Laryngotracheal Anomalies in Children With Syndromic Craniosynostosis Undergoing Tracheostomy

Alli, Adebayo; Gupta, Sanjeev; Elloy, M; Wyatt, Michelle

doi:10.1097/scs.0b013e3182953b43

Cited by 13 publications

(11 citation statements)

References 20 publications

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“…Includes patients from Alli et al, 16 Hockstein et al, 3 Elloy et al, 26 Wenger et al, 10 Zackai et al, 15 Gonzales et al, 27 Cohen and Kreiborg, 2 Inglis et al, 7 Davis et al, 20 and Stater et al 21 …”

Section: Figurementioning

confidence: 99%

Tracheal cartilaginous sleeves in children with syndromic craniosynostosis

Wenger

Dahl

Bhoj

et al. 2017

Genetics in Medicine

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Purpose Because a tracheal cartilaginous sleeve (TCS) confers a significant mortality risk that can be mitigated with appropriate intervention, we sought to describe the prevalence and associated genotypes in a large cohort of children with syndromic craniosynostosis. Methods Chart review of patients with syndromic craniosynostosis across two institutions. Results In a cohort of 86 patients with syndromic craniosynostosis, 31 required airway evaluation under anesthesia. TCS was found in 19, for an overall prevalence of 22%. FGFR2, TWIST1, and FGFR3 mutations were identified in children with TCS. All five children with a W290C mutation in FGFR2 had TCS, and most previously reported children with W290C had identification of TCS or early death. In contrast, TCS was not associated with other mutations at residue 290. Conclusion There is an association between TCS and syndromic craniosynostosis, and it appears to be particularly high in individuals with the W290C mutation in FGFR2. Referral to a pediatric otolaryngologist and consideration of operative airway evaluation (i.e., bronchoscopy or rigid endoscopy) in all patients with syndromic craniosynostosis should be considered to evaluate for TCS. Results from genetic testing may help providers weigh the risks and benefits of early airway evaluation and intervention in children with higher-risk genotypes.

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Section: Figurementioning

confidence: 99%

Tracheal cartilaginous sleeves in children with syndromic craniosynostosis

Wenger

Dahl

Bhoj

et al. 2017

Genetics in Medicine

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“…Almost half of these patients have morphologic airway abnormalities . Upper airway complications reported in these studies are choanal and upper airway stenosis, adenotonsillar hypertrophy (ATH), and nasal narrowing; notable lower airway complications are tracheomalacia, stenosis of the main bronchus, and tracheal cartilaginous sleeve (TCS) . It has also been reported that 40% to 50% of patients with SCS require tracheostomy and that most of these patients later present with severe upper airway stenosis .…”

Section: Introductionmentioning

confidence: 96%

Airway anomalies in patients with craniosynostosis

et al. 2018

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Objectives: 1) Characterize the spectrum of airway anomalies in patients with craniosynostosis, and 2) identify clinical characteristics of these patients that may be associated with the development of airway anomalies.Methods: This study is a retrospective case series assessing the type and frequency of airway anomalies in all patients with craniosynostosis seen at a tertiary-care children's hospital between 2000 and 2016. Cohort analyses were then performed to identify differences in airway anomalies dependent on syndromic associations, multisutural fusion, and location of suture fusion. Clinical characteristics examined included demographics and additional neurologic and craniofacial abnormalities.Results: Four hundred and ninety-six patients with craniosynostosis (83.5% white, 64.5% male; 33.9% sagittal, 28.8% metopic, 11.5% coronal, 1.2% lambdoid, and 24.6% multisutural) were included. Notable airway anomalies included the following: 13.3% adenotonsillar hypertrophy, 8.9% laryngomalacia, 7.3% tracheomalacia, 7.1% subglottic stenosis, 4.0% bronchomalacia, 3.8% laryngeal cleft, and 1.2% vocal fold paresis. Multisutural craniosynostosis patients (n = 122) were more likely to have obstructive sleep apnea (P = 0.005), adenotonsillar hypertrophy (P = 0.014), tracheomalacia (P = 0.011), subglottic stenosis (P < 0.001), and epiglottic/base of tongue collapse (P = 0.003) and require tracheostomy (P = 0.001) and mechanical ventilation (P = 0.017) compared with single suture craniosynostosis. Syndromic craniosynostosis patients (n = 33) were more likely to have obstructive sleep apnea (P < 0.001), laryngomalacia (P = 0.047), and subglottic stenosis (P = 0.009) compared with nonsyndromic patients.Conclusion: Airway anomalies are prevalent in patients with craniosynostosis; patients with multisutural or syndromic types have an increased risk of developing certain abnormalities. There should be a lower threshold for referral for airway evaluation in these populations.

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“…The increased prevalence of CAA in patients with genetic or syndromic diseases has been recognized, such as Trisomy 21, DiGeorge, VACTERL, and Klippel-Feil [5,7,36,40,41] ; however, the mortality risk has not been well evaluated. This demonstrated that CAA patients with chromosome anomaly have a higher mortality risk.…”

Section: Discussionmentioning

confidence: 99%

The comorbidities and risk factors in children with congenital airway anomalies

et al. 2018

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The comorbidities and risk factors associated with congenital airway anomalies (CAAs) in children are undecided. This study aimed to investigate the comorbidities commonly associated with CAA and to explore the prognosis and risk factors in CAA children.This nationwide, population-based cohort study was conducted between 2000 and 2011 with children aged 0 to 5 years assigned to either a CAA group (6341 patients) that diagnosed with CAA or an age- and gender-matched control group (25,159 patients) without CAA, using the Taiwan National Health Insurance Research Database (NHIRD). Descriptive, logistic regression, Kaplan-Meier, and Cox regression analyses were used for the investigation.Cleft lip/palate (adjusted odds ratio [aOR], 7.88; 95% confidence interval [CI], 6.49-9.59), chromosome (aOR, 6.85; 95% CI, 5.03-9.34), and congenital neurologic (aOR, 5.52; 95% CI, 4.45-6.87) anomalies were the comorbidities most highly associated with CAA. Of the 31,500 eligible study patients, 636 (399 in the CAA group and 237 in the control group) died during the follow-up period (6.3% vs 0.9%, P < .001). The mortality risk after adjusting for age, gender, and comorbidities elevated significantly among CAA patients (adjusted hazard ratio [aHR], 4.59; 95% CI, 3.85-5.48). The need for tracheostomy (aHR, 2.98; 95% CI, 2.15-4.15), comorbidity with congenital heart disease (CHD) (aHR, 2.52; 95% CI, 2.05-3.10), and chromosome anomaly (aHR, 2.34; 95% CI, 1.70-3.23) were the independent risk factors most greatly related to CAA mortality.This study demonstrated that CAA was most highly associated with the comorbidities as cleft lip/palate, chromosome, and congenital neurologic anomalies. The CAA children had a significantly elevated mortality risk; the need for tracheostomy, CHD, and chromosome anomaly were the most related risk factors of mortality for CAA. Further studies are warranted to clarify the involved mechanisms.

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Laryngotracheal Anomalies in Children With Syndromic Craniosynostosis Undergoing Tracheostomy

Cited by 13 publications

References 20 publications

Tracheal cartilaginous sleeves in children with syndromic craniosynostosis

Tracheal cartilaginous sleeves in children with syndromic craniosynostosis

Airway anomalies in patients with craniosynostosis

The comorbidities and risk factors in children with congenital airway anomalies

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