Anaesthesia and mitochondrial disease

Abstract

Mitochondrial diseases, or encephalomyopathies, are an uncommon, heterogeneous group of disorders with variable clinical course and presentation. Many of these patients present for surgery, or undergo anaesthesia in the course of investigation of their illness. Unfortunately, little information exists on their management in anaesthetic texts and the literature. We report on the anaesthetic management of a paediatric patient with mitochondrial disease, and briefly discuss the pathophysiology and anaesthetic implications of these disorders.

Anesthetic Management of a Child With Unspecified Mitochondrial Disease in an Outpatient Dental Setting

“…Although most anesthetic treatments in patients with MD are without complications, these patients are considered to be at higher risk due to surgical and anesthetic stress. 24 It falls upon the anesthesiologist to purposefully gather a complete and relevant medical history on the patient in question. Thorough preoperative examination, with a focus on neurologic, respiratory, and cardiac function, is imperative, especially for the anes- thesiologist working in a dental office or other outpatient facility (Table 2).…”

“…23 Furthermore, the resultant hypercarbia can contribute to respiratory acidosis, which may be compounded with underlying lactic acidosis. 24 For opioid maintenance in TIVA with spontaneous respiration, a remifentanil infusion is a logical option, given the ultra-short-acting opioid's ability to be titrated to a rate that is conducive to normocarbia. The effects of ketamine on mitochondrial function have been a topic of disagreement in the current literature.…”

The anaesthetic management of temporal tumor resection in a patient with type 1 glutaric aciduria

“…Mitokondri hastalıkları olan çocuklarda aerobik metabolizma da yetersiz olduğundan bazal metabolizma hızını arttırabilecek hipotermi ve ağrı önlenmelidir (15). Olguda ameliyat süresince, özofagus sıcaklığı ölçülüp, sıcak hava üflemeli battaniyeler kullanarak normotermi sağlanmıştır.…”

A Case of Mitochondrial Myopathy with Cardiac Involvement: Management in the Same Day Surgery Setting

“…Review of literature does not indicate an increased susceptibility to MH by patients diagnosed with mitochondrial myopathy [11,12]. It is best to avoid depolarizing muscle relaxants, although these have been used safely in many patients with mitochondrial diseases [13].…”