Resolution of obstructive sleep apnoea with growth in the Robin sequence.
A 12 year old female with the Robin sequence presented with a one year history of snoring, witnessed apnoeas and daytime sleepiness. Surgery in early childhood had consisted of cleft palate repair, tonsillectomy and adenoidectomy and, later, revision palatoplasty. Overnight polysomnography (PSG) demonstrated severe obstructive sleep apnoea syndrome with an apnoea/hypopnoea index (AHI) of 49 events x h(-1), and repetitive oxygen desaturations below 50%. Nasal continuous positive airway pressure (nCPAP) effectively controlled her sleep abnormalities. After 3 yrs of nCPAP therapy, she requested discontinuation and was fully reassessed. PSG without nCPAP revealed an AHI <5 events x h(-1) with no desaturations below 90% and normal sleep quality. A repeat lateral cephalometrogram showed increased mandibular length and posterior airway space and reduced soft palate length. The patient remains asymptomatic 9 months following nCPAP discontinuation. This case indicates that nasal continuous positive airway pressure is an effective nonsurgical therapy in children with obstructive sleep apnoea syndrome and the Robin sequence. It is likely that mandibular growth, increase in mandibular length and enlargement of the posterior airway space was responsible for the resolution of obstructive sleep apnoea syndrome in this case.
Kiely
J L
JL
Dept of Respiratory Medicine, St Vincent's Hospital, Dublin, Ireland.
Deegan
P C
PC
McNicholas
W T
WT
D002508:Cephalometry; D002648:Child; D005260:Female; D006801:Humans; D008334:Mandible / Q000254:growth & development*; D008445:Maxillofacial Development; D010855:Pierre Robin Syndrome / Q000150:complications* / Q000503:physiopathology; D011175:Positive-Pressure Respiration / Q000379:methods; D012891:Sleep Apnea Syndromes / Q000209:etiology / Q000503:physiopathology* / Q000628:therapy