title: Anesthetic considerations for patients with Bardet-Biedl syndrome: a case series and review of the literature.
abstract: BACKGROUND
  Bardet-Biedl syndrome (BBS) is a rare genetic condition with manifestations that can impact anesthetic and perioperative care. There is a void of literature describing the perioperative anesthetic management in this complex patient population.
  
  
  OBJECTIVE
  The purpose of this retrospective series was to describe the perioperative care of patients diagnosed with BBS at a large academic tertiary referral center with experience in caring for these patients.
  
  
  METHODS
  All patients receiving anesthesia during the time between July 10, 1997 and Jan 1, 2015 were identified. Anesthetic and perioperative records were reviewed in detail for demographic, echocardiographic, preoperative, intraoperative, and postoperative data.
  
  
  RESULTS
  We identified 12 patients with BBS undergoing 40 anesthetics at our institution. The study identified a high risk for difficult airway with need for advanced airway techniques (67% of patient's ≥ 18 years of age required either awake fiberoptic or video laryngoscopy techniques), cardiac abnormalities, renal impairment, morbid obesity, and intellectual disability as the main syndrome manifestations of interest to the anesthesiologist. No patient had perioperative complications directly related to BBS; however, each underwent thorough perioperative evaluation with emphasis on the systemic comorbidities associated with the syndrome. This report illustrates that patients with BBS can safely undergo anesthesia, but a detailed and often multidisciplinary preoperative evaluation is prudent.
authors.affiliation: Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Bradford B
authors.initials: BB
authors.lastname: Smith
authors.affiliation: Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: David W
authors.initials: DW
authors.lastname: Barbara
authors.affiliation: Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Joseph A
authors.initials: JA
authors.lastname: Hyder
authors.affiliation: Department of Anesthesiology, Mayo Clinic, Rochester, MN, USA.
authors.forename: Mark M
authors.initials: MM
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D058109:Airway Management; D000758:Anesthesia; D000777:Anesthetics; D020788:Bardet-Biedl Syndrome / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D004452:Echocardiography; D005260:Female; D006801:Humans; D007223:Infant; D007442:Intubation, Intratracheal; D008297:Male; D008991:Monitoring, Physiologic; D019990:Perioperative Care; D011300:Preoperative Care; D012189:Retrospective Studies; D062606:Tertiary Care Centers; D016896:Treatment Outcome
chemical_list: D000777:Anesthetics
keywords: Bardet-Biedl syndrome; airway management; anesthesia; cardiovascular abnormalities; chronic; congenital; heart defects; kidney failure