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

Abstract

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.

An innovative method for manufacturing the Tuebingen palatal plate for infants with Robin sequence

“…The shape of the TPP's velar extension is modelled from dental wax and attached dorsally to the plaster cast [12]. Its inclination or angle is chosen so that it is positioned adjacent to the dorsum of the tongue and shifts the tongue into a more anterior position, thereby widening the posterior airway space.…”

“…In a type I obstruction according to Sher [4], as often identified in isolated RS, a simple spur is sufficient. In types II to IV, a ring, short tube or an artificial airway have to be added to the spur [12]. In all cases, however, correct adaptation of the spur is mandatory as a first step.…”

“…Mild cases are often treated by prone positioning, but this is fraught with an increased risk of sudden infant death syndrome [6]. In Germany, according to a recent survey [7], 37% of infants with RS are treated with a pre-epiglottic baton plate that is comparable to the Tuebingen Palatal Plate (TPP) and is a less invasive, yet effective and comparatively well studied alternative for patients with isolated [8][9][10][11] and syndromic RS [12]. This treatment consists of the standard or modified TPP in combination with Manual Orofacial Therapy according to Castillo Morales [13] and Brondo [14].…”

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

“…All recordings were manually analyzed for the presence of respiratory events using slightly modified standard criteria [26] as used in our previous work [22][23][24][25]. In brief, total sleep time (TST) was determined from the first 10-min epoch without movement artifact or a distorted pulse waveform to the last such 10-min epoch; recordings comprising less than 3 h of TST were excluded.…”

“…The velar extension shifts the tongue into a more anterior position, the tube functions as an artificial airway to release the UAO. This treatment concept has been successfully evaluated in patients with isolated [22][23][24] and syndromic [25] Robin sequence, but little is known whether it is equally effective in children with SCS.…”