A New Grading of Pierre Robin Sequence

Abstract

Objective: Pierre Robin sequence (PRS) involves the triad of micrognathia, glossoptosis, and cleft palate. Neonates with PRS suffer from two problems—airway obstruction and feeding difficulties—but the severity of these problems varies greatly. The resultant varying definitions of PRS have resulted in inconsistent management of those babies given the diagnosis. This in turn makes comparison of published data and communication between clinicians difficult. Historically at Birmingham Children's Hospital, the cleft team only identified babies presenting with cleft palate and severe respiratory distress as “true” PRS. These babies were admitted to the high-dependency neonatal ward for airway assessment and management. Babies with a mild degree of respiratory obstruction but who were managed successfully at home were not classified as having PRS despite needing considerable input from the Cleft Clinical Nurse Specialist. A system of classifying babies with PRS based on the severity of their symptoms and signs and treatment required was therefore devised and is described in this paper. Results: 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.