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

TL;DR

This paper presents a case of Kearns–Sayre syndrome revealed by ptosis and discusses its surgical management under local anesthesia.

Contribution

The paper contributes a case report and management strategy for ptosis in KSS using local anesthesia to avoid cardiac risks.

Findings

KSS was diagnosed via muscle biopsy showing 'ragged red fibers'.

Ptosis was successfully managed with a frontal suspension under local anesthesia.

The case highlights the need for cautious surgical approaches in KSS due to cardiac risks.

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.

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