Abstract
We describe the anesthesia management of a 15-year-old girl, diagnosed with mitochondrial neurogastrointestinal encephalomyopathy (MNGIE), who presented with gastrointestinal perforation. MNGIE is an autosomal recessive disease associated with multiple deletions and depletion of mitochondrial DNA in skeletal muscle. The disease is characterized clinically by ptosis, progressive external ophthalmoparesis, severe gastrointestinal dysmotility, peripheral neuropathy, and leukoencephalopathy. We report on the anaesthetic management of a paediatric patient with MNGIE, and briefly discuss the pathophysiology and anaesthetic implications of this disorder. We describe the successful administration of a general anesthesia without muscle relaxants, maintenance in a patient with MNGIE.
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