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. 2021 May;56(5):814-822.
doi: 10.1002/ppul.25203. Epub 2021 Jan 12.

Tracheal anomalies associated with Down syndrome: A systematic review

M Matthijs Fockens  1 , Michiel Hölscher  2 , Jacqueline Limpens  3 , Frederik G Dikkers  1
Affiliations

Tracheal anomalies associated with Down syndrome: A systematic review

M Matthijs Fockens et al. Pediatr Pulmonol. 2021 May.

Abstract

Introduction: Airway anomalies are accountable for a substantial part of morbidity and mortality in children with Down syndrome (DS). Although tracheal anomalies occur more often in DS children, a structured overview on the topic is lacking. We systematically reviewed the characteristics of tracheal anomalies in DS children.

Methods: A MEDLINE and EMBASE search for DS and tracheal anomalies was performed. Tracheal anomalies included tracheal stenosis, complete tracheal ring deformity (CTRD), tracheal bronchus, tracheomalacia, tracheal web, tracheal agenesis or atresia, laryngotracheoesophageal cleft type 3 or 4, trachea sleeve, and absent tracheal rings.

Results: Fifty-nine articles were included. The trachea of DS children is significantly smaller than non-DS children. Tracheomalacia and tracheal bronchus are seen significantly more often in DS children. Furthermore, tracheal stenosis, CTRD, and tracheal compression by vascular structures are seen regularly in children with DS. These findings are reflected by the significantly higher frequency of tracheostomy and tracheoplasty performed in DS children.

Conclusion: In children with DS, tracheal anomalies occur more frequently and tracheal surgery is performed more frequently than in non-DS children. When complaints indicative of tracheal airway obstruction like biphasic stridor, dyspnea, or wheezing are present in children with DS, diagnostic rigid laryngotracheobronchoscopy with special attention to the trachea is indicated. Furthermore, imaging studies (computed tomography, magnetic resonance imaging, and ultrasound) play an important role in the workup of DS children with airway symptoms. Management depends on the type, number, and extent of tracheal anomalies. Surgical treatment seems to be the mainstay in severe cases.

Keywords: Down syndrome; complete tracheal ring deformity; tracheal bronchus; tracheal disorders; tracheomalacia.

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Conflict of interest statement

The authors declare that there are no conflict of interests.

Figures

Figure 1
Figure 1
Flowchart of the study selection process for tracheal anomalies in Down syndrome [Color figure can be viewed at wileyonlinelibrary.com]
See this image and copyright information in PMC

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