Anaesthetic management of a patient with Leigh’s syndrome

Outpatient Anesthesia for Oral Surgery in a Juvenile With Leigh Disease

“…12 The most common problems are irregular respiration, apnea, sighing, sobbing, gasping, stridor, and episodes of hyperventilation. 5,10 A patient with respiratory symptoms before surgery that involved general anesthesia is likely to encounter postoperative respiratory complications or respiratory failure. 10 Grattan-Smith et al 12 described 3 young patients who developed respiratory failure after general anesthesia for diagnostic radiography.…”

“…5,10 A patient with respiratory symptoms before surgery that involved general anesthesia is likely to encounter postoperative respiratory complications or respiratory failure. 10 Grattan-Smith et al 12 described 3 young patients who developed respiratory failure after general anesthesia for diagnostic radiography. All 3 had preoperative respiratory abnormalities, such as asthma, stridor, choking fits, and sighing respiration.…”

“…7 Leigh disease is thought to be attributable to disorders of energy metabolism, specifically, mitochondrial respiratory chain defects and deficiencies of the pyruvate dehydrogenase complex. 10 These abnormalities elevate pyruvate levels in the serum and cerebrospinal fluid and also instigate lactic acidosis, all of which are defining clinical characteristics of the disease. 2,10 Recurring focal lesions suggest increasingly insufficient energy supply at these areas as the disease progresses.…”

“…10 These abnormalities elevate pyruvate levels in the serum and cerebrospinal fluid and also instigate lactic acidosis, all of which are defining clinical characteristics of the disease. 2,10 Recurring focal lesions suggest increasingly insufficient energy supply at these areas as the disease progresses. 7 A study by Howell et al 11 reports that a mutation of mitochondrial DNA may be causatively associated with the presentation of the disease.…”

“…Acute intensification of symptoms is a possibility after surgical procedures, and respiratory distress is the most frequent source of postoperative morbidity and mortality. 10,13 General anesthesia has been documented in some instances to precipitate respiratory failure. 12,13 However, respiratory problems tend to decrease in occurrence with increasing age of disease onset, 5 diminishing the risk for surgical operations in juvenile cases.…”