Background Robin sequence (RS), characterised by micrognathia/retrognathia and glossoptosis with or without cleft palate, presents with intermittent upper airway obstruction and feeding difficulties. Active intervention is required to reduce the risk of brain damage or sudden death. Most treatment options are poorly studied and/or invasive. Our group developed a functional approach including early feeding training and the pre-epiglottic baton plate (PEBP), an orthodontic appliance with a velar extension shifting the base of the tongue forward.
Patients and methods We evaluated the effect of this approach on sleep study results and early weight gain by searching our electronic patient database for infants with isolated RS, born at or referred to our department between 1 January 2003 and 31 December 2012 and treated with the PEBP.
Results Of 122 patients identified, 360 overnight sleep study results, obtained at admission, prior to hospital discharge and 3 months postdischarge, were available (117 infants had complete data). These showed a decrease in the mixed-obstructive apnoea index from (median; IQR) 8.8 (2.1–19.7) to 1.8 (0.6–5.4); p<0.001 and 0.2 (0–1.3); p<0.001, respectively. Z-scores for weight improved from −0.7 (−1.39 to −0.24) upon admission to −0.5 (−0.90 to +0.02) at follow-up (p=0.02), accompanied by a decrease in the proportion of infants requiring nasogastric tube feeding from 66% to 8%. No infant required craniofacial surgery or tracheostomy.
Conclusions These longitudinal cohort data suggest that this functional approach may be an option to treat both, upper airway obstruction and feeding problems, in infants with isolated RS.
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Contributors WB wrote the first draft of the manuscript and critically revised it; SW analysed the data and revised the manuscript; MB, SM-H and JA were involved in data collection and critically revised the manuscript and CFP initiated and supervised this study and critically revised the manuscript.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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