Pierre Robin sequence (PRS) is a congenital abnormality which is characterised by the presence of a combination of mandibular hypoplasia, glossoptosis
and often Labio-palatin clefts which is the most common type of craniofacial malformation encountered in practice and also among most common congenital abnormalities.
The urgent problem of the disease is glossoptosis
, which may cause respiratory insufficiency and hypoxia, resulting in death or neurodevelopmental disorders (3, 4).
(1,2) Isolated Pierre Robin syndrome (PRS) is a triad comprising retromicrognathia, glossoptosis
and posterior U-shaped cleft.
Later, it was proposed that retrognathia which is a common finding in PDO sufferers leads to glossoptosis
and consequently narrowing of the airway space .
(2) Cerebrocostomandibular syndrome: Pierre Robin anomaly, speech difficulties, severe micrognathia with glossoptosis
, small thorax with rib-gap defects, occasional intellectual impairment, and spina bifida.
A posteriorly placed tongue (glossoptosis
) results in upper airway obstruction, most noticeable in a supine position.
Craniofacial morphology represents another mechanism by which genetics may influence the development of OSA, the bony and soft tissue structures that are seen from one generation to another in different families, including specific craniofacial disorders, for example, Pierre-Robin syndrome, these patients have micrognathia, glossoptosis
, and cleft palate, the tongue tends to prolapse backward, leading to airway obstruction, and hence, they are more prone to suffer from OSA (39).
The most common presentation is Pierre Robin sequence (PRS) which consists of respiratory distress, micrognathia and glossoptosis
with or without a cleft palate.
On physical examination, micrognathia and upper airway obstruction caused by glossoptosis