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

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.

Neurocognitive development in isolated Robin sequence treated with the Tuebingen palatal plate

“…The TPP consists of a plate with velar extension that pushes the tongue anteriorly, thereby widening the hypopharynx and releasing the UAO [8]. The effectiveness of this method has been extensively studied in prospective mono-and multicentre studies, in isolated and syndromic RS patients [8][9][10][11] as well as in children with syndromic craniosynostosis and sleep-disordered breathing (SDB) [12].…”

Speech Development in Cleft Palate with and without Robin Sequence

“…12 This concept has been successfully evaluated in prospective single-center and multicenter studies, both in isolated and syndromic Robin sequence patients 7,11,13,14 and in children with syndromic craniosynostosis and sleep disordered breathing. 15 However, little is known about normal speech development in Robin sequence children. Previous studies presented contradictory results, but most found impaired speech development.…”

“…12 This concept has been successfully evaluated in prospective single-center and multicenter studies, both in isolated and syndromic Robin sequence patients 7,11,13,14 and in children with syndromic craniosynostosis and sleep disordered breathing. 15…”