Robin Sequence: Neonatal Management

Abstract

Robin sequence (RS) is defined by the clinical triad of micrognathia, glossoptosis, and airway obstruction. The presence of glossoptosis can also obstruct fusion of the palatal shelves during development, resulting in the characteristic wide U-shaped cleft palate. RS can present in isolation (isolated RS) or in association with a congenital syndrome or other abnormalities (syndromic RS or RS plus). Diagnosis is primarily clinical but can be identified prenatally on ultrasonography or magnetic resonance imaging. Management of RS focuses on improving glossoptosis and relieving airway obstruction. Conservative management includes positioning, nasopharyngeal airway, orthodontic appliances, and respiratory support strategies. Surgical interventions include tracheostomy, tongue-lip adhesion, and mandibular distraction osteogenesis. All management strategies can be effective in the appropriately selected patient. Future areas of research include understanding the genetics of RS, improving surgical outcomes with preoperative planning and advanced biomaterials, and improving prenatal identification of children with clinically significant RS.