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Stickler Syndrome: Airway Complications in a Case Series of 502 Patients.

Published version
Peer-reviewed

Type

Article

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Authors

Zimmermann, Julia 
Stubbs, Daniel J 
Richards, Allan J 
Alexander, Philip 
McNinch, Annie M 

Abstract

BACKGROUND: Patients with Stickler syndrome often require emergency surgery and are often anesthetized in nonspecialist 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 nonselected 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 that predicted patients who would require more than 1 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 modeling were performed. RESULTS: Five hundred and twoanesthetic events were analyzed. Three hundred ninety-five (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 4 occasions, and we report no major complications. On logistic regression, modeling receding mandible (P = .0004) and history of cleft palate (P = .0004) were significantly associated with the need for more than 1 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 that patients identified to have a difficult airway wear an alert bracelet.

Description

Keywords

Adolescent, Adult, Aged, Aged, 80 and over, Airway Management, Anesthesia, General, Arthritis, Child, Child, Preschool, Cleft Palate, Cohort Studies, Connective Tissue Diseases, Female, Hearing Loss, Sensorineural, Humans, Infant, Infant, Newborn, Intraoperative Complications, Male, Middle Aged, Pierre Robin Syndrome, Retinal Detachment, Retrospective Studies, Young Adult

Journal Title

Anesth Analg

Conference Name

Journal ISSN

0003-2999
1526-7598

Volume Title

132

Publisher

Ovid Technologies (Wolters Kluwer Health)
Sponsorship
Wellcome Trust (204017/Z/16/Z)
Wellcome Trust Clinician PhD Fellowship to DJS