Distraction osteogenesis and glossopexy for Robin sequence with airway obstruction.

BACKGROUND

We present six cases of infants with Robin sequence and severe obstructive sleep apnoea who failed treatment with nasal mask continuous positive airway pressure (CPAP). Surgical intervention with mandibular distraction osteoneogenesis with glossopexy meant tracheostomy was avoided. Polysomnography (PSG) was used to document the severity of the obstructive sleep apnoea. The aim of this report was to assess the value of mandibular distraction osteogenesis with glossopexy in children with Robin sequence using PSG preoperatively and post-operatively.

METHODS

Retrospective review of medical records to assess details of preoperative clinical progress, CPAP therapy, endoscopy, PSG and respiratory parameters.

RESULTS

Between 2003 and 2008, 20 infants with Robin sequence were admitted to the neonatal intensive care unit of our tertiary paediatric hospital. Six infants (30%) failed a treatment trial of CPAP during that period, but were subsequently successfully managed with mandibular distraction osteoneogenesis and glossopexy. At endoscopy, all had associated airway problems which included congenitally hypoplastic epiglottis (n= 2), laryngomalacia (n= 2), unilateral choanal atresia (n= 1) and long-segment tracheal stenosis (n= 1).

CONCLUSION

Mandibular distraction osteogenesis with glossopexy provides relief of severe upper airway obstruction for infants with Robin sequence. Epiglottic maldevelopment was present in two infants (33%). Endoscopy and PSG assisted clinical management, but highlighted ongoing respiratory issues.