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

Silvia Müller-Hagedorn,Margit Bacher,Wolfgang Buchenau,Siegmar Reinert,Michael Krimmel,Jörg Arand,Cornelia Wiechers,Christian F Poets

doi:10.1186/s13023-018-0808-4

Abstract

BackgroundInfants 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. Here, we evaluated this treatment concept.MethodsOur hospital’s electronic patient files were searched for all children with a diagnosis of SCS admitted between 01/01/2004 and 31/12/2016. Children with isolated craniosynostosis were excluded. OSAS was defined as a mixed-obstructive apnea-hypopnea index (MOAHI) > 1, and UARS as more than 1 episode with nasal flow limitation/h, but absent OSAS. Children with a diagnosis of OSAS received the TPP and fiberoptic nasopharyngoscopy to assess the type of obstruction and to adjust the plate. Growth and weight gain, determined as standard deviation scores, were also evaluated before and during treatment.ResultsOf 34 patients included, 24 presented with SDB (19 OSAS, 5 UARS) and 27 had midface hypoplasia. Proportions of SDB were 78% in those with, and 22% in those without midface hypoplasia. In the OSAS group (n = 19), 13 patients were treated with palatal plates, with the remaining receiving continuous positive airway pressure, midface surgery or tracheal intubation. The MOAHI decreased across all children receiving palatal plate treatment from 14.6 (range 0.0–50.7) at admission to 0.9 (range 0.0–3.5) at discharge (p = 0.002). SDS for weight and body length also improved (p < 0.05 for weight and p = 0.05 for body length). Only one child required tracheostomy.ConclusionTreatment of upper airway obstruction by a modified TPP in these children with SCS was shown to be mostly effective and safe. If confirmed in larger prospective studies, it may help to avoid more invasive interventions.

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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