THE PIERRE ROBIN SYNDROME (GLOSSOPTOSIS, MICROGNATHIA, CLEFT PALATE)

Thirty-nine cases of the Pierre Robin syndrome have been reviewed. In one-fourth of the cases, a history of an intrauterine disturbance in early pregnancy was noted. The entity appears to occur more frequently in offspring of elderly mothers. A positive family history was noted in two instances. The history is typical, with difficulty in swallowing and recurrent cyanotic episodes noted in the newborn. The child often reveals marked sternal retraction, excessive mucous secretion and stertorous respirations which appear quite alarming when in the supine position, but there is usually marked improvement when the child is placed in the prone position, allowing the ptotic tongue to fall forward. Physical examination reveals micrognathia (producing an "Andy Gump" appearance), cleft palate or other defects of the hard and soft palate, and various anomalies of the tongue, the most common being glossoptosis. The associated defects are discussed. In the majority of cases the Pierre Robin syndrome is seen without other significant defects than the triad of micrognathia, cleft palate and glossoptosis. Medical and nursing care are considered. Attention is drawn to the occurrence of ocular disease in these children, e.g., congenital glaucoma and retinal detachment. It is recommended that all cases of the Pierre Robin syndrome have a thorough ophthalmologic examination under anesthesia, including dilatation for ophthalmoscopy and tonometry, prior to 1 year of age.