Anesthesia Management of a Patient with Kearn’s – Sayre Syndrome

Cakmak, Bilgesu; Akpek, Elif; Arslan, G.

doi:10.4328/jcam.643

Cited by 4 publications

(2 citation statements)

References 7 publications

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“…General anesthesia should be avoided as much as possible in order to limit the use of halogenated agents that can cause malignant hyperthermia [12,13]. In the event that general anesthesia is unavoidable, the use of total intravenous anesthesia (TIVA) with propofol, fentanyl and alfentanil is preferable with minimal doses of neuromuscular blocker agents [12,13,14].…”

Section: Accepted Manuscriptmentioning

confidence: 99%

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

Moustaine,

Azemour,

Mohammed

et al. 2023

Arch Plast Surg

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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’s 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 sever 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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Section: Accepted Manuscriptmentioning

confidence: 99%

“…Whatever the type of anesthesia, it's necessary to monitor heart rate and respiratory parameters, maintain normothermia and normoglycemia [14,15].…”

Section: Accepted Manuscriptmentioning

confidence: 99%