Surgical and anesthetic management of patients with mitochondrial dysfunction.

Mitochondrial diseases are primary defects in bio-energy metabolism. They are a heterogeneous group of diseases. The specific constellation of symptoms that will manifest in the effected individual depends on the type of genetic defect in the mitochondria. The goals of medical management, expressed in a highly simplified fashion, are to maintain a normal acid-base balance and to avoid those medical conditions and situations that add extra stress to a compromised bio-energy production system. Infection is a common stress that may cause a crisis in a patient with mitochondrial dysfunction. Treatment and avoidance of infections is a goal of both medical and surgical management. Common childhood illness such as otitis media, tonsillitis and sinusitis are example of such stress inducing infections.