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

Abstract

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; chronic progressive external ophthalmoplegia; fascia lata lid suspension.

Figure 1

Severe eyelid ptosis and severe reduction of eye movements in a patient with Kearns-Sayre syndrome. Notes: (A) Primary gaze position, (B) upper eye gaze, (C) downward eye gaze, and (D) eyelid occlusion.

Figure 2

Eyelid-brow suspension with fascia lata. Notes: (A) A strip of fascia (60 mm ×8 mm) was divided into four thin strips of fascia measuring 60 mm ×2 mm. The strips were then sutured with 5-0 Nylon to the upper anterior part of each tarsus. (B) The fascia strips were passed beneath the orbicularis muscle. (C) They were sutured with 5-0 Nylon to the frontalis muscle above the brow in the manner of a “W”. The central arm of the “W” determined the height of the lid margin and the other two arms of the W were placed to regulate lid contour.

Figure 3

Corneal ulceration and leukoma caused by chronic corneal exposition.

Figure 4

Preoperative and postoperative appearance of a 34-year-old female patient. Notes: (A) Preoperative and (B) 6-month postoperative photographs. (C) Preoperative eyelid closure and (D) postoperative.

Figure 5

Preoperative and postoperative appearance of a 61-year-old female patient. Notes: (A) Preoperative and (B) 1-year postoperative photographs. (C) Perfect preoperative eyelid closure. (D) Postoperative photograph showing a mild lid-lag lagophthalmos.