Literature DB >> 26712482

Upper airway endoscopy to optimize obstructive sleep apnea treatment in Apert and Crouzon syndromes.

Priya N Doerga1, Bart Spruijt2, Irene M J Mathijssen2, Eppo B Wolvius3, Koen F M Joosten4, Marc P van der Schroeff5.   

Abstract

INTRODUCTION: Obstructive sleep apnea (OSA) is highly prevalent in children with Apert and Crouzon syndromes. Although often related to midface hypoplasia, it is a multi-level problem for which routine midface advancement might be a suboptimal treatment choice. We therefore wished to: 1.) use upper airway endoscopy to examine the level of obstruction in children with OSA; 2.) determine the relationship between endoscopic assessment and OSA severity; and 3.) evaluate the effect of surgery on endoscopic assessment and OSA severity.
METHODS: Prospective observational cohort study of patients considered for midface advancement, underwent upper airway endoscopy. Endoscopy findings were scored according to the system of Bachar, based on level (nose, uvulopalatine plane, tongue base, hypopharynx and larynx); and severity (no, partial or complete obstruction). Polysomnography was used to diagnose OSA.
RESULTS: We included 22 children (Apert N = 10, Crouzon N = 12), 17 had OSA, 14 of whom had multilevel obstruction and 3 single-level obstruction. The endoscopy findings were correlated with OSA severity: R = 0.56, P = 0.01. Midface advancement (N = 8) reduced Bachar's severity index in 7 of 8 patients, and OSA in all patients.
CONCLUSIONS: OSA in children with Apert or Crouzon syndrome is often a multi-level problem. Upper airway endoscopy is essential to optimizing OSA treatment.
Copyright © 2015 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

Entities:  

Keywords:  Apert syndrome; Craniosynostoses; Crouzon syndrome; Monobloc; Obstructive sleep apnea; Respiratory system

Mesh:

Year:  2015        PMID: 26712482     DOI: 10.1016/j.jcms.2015.11.004

Source DB:  PubMed          Journal:  J Craniomaxillofac Surg        ISSN: 1010-5182            Impact factor:   2.078


  5 in total

1.  Mutation c.943G>T (p.Ala315Ser) in FGFR2 Causing a Mild Phenotype of Crouzon Craniofacial Dysostosis in a Three-Generation Family.

Authors:  Luitgard M Graul-Neumann; Eva Klopocki; Nicolai Adolphs; Martin A Mensah; Wolfram Kress
Journal:  Mol Syndromol       Date:  2017-01-13

2.  Cervical Spinal Cord Compression and Sleep-Disordered Breathing in Syndromic Craniosynostosis.

Authors:  B K den Ottelander; R de Goederen; C A de Planque; S J Baart; M L C van Veelen; L J A Corel; K F M Joosten; I M J Mathijssen; M H G Dremmen
Journal:  AJNR Am J Neuroradiol       Date:  2020-12-03       Impact factor: 3.825

3.  Apert Syndrome: Report of a rare congenital malformation.

Authors:  Ehsan Rathore; Altaf Hussain Rathore
Journal:  Pak J Med Sci       Date:  2017 May-Jun       Impact factor: 1.088

4.  Improvement in Sleep Architecture is associated with the Indication of Surgery in Syndromic Craniosynostosis.

Authors:  Robbin de Goederen; Koen F M Joosten; Bianca K den Ottelander; Mark J W van der Oest; Els M M Bröker-Schenk; Marie-Lise C van Veelen; Eppo B Wolvius; Sarah L Versnel; Robert C Tasker; Irene M J Mathijssen
Journal:  Plast Reconstr Surg Glob Open       Date:  2019-09-10

5.  Obstructing Sleep Apnea in Children with Genetic Disorders-A Special Need for Early Multidisciplinary Diagnosis and Treatment.

Authors:  Mihaela Oros; Lucica Baranga; Vasilica Plaiasu; Sebastian R Cozma; Adriana Neagos; Luminita Paduraru; Violeta Necula; Cristian Martu; Lucia Corina Dima-Cozma; Dan Cristian Gheorghe
Journal:  J Clin Med       Date:  2021-05-17       Impact factor: 4.241

  5 in total

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