The Pierre Robin syndrome1-4 is characterized by three defects: (1) micrognathia, (2) cleft palate, and (3) glossoptosis. This clinical entity is readily apparent at birth as the newborn has immediate difficulty in breathing and nursing. Nasal regurgitation of milk is often noted. Frequent suction and careful nursing care are essential to prevent aspiration in these infants. However, if these children can be maintained until age 5 or 6 years, virtually normal swallowing function will usually develop.
After encountering a 2-month-old white boy with Pierre Robin syndrome and severe bilateral congenital glaucoma, our attention was directed to ocular involvement in these cases. This is a report of seven cases of Pierre Robin syndrome, four of whom had bilateral ocular disease. To our knowledge, ocular involvement is a hitherto unrecognized part of this clinical picture.
Report of Cases
—This 2-year-old white boy weighed 5 lb. 7 oz. at birth
SMITH JL, CAVANAUGH JJA, STOWE FC. Ocular Manifestations of the Pierre Robin Syndrome. AMA Arch Ophthalmol. 1960;63(6):984-992. doi:10.1001/archopht.1960.00950020986013