IMPORTANCE Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. OBJECTIVE To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. EVIDENCE REVIEW Based on a literature review and expert opinion, a clinical consensus report was generated. FINDINGS Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the possibility of multilevel obstruction exists. The initial assessment of the clinical features and severity of respiratory distress is important and has practical implications. Signs of upper airway obstruction can be intermittent and are more likely to be present when the infant is asleep. Therefore, sleep studies are recommended. Feeding problems are common and may be exacerbated by the presence of a cleft palate. The clinical features and their severity can vary widely and ultimately dictate the required investigations and treatments. CONCLUSIONS AND RELEVANCE Agreed-on recommendations for the initial evaluation of RS and clinical descriptors are provided in this consensus report. Researchers and clinicians will ideally use uniform definitions and comparable assessments. Prospective studies and the standard application of validated assessments are needed to build an evidence base guiding standards of care for infants and children with RS.

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This series demonstrates the revised management protocol followed has reduced in-patient stays and remained effective, with infants continuing to thrive after discharge home, and has a low complication rate.

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Since implementation, this new classification of babies with PRS into three grades has clarified care pathways and enhanced communication between the Cleft Clinical Nurse Specialists and other health professionals involved with the care of these babies.

Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.

For some patients who have had a cleft lip and/or palate, problems continue into adulthood or arise later in life. These problems are often multiple and treatment often requires the input of more than one specialist. The results of this audit support the need for coordinated multidisciplinary care for adults who have had a cleft lip and/or palate.

Objective Several methods of treating babies with Pierre Robin sequence have been described since the condition itself was first documented in 1923. The main aim of treatment has been to relieve upper airway obstruction. Treatment methods used range from positioning of the baby to invasive surgery. The aim of this article was to describe the assessment, treatment, and monitoring methods used for babies referred with Pierre Robin sequence (PRS). Setting/Patients From December 1995 to May 2000, 22 consecutive patients were admitted to Birmingham Children's Hospital with PRS. Their airway and nutritional status were assessed and continuously monitored. Interventions Treatment concentrated on the relief of airway obstruction with a nasopharyngeal airway (NPA) and nutritional support of the babies until they grew out of their respiratory and feeding difficulties. Main Outcome Measures Outcome measures were oxygen saturation, growth of the babies, and the need for surgery. Results All babies were managed successfully with an NPA and nutritional support. No baby required surgery, and the majority showed good weight gain. Conclusion Relieving airway obstruction by NPA is an effective and safe treatment for babies with PRS until they have grown out of their respiratory and feeding difficulties. It avoids the need for surgery and can be used on neonatal wards using the monitoring described.