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June 1960

Ocular Manifestations of the Pierre Robin Syndrome

Author Affiliations

From the Wilmer Institute of Ophthalmology, The Johns Hopkins Hospital.; Department of Pediatrics, The Johns Hopkins Hospital, Baltimore 5 (Dr. Stowe).; Current address: Division of Immunochemistry, Department of Medicine, Massachusetts General Hospital, Boston 14 (Dr. Cavanaugh).

AMA Arch Ophthalmol. 1960;63(6):984-992. doi:10.1001/archopht.1960.00950020986013

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 

Case 1.  —This 2-year-old white boy weighed 5 lb. 7 oz. at birth

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