Postoperative airway events of individuals with CHARGE syndrome

Int J Pediatr Otorhinolaryngol. 2009 Feb;73(2):219-26. doi: 10.1016/j.ijporl.2008.10.005. Epub 2008 Dec 5.


Objective: CHARGE syndrome is a heterogeneous genetic disorder comprising multiple congenital anomalies. Major clinical diagnostic criteria include ocular coloboma, choanal atresia/stenosis, characteristic ear abnormalities, and cranial nerve abnormalities. CHARGE syndrome is caused by a mutation in the gene CHD7 located on chromosome 8. Patients with CHARGE syndrome require multiple anesthetics for surgical and otorhinolaryngology procedures. This study describes the postoperative anesthetic related airway events (i.e. re-intubations for apneas and desaturations, airway obstruction due to excessive secretions) of nine individuals with CHARGE syndrome.

Methods: Detailed chart audits were performed on nine patients diagnosed clinically with CHARGE syndrome who had undergone surgery at a single tertiary health centre. The CHARGE characteristics present in each individual, the number and types of surgeries and anesthetics, and the related postoperative airway events were determined.

Results: The mean+/-age of the population at chart review was 11.8 years (+/-8.0). The total number of anesthetics was 147, with a mean of 16.2(+/-8.4). Of the 215 surgical procedures (mean 21.9, +/-12.2), 30% were otorhinolaryngological. Postoperative airway events occurred after 35% of anesthetics. Surgeries resulting in the most airway events involved the heart (65%), the gastrointestinal tract (39%), and airway diagnostic scopes, i.e., bronchoscopy, laryngoscopy, and nasopharyngoscopy (36%). Combining multiple surgical procedures under one anesthetic did not increase the risk of postoperative airway events. As individuals aged, they had fewer surgeries and anesthetics, as well as a lower risk of postoperative airway events.

Conclusion: Individuals with CHARGE syndrome face a significant risk of postoperative airway events with anesthesia, and this is exacerbated by the high number of surgeries they require. Surgeons and anesthesiologists should be aware of potential for postoperative airway events in individuals with CHARGE syndrome and plan accordingly.

MeSH terms

  • Abnormalities, Multiple / genetics
  • Abnormalities, Multiple / surgery*
  • Analgesics, Opioid / adverse effects*
  • Anesthesia, General / adverse effects*
  • Anesthesia, General / methods
  • Anesthetics, General / adverse effects*
  • Choanal Atresia / surgery
  • Coloboma / surgery
  • Cranial Nerves / abnormalities
  • Cranial Nerves / surgery
  • DNA Helicases / genetics
  • DNA-Binding Proteins / genetics
  • Ear / abnormalities
  • Ear / surgery
  • Female
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Mutation
  • Postoperative Complications
  • Respiration Disorders / etiology*
  • Retrospective Studies
  • Syndrome
  • Treatment Outcome


  • Analgesics, Opioid
  • Anesthetics, General
  • DNA-Binding Proteins
  • DNA Helicases
  • CHD7 protein, human