in these three patients exceeded that predicted in standard strabismus tables. The myopathic process that results in chronic progressive external ophthalmoplegia renders rectus mus...Continue Reading

References

Sep 1, 1979·American Journal of Ophthalmology·A Jampolsky
Jan 1, 1977·Annals of Neurology·R A BerenbergL P Rowland
Jan 1, 1990·Acta Neuropathologica·S TakedaF Ikuta

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Citations

Jul 30, 2016·Journal of the Formosan Medical Association = Taiwan Yi Zhi·Chia-Chieh HsiaoTzu-Hsun Tsai
Apr 25, 2006·The Journal of Craniofacial Surgery·Luigi ClauserManlio Galiè
Mar 28, 2018·International Ophthalmology·Klio I ChatzistefanouChryssanthi Koutsandrea
Jan 1, 2003·The American Orthoptic Journal·Ian M MacdonaldBrad Wakeman
Nov 17, 2020·Acta Ophthalmologica·Ji Yeon KimJeong-Min Hwang
Mar 27, 2021·Taiwan Journal of Ophthalmology·Jane H LockNancy J Newman

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