Pierre-Robin Syndrome

Written by: Meghan Sullivan
Dictated by: Adam Bottrill
April 4, 2011
LMC Pediatric Dentistry- Providence
Literature Review



Pierre-Robin Syndrome
Clinical presentation of micrognathia, glossoptosis and high arched or cleft palate in neonates.


Etiology:
A gene may have been identified at 2q32.3-q33.2 where an unbalanced reciprocal translocation shows a contribution to PRS syndrome. Evidence has also shown that the primary mandibular defect of these patients may be due to genetically influenced metabolic growth disruption.

Diagnosis: Diagnosis occurs shortly after birth. The incidence is 5.3 to 22.7 per 100,000 births.














Associated Systemic and Medical conditions: Infants present with severe micrognathia and mandibular hypoplasia. A U-shaped cleft palate may be seen as well as a high palatal arch. Glossoptosis results from the retropositional attachment of the genioglossus muscle. The geniohyoid muscle is foreshortened and strap muscles of the larynx are also compromised. Immediate postnatal and neonatal periods may present with respiratory and feeding problems. Constant medical supervision, repositioning of the infant and possible medical intervention may be necessary. Nasogastric feeding tubes may be required. After the first few months mandibular growth and control of the tongue is improved. The mandible continues to grow during the first four years of life and a normal profile is typical achieved between 4 and 6 years of age.

Dental needs or special considerations: Some patients have mild mandibular retrognathia requiring treatment later in life. The cleft palate is usually repaired at 6.5 months to 2 years of age.

Recent information: A second source states that the gene for PRS may have been identified as a dysregulation of genes SOX9 and KCNJ2.