Abstract
Introduction There is limited data available that describe the pattern
of change in severity of airway obstruction and treatment requirements
of infants with Robin sequence (RS). Methods We undertook retrospective
review of 3 infants with RS and severe OSA who were treated with CPAP in
early infancy. All were managed at the Children’s Hospital at Westmead.
Interval CPAP pressure requirements, sleep screening studies and PSG
data were reviewed and obstructive apnoea-hypopnea index (OAHI), oxygen
desaturation index (ODI), along with percentage of time <90%
oxygen saturation were obtained. Results: For two patients, an early
increase in CPAP pressure was observed with peak pressure reached at 5
weeks and 7 weeks, respectively, followed by reduction of pressure
requirements and cessation of therapy at age 39 weeks for Patient 1 &
74 weeks for Patient 2. By way of contrast, the clinical course of a 3rd
patient included jaw distraction at 17 weeks age, time off CPAP, a
requirement to recommence CPAP at 21 weeks, and trials of other therapy,
but treatment was eventually ceased at 75 weeks. Apnoea indices on PSG
did not reflect the CPAP pressure requirements for any of the infants.
Conclusions: Our data suggests a trend toward early increases in CPAP
pressure requirements in typical infants with RS, before a decrease and
cessation later in infancy. The rate at which this occurs may reflect
the underlying severity of obstruction. Further research could help
confirm this pattern. KEYWORDS Robin sequence, OSA, CPAP,
polysomnography, OAHI, ODI, jaw distraction