Anaesthetic Management of Autosomal Recessive Progressive External Ophthalmoplegia (AR-PEO)  Patient in Emergency Operating Room (OR).
-A case report-

Galacho, J., Caldeira. A., Santos. J..

Hospital Santa Maria – Centro Hospital Lisboa Norte

Background
AR-PEO is a mithocondrial disease (MD) characterized by weakness of the eye muscles, with ptosis and ophtalmoplegia. Additional signs and symptoms can include myopathy, sensorineural hearing loss, neuropathy and ataxia. 
A careful approach is needed during anaesthesia because serious complications and altered sensivity to anesthetic agents/muscle relaxants can occur.

Case report
A 10-year-old girl with respiratory infection was admitted in the emergency OR for replacement of central venous catheter needed for antibiotherapy.
At 7 years of age, she was evaluated  for ptosis, limited ocular mobility  and  gait instability. During the pediatric evaluation, physical development was noted to be delayed and she was taking medication to control epileptic seizures. The muscle biopsy showed ragged red fibers and cytochrome-c oxidase-negative fibers  which is compatible with MD. The POLG gene study identifies a variant c.911T>G, compatible with PEO. The cardiac evaluation showed a small mitral valve prolapse.
A brief procedural sedation was planned with ketamine and monitoring of the ECG, non-invasive blood pressure, SpO2, EtCO2 and temperature. Glicemia monitoring and use of lactate-free solution were also contemplated.
Pre-medication with Midazolam 1 mg IV was given and then anaesthesia was conducted with 30 mg of Ketamine.
The vital signs were stable during the intervention, with a oxygen at 5.0 L/min by face mask. Dantrolene was immediately available.
Other than the need to aspirate excessive salivar secretion, there were no relevant occurrences during the 20-minute intervention.
The patient was discharged from the Post-anaesthesia Care Unit 1 hour after procedure, without any complications.


Discussion
The anaesthetic implication in MD and PEO is an increased susceptibility for malignant hyperthermia, therefore avoidance of triggering agents and monitoring are recomended, and lactate solution is better to avoid, as in our case. 
Pre-operative evaluation with focus on cardiac, respiratory, neurologic, endocrine and metabolic compromise is needed.
Shivering, hypoxia, fasting, hypotension and post-operative pain should be avoided because mitochondrial function may be depressed and lactic acidosis may be exacerbated in conditions which metabolic demand is increasing.


References
-Genetics Home Reference- https://ghr.nlm.nih.gov/condition/progressive-external-ophthalmoplegia
-Anaesthesia recommendations for patients suferring from Mitochondrial Cytopathy, Mitochondrial Myopathy, Mitochondrial Encephalomyopathy – orphananesthesia

Learning points
Anaesthetic management of patients with MD is challenging because it can affect several organ system.

Anaesthetic management of a pediatric patient with Autosomal Recessive Progressive External Ophthalmoplegia (AR-PEO) in the emergency operating room: a case report