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

Abstract

Roberto Sebastiá,1,2 Ester Fallico,3 Matteo Fallico,4 Eduardo Fortuna,5 Sergio Lessa,2,6 Guilherme Herzog Neto1 1Department of Ophthalmology, Fluminense Federal University, Niterói, 2Oculoplastic 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; 3Clinica Gretter, 4Facoltà di Medicina e Chirurgia, Università degli Studi di Catania, Catania, Italy; 5Hospital da Plástica, 6Post-Graduate Program in Physiopathology in Surgical Science, State University of Rio de Janeiro, Rio de Janeiro, Brazil 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. Keywords: Kearns-Sayre syndrome, blepharoptosis, fascia lata lid suspension, chronic progressive external ophthalmoplegia