title: Anesthetic management of a pediatric patient with leigh syndrome.
abstract: OBJECTIVE
  Leigh syndrome (LS) is a rare disease caused by abnormalities of mitochondrial energy generation. The central nervous system is most frequently affected, with psychomotor underdevelopment, seizures, nystagmus, ophthalmoparesis, optic atrophy, ataxia, dystonia, or respiratory failure. Surgical and anesthetic procedures stimulate the tracheal irritability, and could exacerbate risks of aspiration, wheezing, breathing difficulties, gasping, hypoventilation, and apnea.
  
  
  METHODS
  We present the anesthetic management for a six-year-old boy with severe form of LS, involving repair of a femur fracture. Propofol and remifentanil were infused for general anesthesia. The patient was closely monitored during anesthesia and in the intensive care unit in the early postoperative period.
  
  
  CONCLUSIONS
  Close intraoperative monitoring of patients, including invasive arterial blood pressure monitoring and frequently measuring the levels of blood gases, glucose, and lactate, made this procedure run smoothly. Intensive care and breathing support for the patient with LS, under sedation with an analgesic combination during the early postoperative period, minimized the stress response due to pain after surgery.
authors.affiliation: MD; Associate Professor, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, Turkey. Electronic address: serhatk@omu.edu.tr.
authors.forename: Ismail Serhat
authors.initials: IS
authors.lastname: Kocamanoglu
authors.affiliation: MR, Department of Anesthesia, Ondokuz Mayis School of Medicine, Samsun, Turkey.
authors.forename: Esra
authors.initials: E
authors.lastname: Sarihasan
mesh_terms: D000768:Anesthesia, General; D018686:Anesthetics, Intravenous / Q000008:administration & dosage*; D002648:Child; D005264:Femoral Fractures / Q000150:complications / Q000601:surgery*; D006801:Humans; D007888:Leigh Disease / Q000150:complications; D008297:Male; D010880:Piperidines / Q000008:administration & dosage*; D015742:Propofol / Q000008:administration & dosage*; D000077208:Remifentanil
chemical_list: D018686:Anesthetics, Intravenous; D010880:Piperidines; D000077208:Remifentanil; D015742:Propofol
keywords: Anesthesia; General; Intensive Care; Leigh Disease; Monitoring; Physiologic