Survey of anesthetic and airway management in children and adults with Cornelia de Lange syndrome: parents' perceptions and perspectives.

Objective: Cornelia de Lange syndrome (CdLS) is characterized by multiple anatomic malformations, which may affect airway and anesthetic management. The study's objective was to collect information from families of CdLS patients regarding their perception of their airway and anesthesia experiences and associated complications. This would allow anesthesiologists to evaluate the families' perspective in order to create a better approach to treating patients. Methodology: A web-based, 24 question survey was solicited via the CdLS Foundation's quarterly newsletter (circulation 4,000) It was followed up, via phone, Skype, and e-mail, with questions targeting intubation, ventilation, oxygenation, aspiration, and complications during surgery and procedures requiring sedation. A paper version of the survey was distributed to families and caregivers of the patients at the 2014 CdLS Foundation National Family Conference. The focus of the survey was to obtain the families'/caregivers' perception related to airway and anesthetic management experiences and perspectives regarding overall clinical care. Results: We received 70 survey responses (52 web, 18 paper). 23 of the CdLS patients were adults, while 47 were pediatric patients. Fifty-one respondents (72.9%) reported difficulty breathing due to anatomical reasons. Compared to adults, pediatric CdLS patients were reported to have more airway complications with a significantly higher incidence of oxygen desaturation (19.2% vs. 0.0%). Cardiac arrest occurred in 12.8% of pediatric CdLS patients. Thirty-one (44.3%) respondents reported complications caused by the anesthetic or analgesic medication. The most commonly reported complication was unfavorable emergence from anesthesia (52.9%). Upon follow up, we found that a large number of parents did not complete responses due to their lack of understanding or awareness. The perspectives from both caregivers and families illustrated the need to develop patient centered approach for patients with CdLS. Conclusion: Our study found that CdLS patients experienced extensive problems during airway and anesthetic management. In addition, certain complications were found more frequently in pediatric patients such as, cardiac arrest and difficult intubation. We found that the families were very involved and knowledgeable about their children's care. However, there was still a lack of awareness in certain areas of airway and anesthetic management. The value of their perspective highlighted the need for a more focused patient centered care for CdLS patients and their families. [ABSTRACT FROM AUTHOR]