Anesthetic management for a patient with Kearns-Sayre syndrome.

Introduction Herein, we describe the interesting case of a 13-year-old female with Kearns-Sayre syndrome (KSS) who underwent a successful T3 ~ L3 posterior spinal instrumentation and fusion (PSIF). KSS is a rare mitochondrial myopathy with only 226 cases reported in published literature as of 1992. It is the result of deletions in mitochondrial DNA and manifests as a triad of: 1) chronic progressive external ophthalmoplegia 2) bilateral pigmentary retinopathy 3) cardiac conduction abnormalities.