Related Experiment Videos
Airway complications in CHARGE association
B F Asher1, T J McGill, L Kaplan
1Department of Otolaryngology, St Louis (Mo) Children's Hospital.
Insights
Children with CHARGE association are prone to airway instability. Respiratory arrests in these patients can worsen developmental delay, suggesting cerebral hypoxia plays a role. Early tracheotomy is recommended to protect the central nervous system.
Area of Science:
- Pediatric Medicine
- Genetics
- Neurology
Background:
- CHARGE association is a complex genetic disorder affecting multiple systems.
- Airway instability is a known complication in CHARGE association.
- Developmental delay is common in affected children.
Purpose of the Study:
- To investigate the association between catastrophic airway events and developmental delay in patients with CHARGE association.
- To determine the impact of respiratory arrests on neurodevelopmental outcomes.
Main Methods:
- Retrospective chart analysis of 16 patients with CHARGE association at The Children's Hospital, Boston.
- Categorization of patients based on the occurrence of respiratory arrests.
- Assessment of developmental delay severity in relation to airway events.
Main Results:
- Nine out of 16 patients experienced at least one respiratory arrest.
- Fourteen patients exhibited some degree of developmental delay.
- Developmental delay was most severe in patients who suffered respiratory arrests, indicating potential cerebral hypoxia.
Conclusions:
- Children with CHARGE association have a significant propensity for airway instability.
- Cerebral hypoxia resulting from airway events may contribute to developmental delay.
- Early tracheotomy is recommended over choanal atresia repair to safeguard neurodevelopment in these patients.
Abstract:
The association between catastrophic airway events and developmental delay was examined in patients with CHARGE (coloboma, heart disease, atresia choanae, retarded growth and/or development, genital hypoplasia, and ear anomalies and/or deafness) association. A retrospective chart analysis was performed from The Children's Hospital in Boston, Mass. Sixteen patients were identified with CHARGE association. Nine patients had at least one respiratory arrest, and 7 had no airway difficulties. Some degree of developmental delay was seen in 14 patients, but was most severe in those patients who suffered a respiratory arrest. We conclude that children with CHARGE association have a propensity for airway instability and that cerebral hypoxia contributed to the developmental delay in some of our patients. We recommend early tracheotomy rather than early choanal atresia repair in these patients to protect the central nervous system.