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To search for studies on tongue–lip adhesion and tongue repositioning used as isolated treatments for obstructive sleep apnoea in children with Pierre Robin sequence.
Methods:
A systematic literature search of PubMed/Medline and three additional databases, from inception through to 8 July 2016, was performed by two authors.
Results:
Seven studies with 90 patients (59 tongue–lip adhesion and 31 tongue repositioning patients) met the inclusion criteria. Tongue–lip adhesion reduced the mean (± standard deviation) apnoea/hypopnoea index from 30.8 ± 22.3 to 15.4 ± 18.9 events per hour (50 per cent reduction). The apnoea/hypopnoea index mean difference for tongue–lip adhesion was −15.28 events per hour (95 per cent confidence interval = −30.70 to 0.15; p = 0.05). Tongue–lip adhesion improved the lowest oxygen saturation from 75.8 ± 6.8 to 84.4 ± 7.3 per cent. Tongue repositioning reduced the apnoea/hypopnoea index from 46.5 to 17.4 events per hour (62.6 per cent reduction). Tongue repositioning improved the mean oxygen saturation from 90.8 ± 1.2 to 95.0 ± 0.5 per cent.
Conclusion:
Tongue–lip adhesion and tongue repositioning can improve apnoea/hypopnoea index and oxygenation parameters in children with Pierre Robin sequence and obstructive sleep apnoea.
Pierre Robin sequence (PRS) presents in the neonatal period with upper airway obstruction and feeding difficulties. Infants with pronounced micrognathia may fail to thrive because of chronic airway obstruction, or experience severe respiratory distress. This is potentially fatal and surgical intervention in these cases is necessary. We present our series of cases with severe PRS requiring surgical relief of their airway obstruction, and the reasons for preferring tracheostomy over glossopexy.
Hearing loss was studied in 22 patients with Pierre Robin syndrome (PRS) aged three to 12 years (median 5.0 years). The results were compared to those obtained in 62 patients with isolated cleft palate (ICP) aged one to 27 years (median 5.5 years). Hearing loss was more frequently found in PRS (73.3 per cent) than in ICP (58.1 per cent) patients (p = 0.02). PRS patients had more ears with moderate (21–40 dB) and severe (>40 dB) hearing loss, disturbing their social contact, with no tendency to normalization with age (Spearman r = 0.065). In contrast to PRS, ICP patients showed a significant tendency to hearing level normalization with ageing (Spearman r = −0.453; p = 0.001). Planigraphs of temporal bones showed inadequately developed pneumatization of the mastoid bone in all PRS patients and in most ICP patients. No malformation of the inner or middle ear was found in either group. PRS patients have a significantly higher risk of conductive hearing loss than those with ICP. Use of tympanostomy (ventilation) tubes is therapy of choice in patients with Pierre Robin syndrome, and it should be introduced as early as possible, even at the same time as palatoplasty.
A 12-year-old schoolgirl presented with severe obstructive sleep apnoea due to the Robin sequence. The sleep apnoea, together with the associated findings of daytime sleepiness, nocturia, right heart strain and growth retardation, were successfully reversed by nasal CPAP therapy. This therapy allows postponement of a decision concerning corrective surgery until after full growth has occurred.
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