Stickler Syndrome: Airway Complications in a Case Series of 502 Patients.
McNinch, Annie M
Snead, Martin P
Anesthesia and analgesia
Wolters Kluwer Health
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Zimmermann, J., Stubbs, D., Richards, A., Alexander, P., McNinch, A. M., Matta, B., & Snead, M. P. (2019). Stickler Syndrome: Airway Complications in a Case Series of 502 Patients.. Anesthesia and analgesia https://doi.org/10.1213/ane.0000000000004582
Background Patients with Stickler syndrome often require emergency surgery and are often anesthetized in non-specialist units, typically for retinal detachment repair. Despite the occurrence of cleft palate and Pierre-Robin sequence there is little published literature on airway complications. Our aim was to describe anesthetic practice and complications in a non-selected series of Stickler syndrome cases. To our knowledge this is the largest such series in the published literature. Methods We retrospectively identified patients with genetically confirmed Stickler syndrome who had undergone general anesthesia in a major teaching hospital; seeking to identify factors which predicted patients that would require more than one attempt to correctly site an endotracheal tube (ETT) or supraglottic airway device (SAD). Patient demographics, associated factors and anesthetic complications were collected. Descriptive statistical analysis and logistic regression modelling was performed. Results 502 anesthetic events were analyzed. 395 (92.7%) type 1 Stickler and 63 (96.9%) type 2 Stickler patients could be managed with a single attempt of passing an ETT or SAD. Advanced airway techniques were required on four occasions and we report no major complications. On logistic regression modelling receding mandible (p=0.0004) and history of cleft palate (p=0.0004) were significantly associated with the need for more than one attempt at airway manipulation. Conclusions The majority of Stickler patients can be anesthetized safely with standard management. If patients have a receding mandible or history of cleft, an experienced anesthetist familiar with Stickler syndrome should manage the patient. We recommend patients identified to have a difficult airway to wear an alert bracelet.
Wellcome Trust Clinician PhD Fellowship to DJS
Wellcome Trust (204017/Z/16/Z)
External DOI: https://doi.org/10.1213/ane.0000000000004582
This record's URL: https://www.repository.cam.ac.uk/handle/1810/300820
Attribution 4.0 International (CC BY)
Licence URL: http://creativecommons.org/licenses/by/4.0/