Less invasive treatment of sleep-disordered breathing in children with syndromic craniosynostosis

S Mueller-Hagedorn,C Wiechers,J Arand,W Buchenau,M Bacher,M Krimmel,S Reinert,C Poets

doi:10.1016/j.ijom.2019.03.056

S Mueller-Hagedorn, C Wiechers + Show 6 more

Open Access

https://doi.org/10.1016/j.ijom.2019.03.056

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Abstract

Background: Infants and children with syndromic craniosynostosis (SCS), such as Apert-, Crouzon- or Pfeiffer syndrome, are prone to sleep disordered breathing (SDB) including obstructive sleep apnea and upper airway resistance syndrome (OSAS, UARS), potentially leading to tracheostomy. We modified the Tübingen Palatal Plate (TPP), an oral appliance with a velar extension effectively treating airway obstruction in Robin sequence, by attaching a tube to its velar extension to bridge the narrow pharyngeal airway in SCS patients.

Highlights

Infants and children with syndromic craniosynostosis (SCS), such as Apert, Crouzon- or Pfeiffer syndrome, are prone to sleep disordered breathing (SDB) including obstructive sleep apnea and upper airway resistance syndrome (OSAS, Upper airway resistance syndrome (UARS)), potentially leading to tracheostomy
This midface hypoplasia is caused by a lack of sutural growth and an abnormal remodeling pattern resulting in a deficient maxilla that is small in all 3 planes (Fig. 1) [8]
Eight children presented with a cleft palate, 5 of them were diagnosed as Apert syndrome, one as Pfeiffer syndrome and two with an as yet unidentified craniosynostosis syndrome

Summary

Introduction

Infants and children with syndromic craniosynostosis (SCS), such as Apert-, Crouzon- or Pfeiffer syndrome, are prone to sleep disordered breathing (SDB) including obstructive sleep apnea and upper airway resistance syndrome (OSAS, UARS), potentially leading to tracheostomy. We modified the Tübingen Palatal Plate (TPP), an oral appliance with a velar extension effectively treating airway obstruction in Robin sequence, by attaching a tube to its velar extension to bridge the narrow pharyngeal airway in SCS patients. The primary anomaly involves the cranial base [7] and midface, where a premature fusion of craniofacial sutures leads to midface hypoplasia and retrusion This midface hypoplasia is caused by a lack of sutural growth and an abnormal remodeling pattern resulting in a deficient maxilla that is small in all 3 planes (Fig. 1) [8]. This deficiency is aggravated during growth [9]

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