Controversies in the Diagnosis and Management of the Robin Sequence

Abstract

The Robin sequence, previously known as the Pierre Robin syndrome, is characterized by the sequence of clinical events that result from a small mandible. The tongue becomes posteriorly displaced (glossoptosis) and obstructs the airway. The obstructing tongue also makes oral feeding difficult and, in severe cases, impossible. Patients with Robin sequence may also present with a cleft palate, although this is not necessary to make the diagnosis. The small mandible either results from an inherent growth problem (genetic or syndromic) or is deformational where intrauterine growth of the mandible is restricted. Conditions of syndromic patients are generally more severe and difficult to treat. The phenomenon of "catch-up" growth is disputed, although it is likely to occur in deformational but not syndromic patients. Patients with Robin sequence are best managed by a multidisciplinary team familiar with the diagnoses and treatment of difficult pediatric airways. Approximately 70% of Robin infants are successfully managed by prone positioning alone. Tracheostomy is necessary in approximately 10% of these patients, especially for subglottic obstructions including laryngomalacia and tracheomalacia. Tracheostomy should be avoided if possible. Treatment of the remaining 20% remains highly contentious. Tongue-lip adhesion, nasopharyngeal airways, and distraction osteogenesis all have strong advocates. Ideally, treatment should be individualized. Patients who will have catch-up growth of the mandible will only need a tongue-lip or nasopharyngeal airway as a temporary measure. Patients who we know will not have catch-up growth will benefit from early distraction osteogenesis.