Pierre Robin sequence (PRS) consists of a clinical triad of micrognathia, glossoptosis and airway involvement with variable inclusion of cleft palate. PRS causes obstruction of the upper airway, therefore, it generates respiratory problems and difficulties in ingesting food.
Objective: To analyze specific information on Pierre Robin Syndrome such as etiology, clinical characteristics, diagnosis and treatment with a dental approach.
Methodology: In order to carry out this literature review, an electronic search was necessary using PubMed and Google Scholar with the words Pierre Robin Syndrome and dentistry, treatment, diagnosis and clinical manifestations".
Results: Its etiology still remains uncertain, there are associated genes and environmental theories justifying the reason for this syndrome. The gene most associated with PRS is the SOX9 gene. It consists of a clinical triad of micrognathia, glossoptosis and airway involvement with variable inclusion of cleft palate. Tooth agenesis can occur as an isolated trait or as part of a recognized congenital syndrome such as PRS. Its diagnosis can be by ultrasound, MRI, polysomnography and clinical features. Non-surgical options include prone sleeping position, supplemental oxygen, use of a mouthpiece with velar extension, oropharyngeal or nasopharyngeal tube, non-invasive ventilation and endotracheal tube placement. Surgical options are mandibular osteogenic distraction (MDO) and tongue lip ligation.
Conclusion: PRS requires a comprehensive approach from the medical staff, maxillofacial surgeon and dentist. The main dental characteristics are agenesis, hypodontia and taurodontism.