Robin sequence: A European survey on current practice patterns

Lieshout, Manouk J.S. van; Joosten, Koen; Mathijssen, Irene M.J.; Hoeve, Hans; Schroeff, Marc P. van der; Wolvius, Eppo B.

doi:10.1016/j.jcms.2015.07.008

Cited by 46 publications

(40 citation statements)

References 41 publications

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“…There is heterogeneity of approach to management of UAO in RS, with no evidence‐based clinical practice guidelines to direct management . This lack of uniformity in approach was highlighted by a recent survey of practices across 101 European centers, which showed that NPA, CPAP, and palatal plates were strongly favored in the UK, France, and Germany, respectively . Only 7% of our cohort required surgical airway management which concurs with the findings of recent large studies .…”

Section: Discussionsupporting

confidence: 81%

“…10 Such diagnostic inconsistency is illustrated by the various differing case definitions used in the above studies, and has been identified as a barrier to ascertainment of true incidence. Our chosen case definition of micrognathia, glossoptosis, and cleft palate, which is the definition most commonly used by medical professionals involved in RS management, 6,7 is arguably more inclusive than criteria used in some previous studies. Inclusion of cleft palate was necessary for case identification, as an established registry for this patient group exists in our region and cases of missed cleft palate are rare.…”

Section: Discussionmentioning

confidence: 99%

“…The case definition of RS used for study eligibility was the presence of all of the following clinical features: (1) cleft palate; (2) glossoptosis; and (3) micro‐ or retrognathia. This definition is reflective of the diagnostic criteria most commonly used by both European and North American clinicians involved in RS management . Children born within the RHSC catchment area but later moving to another region or receiving care from a different cleft service were included in the study to ensure accuracy of birth prevalence calculations.…”

Section: Methodsmentioning

confidence: 99%

“…We also describe the airway management of infants with RS at our center, where a multidisciplinary team involving respiratory and cleft specialists adopt a shared care approach to RS management. 6,7 Children born within the RHSC catchment area but later moving to another region or receiving care from a different cleft service were included in the study to ensure accuracy of birth prevalence calculations. Similarly, children born outside of the included NHS Boards but followed up at RHSC were excluded from analysis.…”

Section: Introductionmentioning

confidence: 99%

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Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Wright

Mehendale

Urquhart

2018

Pediatric Pulmonology

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Section: Discussionsupporting

confidence: 81%

Section: Discussionmentioning

confidence: 99%

Section: Methodsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Wright

Mehendale

Urquhart

2018

Pediatric Pulmonology

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“…Airway management in children with isolated or syndromic RS is still a challenge: many treatment options are reported in the literature [32], with no consensus about the ideal treatment. While we clearly show that the treatment modality reported here improves airway obstruction and failure to thrive, interpretability is limited by our retrospective study design.…”

Section: Discussionmentioning

confidence: 99%

Treatment of infants with Syndromic Robin sequence with modified palatal plates: a minimally invasive treatment option

Müller-Hagedorn

Buchenau²,

Arand³

et al. 2017

Head Face Med

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BackgroundInfants with Robin sequence (RS) suffer from upper airway obstruction (UAO) and feeding problems. We developed an oral appliance with a velar extension in combination with functional treatment and appropriate feeding techniques, which was proven effective in isolated RS. As the above problems are particularly challenging in syndromic RS, we set out to evaluate our treatment concept also in these patients.MethodsWe searched our electronic departmental database to identify all children admitted to our department between 01/01/2003 and 31/12/2009 because of syndromic RS. UAO was quantified by cardiorespiratory sleep studies performed before and during treatment with a modified palatal plate. This appliance consists of a palatal part, covering the hard palate as well as the alveolar ridges and the potential cleft, and a velar extension shifting the tongue in a more anterior position, thereby opening the pharyngeal airway. It is adjusted by fiberoptic nasopharyngoscopy and controlled by cardiorespiratory sleep studies. Obstructive sleep apnea was defined as a mixed obstructive sleep apnea index (MOAI) >3/h. Feeding modalities before and after treatment and weight gain, determined as standard deviation score, were also evaluated.ResultsOf 68 children meeting inclusion criteria, 56 completed treatment (46 of these being infants). Underlying diagnoses included craniofacial dysostosis (N = 13) and synostosis syndromes (N = 5), unspecified dysmorphic syndromes (N = 23) and miscellaneous rare conditions (N = 27).Median MOAI decreased from 8.5 (range 0.3–76.0) at admission to 1.1 (0.0–5.2) at discharge (p < 0.001). 51 children received only a TPP and 5 additionally continuous positive airway pressure (CPAP) or high-flow nasal cannula during sleep for mild residual OSA. Three children ultimately required tracheostomy. The number of exclusively gavage fed infants was reduced from 23 to 7. Conversely, the number of children fed exclusively by mouth increased from 18 to 30. Median SDS for weight decreased from −1.6 (−3.5–1.7) to −1.3 (−4.1-2.5). Twelve children had their treatment prematurely discontinued, e.g. due to laryngeal collapse/laryngomalacia. No patient died during treatment.ConclusionTreatment of UAO and feeding problems in these children with syndromic RS by a modified palatal plate with a velar extension was shown to be effective and safe. If confirmed in prospective studies, it may help to avoid more invasive interventions.

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Pierre Robin and breathing: What to do and when?

Poets

Wiechers

Koos

et al. 2021

Pediatric Pulmonology

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Robin sequence is characterized by mandibular retrognathia, airway obstruction, and glossoptosis; 80%–90% also have a cleft palate. Various treatment approaches exist, and although controlled studies are rare, objective assessment of treatment outcomes that address the leading clinical issues, namely obstructive sleep apnea and failure to thrive, are essential. Sleep‐disordered breathing may be detected using cardiorespiratory polygraphy or polysomnography. Pulse oximetry alone may miss infants with frequent obstructive apneas, yet no intermittent hypoxia. Among conservative treatment options, the Tubingen Palatal Plate with a velar extension shifting the tongue base forward is the only approach that corrects the underlying anatomy and that has undergone appropriate evaluation. Of the surgical treatment options, which are not necessarily the first line of therapy, mandibular distraction osteogenesis (MDO) is effective and has been most extensively adopted. Notwithstanding, it is puzzling that MDO is frequently used in some countries, yet hardly ever in others, despite similar tracheostomy rates. Thus, prospective multicenter studies with side‐by‐side comparisons aimed at identifying an optimal treatment paradigm for this potentially life‐threatening condition are urgently needed.

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Robin sequence: A European survey on current practice patterns

Cited by 46 publications

References 41 publications

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Epidemiology of Robin sequence with cleft palate in the East of Scotland between 2004 and 2013

Treatment of infants with Syndromic Robin sequence with modified palatal plates: a minimally invasive treatment option

Pierre Robin and breathing: What to do and when?

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