Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress 2023 EHA Congress 2023 ASCO Annual Meeting
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on sleep in brain health

LIVE: Thursday 2nd May 2024, 18:00-19:30 (CEST)

Quality sleep is essential for health. But what happens to our brains when sleep patterns are disturbed? Join our experts to explore the interplay between sleep disruption and neurological diseases, and the questions that you need to be asking your patients to help you prevent the harmful effects of sleep deprivation.
ADVANCED SEARCH
Log in
Top
Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2017

Open Access 01-12-2017 | Research

Multicenter study on the effectiveness of the pre-epiglottic baton plate for airway obstruction and feeding problems in Robin sequence

Authors: Christian F. Poets, Christoph Maas, Wolfgang Buchenau, Joerg Arand, Anne Vierzig, Bert Braumann, Silvia Müller-Hagedorn

Published in: Orphanet Journal of Rare Diseases | Issue 1/2017

Login to get access

Abstract

Background

Treatment of Robin sequence is often either invasive or of unproven effectiveness. The pre-epiglottic baton plate (PEBP) is a well-studied alternative, yet is not widely applied internationally. We report on a prospective 3-center cohort study investigating this treatment. Based on an agreed protocol, parents of infants with Robin sequence referred to participating centers were offered enrollment, which involved taking a maxillary cast followed by endoscopy to fit the plate and sleep studies to monitor its effectiveness. Recordings were centrally analyzed by sleep specialists blinded to timing and center. Primary outcome was the mixed-obstructive apnea index, defined as the number of such apneas/h of sleep; secondary outcomes included the desaturation index to <80% pulse oximeter saturation and weight gain.

Results

Of 75 infants referred, 49 could be included; 1 center failed to perform appropriate sleep studies. Within a mean hospitalization of 3 weeks, the mixed-obstructive apnea index decreased (median; interquartile range) from 15.9 (6.3–31.5) to 2.3 (1.2–5.4); it decreased further to 0.7 (0.1–2.4) in the 32 infants who had a successful 3-month follow-up sleep study performed. The desaturation index normalized (from 0.38 (0–2.7) to 0.0 (0–0.1)). Mean standard deviation score for weight remained unchanged between admission and follow-up, while the proportion of tube-fed infants decreased from 74 to 14%.

Conclusions

This prospective multi-center cohort study confirms retrospective audits on the effectiveness of PEBP treatment in improving upper airway obstruction and feeding problems, the main clinical problems of infants with Robin sequence. International collaboration is required to compare this with other treatment approaches.

Trial registration

Number NCT02266043, Registered 30/09/2014; registered partially retrospectively.
Literature
1.
Poets CF, Bacher M. Treatment of upper airway obstruction and feeding problems in robin-like phenotype. J Pediatr. 2011;159(6):887–92.CrossRefPubMed
2.
Maas C, Poets CF. Initial treatment and early weight gain of children with Robin Sequence in Germany: a prospective epidemiological study. Arch Dis Child Fetal Neonatal Ed. 2014;99(6):F491–4.CrossRefPubMed
3.
Buchenau W, Wenzel S, Bacher M, Muller-Hagedorn S, Arand J, Poets CF. Functional treatment of airway obstruction and feeding problems in infants with Robin sequence. Arch Dis Child Fetal Neonatal Ed. 2017;102(2):F142-F146.
4.
Buchenau W, Urschitz MS, Sautermeister J, Bacher M, Herberts T, Arand J, et al. A randomized clinical trial of a new orthodontic appliance to improve upper airway obstruction in infants with Pierre Robin sequence. J Pediatr. 2007;151(2):145–9.CrossRefPubMed
5.
Butow KW, Naidoo S, Zwahlen RA, Morkel JA. Pierre Robin sequence: subdivision, data, theories, and treatment - part 4: recommended management and treatment of Pierre Robin sequence and its application. Ann Maxillofac Surg. 2016;6(1):44–9.CrossRefPubMedPubMedCentral
6.
Sher AE. Mechanisms of airway obstruction in Robin sequence: implications for treatment. Cleft Palate Craniofacial J. 1992;29:224–31.CrossRef
7.
Berry RB, Budhiraja R, Gottlieb DJ, Gozal D, Iber C, Kapur VK, et al. Rules for scoring respiratory events in sleep: update of the 2007 AASM Manual for the Scoring of Sleep and Associated Events. Deliberations of the Sleep Apnea Definitions Task Force of the American Academy of Sleep Medicine. J Clin Sleep Med. 2012;8(5):597–619.PubMedPubMedCentral
8.
Poets CF. Polygraphic sleep studies in infants and children. In: Carlsen KH, Sennhauser F, Warner JO, Zach MS, editor. New Diagnostic Techniques in Paediatric Respiratory Medicine. 2: European Respiratory Monograph; 1997. p. 179–213.
9.
Printzlau A, Andersen M. Pierre Robin sequence in Denmark: a retrospective population-based epidemiological study. Cleft Palate Craniofac J. 2004;41(1):47–52.CrossRefPubMed
10.
Evans AK, Rahbar R, Rogers GF, Mulliken JB, Volk MS. Robin sequence: a retrospective review of 115 patients. Int J Pediatr Otorhinolaryngol. 2006;70(6):973–80.CrossRefPubMed
11.
Marques IL, Bettiol H, de Souza L, Barbieri MA, Bachega MI. Longitudinal study of the growth of infants with isolated Robin sequence considered being severe cases. Acta Paediatr. 2008;97(3):371–5.CrossRefPubMed
12.
Breugem CC, Evans KN, Poets CF, Suri S, Picard A, Filip C, et al. Best practices for the diagnosis and evaluation of infants with robin sequence: a clinical consensus report. JAMA Pediatr. 2016;170(9):894–902.CrossRefPubMed
13.
Maas C, Poets CF, Franz AR. Avoiding postnatal undernutrition of VLBW infants during neonatal intensive care: evidence and personal view in the absence of evidence. Arch Dis Child Fetal Neonatal Ed. 2015;100(1):F76–81.CrossRefPubMed
14.
Linz A, Urschitz MS, Bacher M, Brockmann PE, Buchenau W, Poets CF. Treatment of obstructive sleep apnea in infants with trisomy 21 using oral appliances. Cleft Palate Craniofac J. 2013;50(6):648–54.CrossRefPubMed
Metadata
Title
Multicenter study on the effectiveness of the pre-epiglottic baton plate for airway obstruction and feeding problems in Robin sequence
Authors
Christian F. Poets
Christoph Maas
Wolfgang Buchenau
Joerg Arand
Anne Vierzig
Bert Braumann
Silvia Müller-Hagedorn
Publication date
01-12-2017
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2017
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-017-0602-8

Other articles of this Issue 1/2017

Orphanet Journal of Rare Diseases 1/2017 Go to the issue

Research

KH176 under development for rare mitochondrial disease: a first in man randomized controlled clinical trial in healthy male volunteers

Review

Evaluation of pre-symptomatic nitisinone treatment on long-term outcomes in Tyrosinemia type 1 patients: a systematic review

Research

Autosomal recessive inherited bleeding disorders in Pakistan: a cross-sectional study from selected regions

Research

An example of the utility of genomic analysis for fast and accurate clinical diagnosis of complex rare phenotypes

Research

Phenotype prediction for mucopolysaccharidosis type I by in silico analysis

Review

Safety and potential efficacy of gemfibrozil as a supportive treatment for children with late infantile neuronal ceroid lipofuscinosis and other lipid storage disorders