title: Leigh syndrome: anesthetic management in complicated endoscopic procedures.
abstract: BACKGROUND
  Leigh's syndrome, a disorder of infancy and childhood, is characterized by gray matter degeneration and focal brainstem necrosis. It presents with special clinical features such as developmental delay, nervous system dysfunction, respiratory abnormalities, and hypertrophic cardiomyopathy that can be a real challenge to the anesthesiologist. Anesthesia or sedation has rarely been reported in patients with Leigh disease. We report our experience in sedating five children with Leigh syndrome in seven procedures undertaken in the endoscopy suite (outside the operating room).
  
  
  METHODS
  Five children with Leigh disease, three girls and two boys, have been referred to us for percutaneous endoscopic gastrostomy (PEG) insertion and or replacement (a total of seven procedures). The average age was 2.6 years with a range of 4 months to 6 years. Informed consent was obtained from the patient's parents or guardian. An anesthesia machine, scavenging system, O(2) source and routine monitoring were available. Sedation was accomplished with propofol intravenous (i.v.) (0.5-1 mg x kg(-1)) maintained with a propofol infusion (50-100 microg x kg(-1) x min(-1)). The spontaneously breathing patients received oxygen through an oxygen facemask during the procedure and afterwards recovery was managed in the gastroenterology unit.
  
  
  RESULTS
  All the children underwent the procedure without complications. One patient developed transient desaturation (SpO(2) 80%) for a few seconds. Body temperature, heart rate, arterial blood pressure, O(2) saturation and endtidal CO(2) were stable during the endoscopies. No special post-procedure management was required; the patients woke up at the end of the endoscopy and were able to drink and eat as usual.
  
  
  CONCLUSIONS
  This rare mitochondrial disease presents unique management problems to the anesthesiologist when using general anesthesia. Our patients were managed appropriately before endoscopy and underwent the procedure under deep sedation. No complications occurred. We concluded that deep sedation in the endoscopy suite was safe in this small series of patients with this rare disease.
authors.affiliation: Department of Pediatric Sedation Service, Anesthesiology, Hadassah University Hospital, Jerusalem, Israel.
authors.forename: David
authors.initials: D
authors.lastname: Gozal
authors.forename: Eran
authors.initials: E
authors.lastname: Goldin
authors.forename: Sigal
authors.initials: S
authors.lastname: Shafran-Tikva
authors.forename: Dalia
authors.initials: D
authors.lastname: Tal
authors.forename: Dov
authors.initials: D
authors.lastname: Wengrower
mesh_terms: D018686:Anesthetics, Intravenous; D002648:Child; D002675:Child, Preschool; D016292:Procedural Sedation / Q000379:methods*; D016099:Endoscopy, Gastrointestinal / Q000379:methods*; D005260:Female; D005774:Gastrostomy / Q000379:methods*; D006801:Humans; D007223:Infant; D007888:Leigh Disease / Q000503:physiopathology / Q000601:surgery*; D008297:Male; D015742:Propofol
chemical_list: D018686:Anesthetics, Intravenous; D015742:Propofol