An adult patient with Klippel-Feil syndrome presenting for repeat operation: a cautionary tale of the GlideScope

The American Society of Anesthesiologists; All India Difficult Airway Association; European Airway Management Society; European Society of Anaesthesiology and Intensive Care; Italian Society of Anesthesiology, Analgesia, Resuscitation and Intensive Care, Learning, Teaching and Investigation Difficult Airway Group; Society for Airway Management; Society for Ambulatory Anesthesia; Society for Head and Neck Anesthesia; Society for Pediatric Anesthesia; Society of Critical Care Anesthesiologists; and the Trauma Anesthesiology Society present an updated report of the Practice Guidelines for Management of the Difficult Airway.

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“…224–227,300–303 Case reports also observed successful intubation with flexible intubation scopes (Category B4-B evidence ). 304–356…”

Section: Anticipated Difficult Airway Managementmentioning

confidence: 99%

2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway *

Apfelbaum¹,

Hagberg²,

Connis³

et al. 2021

Anesthesiology

663

547

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“…Several case reports with respect to difficult intubations in patients with KFS exist in the otolaryngology literature. [13][14][15][38][39][40][41][42][43] A useful strategy, as used in one of our patients with a grade 4 view on direct laryngoscopy, is to perform intubation with an endotracheal tube loaded on a flexible bronchoscope advanced through an laryngeal mask airway. 14,39,40,42 Under such circumstances, it is imperative to maintain spontaneous ventilation, as even this intubation technique may not prove successful.…”

Section: Discussionmentioning

confidence: 99%

“…Several case reports with respect to difficult intubations in patients with KFS exist in the otolaryngology literature . A useful strategy, as used in one of our patients with a grade 4 view on direct laryngoscopy, is to perform intubation with an endotracheal tube loaded on a flexible bronchoscope advanced through an laryngeal mask airway .…”

Section: Discussionmentioning

confidence: 99%

“…[13][14][15][38][39][40][41][42][43] A useful strategy, as used in one of our patients with a grade 4 view on direct laryngoscopy, is to perform intubation with an endotracheal tube loaded on a flexible bronchoscope advanced through an laryngeal mask airway. 14,39,40,42 Under such circumstances, it is imperative to maintain spontaneous ventilation, as even this intubation technique may not prove successful. A tracheotomy is the ultimate airway stabilization procedure if more conservative airway management strategies fail.…”

Section: Limitationsmentioning

confidence: 99%

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Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children

Kenna

Irace

Strychowsky

et al. 2018

JAMA Otolaryngol Head Neck Surg

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IMPORTANCE Children with Klippel-Feil syndrome (KFS), characterized principally by abnormal fusion of 2 or more cervical vertebrae, may have many additional congenital anomalies. The overall prevalence of otolaryngologic manifestations among patients with KFS has not been previously characterized. OBJECTIVE To define the otolaryngologic diagnoses made and procedures performed in 95 patients with KFS, which, to our knowledge, is the largest series of this challenging patient population published to date. DESIGN, SETTING, AND PARTICIPANTS For this retrospective review, all patients with KFS who underwent otolaryngology consultation at our institution over a 26-year period (January 1989 to December 2015) were included. Patients were identified using International Classification of Diseases, Ninth Revision (ICD-9) codes and were confirmed through individual medical record review. Relevant otolaryngologic diagnoses and procedures were extracted using ICD-9 and Current Procedural Terminology codes, respectively. Selected demographics included age, sex, number of clinic visits, and number of procedures. MAIN OUTCOMES AND MEASURESThe primary outcomes were the otolaryngologic diagnoses and procedures associated with the KFS patient population; the secondary outcome was Cormack-Lehane classification documented during airway procedures. RESULTSOverall, 95 patients with KFS were included in this study (55 males [58%] and 40 females [42%]); mean (range) age at time of presentation to the otorhinolaryngology clinic was 5.8 (birth-23.0) years. Each patient with KFS averaged 8 visits to the otorhinolaryngology office and 5 otolaryngologic diagnoses. The most common diagnosis was conductive hearing loss (n = 49 [52%]), followed by sensorineural hearing loss (n = 38 [40%]), and dysphagia (n = 37 [39%]). Sixty-two (65%) patients underwent otolaryngologic procedures, with 44 (46%) undergoing multiple procedures. The most common procedure was tympanostomy tube placement (n = 36 [38%]), followed by office flexible endoscopy (n = 23 [24%]). Twelve of the 20 patients who underwent direct laryngoscopy had documented Cormack-Lehane classification; 5 of 12 patients (42%) had a compromised view (grade 2, 3, or 4) of the larynx. Three patients required tracheotomies at this institution for airway stabilization purposes; each had severe upper airway obstruction leading to respiratory failure. CONCLUSIONS AND RELEVANCE Patients with KFS require consultation for a variety of otolaryngologic conditions. Among these, hearing loss is the most common, but airway issues related to cervical spine fusion are the most challenging. Formulating an appropriate care plan in advance is paramount, even for routine otolaryngology procedures.

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An adult patient with Klippel-Feil syndrome presenting for repeat operation: a cautionary tale of the GlideScope

Cited by 2 publications

References 15 publications

2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway *

2022 American Society of Anesthesiologists Practice Guidelines for Management of the Difficult Airway *

Otolaryngologic Manifestations of Klippel-Feil Syndrome in Children

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