Pierre Robin Syndrome: A Literature Review

سندرم پیرروبین ( THE PIERRE ROBIN SYNDROME )

The Robin anomalad (Pierre Robin syndrome)--a follow up study.

During a 10-year period 55 patients with the Robin anomalad were admitted to the Liverpool Regional Cleft Palate Units. Fourteen (25%) children died. All deaths were within 3 months of birth. Congenital abnormalities other than mandibular retrognathia and cleft palate were present in 14 (26%) children. Peripheral limb defects were particularly common. Thirty children were recalled and reviewed ...

Role of SOX9 in the Etiology of Pierre-Robin Syndrome

Objective(s:Cleft lip/palate are common congenital anomalies, affecting approximately 2/1000 live births. Pierre Robin Sequence is a subgroup of the cleft palate population. Chromosomal abnormalities near the SOX9 gene disrupt the regulation of this gene and prevent the SOX9 protein from properly controlling the development of facial structures, which leads to isolated PRS. The present study wa...

Non-surgical management of Pierre Robin syndrome.

A review of relevant published reports seems to indicate that in the case of babies suffering from intermittent airway obstruction due to a combination of micrognathia, glossoptosis, and often cleft of hard and soft palate (the Pierre Robin syndrome), successful treatment usually includes surgery of one kind or another. However, some non-surgical methods have been advocated. Pierre Robin (1934)...

Prenatal Identification of Pierre Robin Sequence: A Review of the Literature and Look towards the Future.

Fetal ultrasonography is an important tool used to prenatally diagnose many craniofacial conditions. Pierre Robin sequence (PRS) is a rare congenital deformation characterized by micrognathia, glossoptosis, and airway obstruction. PRS can present as a perinatal emergency when the retropositioned tongue obstructs the airway leading to respiratory compromise. More predictable and reliable diagnos...