Mitochondrial disease that primarily affects the retina and the optic nerve (atrophy).

Caused by mutations in mitochondrial DNA. Affects more males. Possible onset at any age, generally end of first decade.

Sometimes associated with cardiac conduction disturbances.

Anesthetic implications:

preoperative ECG and echocardiography; management of a mitochondrial cytopathy (see this topic). Avoid the use of propofol-based total IV anesthesia.

References: 

--        Vanlander AV, Jorens PG, Smet J, De Paepe B et al.
Inborn oxidative phosphorylation defect as risk factor for propofol infusion syndrome.
Acta Anaesthesiol Scand 2012; 56:520-5.

-        Niezgoda J, Morgan PG.
Anesthetic considerations in patients with mitochondrial defects.
Pediatr Anesth 2013; 23: 785-93.

-        Nouette-Gaulain K, Robin F, Semjen F, Obre E, Bellance N, Biais M, Rossignol R.
Cytopathies mitochondriales et anesthésie.
Anesth Réanim 2016 ; 2 : 300-8.

-        Smith A, Dunne E, Mannion M, O’Connor C, Knerr I et al.
A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders.
Eur J Pediatr 2017; 176: 83-8

Updated: February 2019