title: Anesthesia-related morbidity and mortality after surgery for muscle biopsy in children with mitochondrial defects.
abstract: BACKGROUND
  Children with mitochondrial defects (MD) may have an increased risk for cardiorespiratory and neurological complications from anesthesia. The aim of this study was to determine the incidence of perioperative complications and adverse events in children with MD.
  
  
  METHODS
  We performed a retrospective review of the anesthesia, surgical and medical records of 155 children up to 10 years who underwent a diagnostic surgical muscle biopsy for suspected mitochondrial and muscle disorders between 1999 and 2003. The data of the 122 patients where a definite MD diagnosis was found were analyzed. Anesthesia was conducted according to the discretion of the attending staff. In children with MD the activity of the different complexes of the oxidative phosphorylation system was measured.
  
  
  RESULTS
  From the preoperative assessment, signs of encephalopathy were most frequent (n = 93), followed by muscle weakness (n = 32), lactic acidosis (n = 15), cardiomyopathy and/or conduction defects (n = 10) and chronic respiratory problems (n = 7). The mean age of the children with MD was 32.4 months (SD = 26.9). The mean duration of anesthesia was 36.0 min (SD = 12.6) and the mean length of stay in the recovery room was 33.0 min (SD = 24.0). There were no major changes in heart rate or blood pressure which required pharmacological intervention during anesthesia or postanesthesia care unit (PACU) stay. A short episode of SpO2 <80% with airway obstruction occurred once in the PACU. Biochemical analysis in the children with a MD showed an isolated deficiency of one of the five protein complexes of oxidative phosphorylation in 42 children, a combination of complex deficiencies in 41 and no definite localization in 39 children.
  
  
  CONCLUSIONS
  With standard preoperative assessment, monitoring and anesthesia management, there were no major peroperative and postoperative anesthesia-related complications in children undergoing surgical muscle biopsy with a MD diagnosis.
authors.affiliation: Department of Anesthesiology, Radboud University Medical Center, Nijmegen, The Netherlands. jj.driessen@anes.umcn.nl
authors.forename: Jacques
authors.initials: J
authors.lastname: Driessen
authors.forename: Simone
authors.initials: S
authors.lastname: Willems
authors.forename: Sander
authors.initials: S
authors.lastname: Dercksen
authors.forename: Janneke
authors.initials: J
authors.lastname: Giele
authors.forename: Frans
authors.initials: F
authors.lastname: van der Staak
authors.forename: Jan
authors.initials: J
authors.lastname: Smeitink
mesh_terms: D000758:Anesthesia / Q000009:adverse effects*; D001706:Biopsy / Q000379:methods; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D007902:Length of Stay; D008297:Male; D028361:Mitochondrial Diseases / Q000175:diagnosis* / Q000473:pathology; D018482:Muscle, Skeletal / Q000473:pathology*; D011183:Postoperative Complications / Q000453:epidemiology / Q000209:etiology*; D012189:Retrospective Studies; D012307:Risk Factors; D013997:Time Factors