[Ophthalmologic manifestations of the Pierre Robin syndrome. Report of a case of microphthalmia].

The classical description of the Pierre Robin syndrome includes micrognathia, glossoptosis, airway obstruction, and usual presence of a cleft palate. The Pierre Robin syndrome is currently defined as the combination of retrognathia, cleft palate, and respiratory distress. This last is mixed, with a peripheral component due to glossoptosis and a central component due to brain stem immaturity. The main ocular manifestations found in the Pierre Robin syndrome are congenital glaucoma and severe congenital mypopia responsible for retinal detachment. Microphthalmia is infrequent. We report the case of a neonate with severe Pierre Robin syndrome and major microphthalmia documented by CT scan.