Pierre-Robin sequence

Pierre-Robin syndrome consists of:

• severe micrognathia
• midline cleft palate without cleft lip
• glossoptosis - an abnormal attachment of the genioglossi muscles allowing the normally sized tongue to fall back and block the airway especially during feeding and in the supine position

The primary abnormality is likely to be hypoplasia of the mandibular area before the ninth week of intrauterine life. This pushes the tongue back and prevents normal closure of the posterior palate.

This abnormality is seen commonly in otherwise normal babies.