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. 2024 Apr 8;51(2):182-186.
doi: 10.1055/a-2207-7587. eCollection 2024 Mar.

Management of Ptosis in Kearns-Sayre Syndrome: A Case Report and Literature Review

Moulay O Moustaine  1 , Zakaria Azemour  1 , Frarchi Mohammed  1 , Othman Benlanda  2 , Hicham Nassik  2 , Mehdi Karkouri  3
Affiliations

Management of Ptosis in Kearns-Sayre Syndrome: A Case Report and Literature Review

Moulay O Moustaine et al. Arch Plast Surg. .

Abstract

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease that affects young adults, due to a deletion of mitochondrial DNA and characterized by the triad: age of onset lower than 20 years, chronic progressive external ophthalmoplegia, and an atypical pigmentary retinopathy. It is also characterized by other endocrine, neurological, and especially cardiac impairment with a very high risk of cardiac complications during surgical procedures under all types of anesthesia. We report a case of KSS revealed by severe bilateral ptosis and confirmed by a muscle biopsy with "ragged red fibers." The ptosis was surgically managed by cautious Frontal suspension under local anesthesia "Frontal nerve block." Through this case, we discuss challenges in the management of KSS patients.

Keywords: Frontal suspension; Kearns–Sayre syndrome; chronic progressive external ophthalmoplegia; ptosis.

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Conflict of interest statement

Conflict of Interest None declared.

Figures

Fig. 1.
Fig. 1.
Bilateral severe ptosis more marked in the left eye with forehead wrinkling.
Fig. 2.
Fig. 2.
Bilateral external ophthalmoplegia.
Fig. 3.
Fig. 3.
Fundus ( A ) and fluorescein angiography photography ( B ) showed an atypical pigmentary retinopathy with a “salt and pepper” appearance.
Fig. 4.
Fig. 4.
Biopsy of deltoid muscle reveals. ( A ) “Ragged red fibers” on Gomori staining (grossissement × 40). ( B ) Muscle fibers with a defective cytochrome C oxidative activity on coloration hematein–eosin (grossissement × 40).
Fig. 5.
Fig. 5.
Palpebral suspension using the Fox procedure.
Fig. 6.
Fig. 6.
( A ) Preoperative appearance. ( B ) Appearance, 5 days after surgery. ( C ) Appearance, 3 months after surgery.
See this image and copyright information in PMC

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