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, tonsillectom
y and adenoidectomy and, later, revision palatoplasty, Overnight polys
omnography (PSG) demonstrated severe obstructive sleep apnoea syndrome
with an apnoea/hypopnoea index (AHI) of 49 events.h(-1) and repetitiv
e oxygen desaturations below 50%, Nasal continuous positive airway pre
ssure (nCPAP) effectively controlled her sleep abnormalities. After 3
yrs of nCPAP therapy, she requested discontinuation and was fully reas
sessed. PSG without nCPAP revealed an AHI <5 events.h(-1) with no desa
turations below 90% and normal sleep quality, A repeat lateral cephalo
metrogram showed increased mandibular length and posterior airway spac
e and reduced soft palate length. The patient remains asymptomatic 9 m
onths following nCPAP discontinuation. This case indicates that nasal
continuous positive airway pressure is an effective nonsurgical therap
y in children with obstructive sleep apnoea syndrome and the Robin seq
uence. It is likely that mandibular growth, increase in mandibular len
gth and enlargement of the posterior airway space was responsible for
the resolution of obstructive sleep apnoea syndrome in this case.