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

Abstract

BackgroundThe 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.MethodsThis 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.ResultsThe 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.ConclusionThe 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.

Argus II retinal prosthesis implantation with scleral flap and autogenous temporalis fascia as alternative patch graft material: a 4-year follow-up

“…Fascia sampled from other sites, such as fascia lata, are also often employed for eyelid surgery 16–18. Temporalis fascia is a double layer of loose connective tissue.…”

“… 15 Fascia sampled from other sites, such as fascia lata, are also often employed for eyelid surgery. 16 – 18 Temporalis fascia is a double layer of loose connective tissue. The superficial fascia lies directly below the fatty layer and hair follicles of the scalp; removal of a fragment of superficial fascia is not reversible but is devoid of any functional consequences.…”

Clinical-Radiologic Correlation of Extraocular Eye Movement Disorders: Seeing beneath the Surface

“…CPEO is a mitochondrial disorder that results in progressive myopathy and atrophy of the EOMs and muscles of the eyelid. The most severe form of CPEO is Kearns-Sayre syndrome, a mitochondrial encephalomyopathy resulting in CPEO, bilateral pigmentary retinopathy, ataxia, and cardiac conduction abnormalities (58). Kearns-Sayre syndrome is a noninherited condition that results from a somatic mutation resulting in impaired oxidative phosphorylation within the mitochondria.…”

Ophthalmic manifestations suggesting Kearns--Sayre Syndrome among young adults with cardiac conduction defects (RCD code: III-1A.5a)

“…Management of ptosis is always surgical. The most frequently recommended correction procedure is lid brow suspension with the use of either silicone tape or fascia lata autograft [18][19][20].…”