Skip to main content
main-content

01.12.2018 | Research | Ausgabe 1/2018 Open Access

Orphanet Journal of Rare Diseases 1/2018

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

Zeitschrift:
Orphanet Journal of Rare Diseases > Ausgabe 1/2018
Autoren:
Silvia Müller-Hagedorn, Cornelia Wiechers, Jörg Arand, Wolfgang Buchenau, Margit Bacher, Michael Krimmel, Siegmar Reinert, Christian F. Poets

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. Here, we evaluated this treatment concept.

Methods

Our 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.

Results

Of 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.

Conclusion

Treatment 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.
Literatur
Über diesen Artikel

Weitere Artikel der Ausgabe 1/2018

Orphanet Journal of Rare Diseases 1/2018 Zur Ausgabe