title: Anesthetic and perioperative risk in the patient with Ataxia-Telangiectasia.
abstract: OBJECTIVE
  To report our relatively large experience with perioperative care for patients with Ataxia-Telangiectasia (A-T) and to identify the nature and frequency of complications.
  
  
  BACKGROUND
  Ataxia-Telangiectasia is a rare autosomal recessive genetic disorder resulting in progressive multisystem degeneration and characteristic findings including complex neurodegeneration, immunodeficiency, increased risk of malignancy, and lung disease. Anecdotal reports have suggested high perioperative morbidity in patients with A-T, but few data exist.
  
  
  METHODS
  The Ataxia-Telangiectasia Clinical Center database was cross-referenced with operative records between 1995 and 2009 to identify patients with perioperative A-T, and medical records were reviewed for preoperative history, management techniques, and complications.
  
  
  RESULTS
  Twenty-one patients with A-T underwent 34 anesthetics during the study period. The median age was 12.5 years (range 6-33 years). Common comorbidities included neurologic (100%), pulmonary (68%), immunologic (50%), oncologic (47%), and gastroenterologic (35%) disorders. Supplemental oxygen was required on postanesthesia care unit discharge for 24% of patients with a maximal duration of 24 h. Although mild postoperative hypothermia was relatively common (44% of anesthetics), there were no major complications, no unplanned admissions, and no mortality in this series.
  
  
  CONCLUSIONS
  Although limited by its retrospective nature, this is the first series describing perioperative risk for patients with A-T. Our results indicate that general anesthesia, airway manipulation, and perioperative mechanical ventilation may be tolerated with only minor postoperative anesthetic concerns. Perioperative providers should be aware of the complex multisystem medical concerns that may arise in these patients.
authors.affiliation: Department of Anesthesiology and Critical Care Medicine, The Johns Hopkins Hospital, Baltimore, MD, USA.
authors.forename: Justin L
authors.initials: JL
authors.lastname: Lockman
authors.forename: Andrew J
authors.initials: AJ
authors.lastname: Iskander
authors.forename: Melania
authors.initials: M
authors.lastname: Bembea
authors.forename: Thomas O
authors.initials: TO
authors.lastname: Crawford
authors.forename: Howard M
authors.initials: HM
authors.lastname: Lederman
authors.forename: Sharon
authors.initials: S
authors.lastname: McGrath-Morrow
authors.forename: R Blaine
authors.initials: RB
authors.lastname: Easley
mesh_terms: D000293:Adolescent; D000328:Adult; D058109:Airway Management / Q000009:adverse effects / Q000379:methods; D000758:Anesthesia / Q000009:adverse effects*; D000768:Anesthesia, General / Q000009:adverse effects; D000777:Anesthetics / Q000009:adverse effects*; D001260:Ataxia Telangiectasia / Q000150:complications*; D002245:Carbon Dioxide / Q000097:blood; D002648:Child; D003422:Critical Care; D005260:Female; D006801:Humans; D008297:Male; D016343:Monitoring, Intraoperative; D010092:Oximetry; D010101:Oxygen Consumption / Q000502:physiology; D010351:Patient Discharge; D019990:Perioperative Care; D059035:Perioperative Period; D011229:Preanesthetic Medication; D056152:Respiratory Rate / Q000502:physiology; D012189:Retrospective Studies; D012306:Risk; D013990:Tidal Volume; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D000777:Anesthetics; D002245:Carbon Dioxide