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January 1966

Retinitis Pigmentosa Associated With Sturge-Weber Syndrome

Author Affiliations

From the Wilmer Ophthalmological Institute, the Johns Hopkins Hospital, Baltimore.

Arch Ophthalmol. 1966;75(1):72-76. doi:10.1001/archopht.1966.00970050074014

The occurrence of the Sturge-Weber syndrome with other congenital disorders except glaucoma and another of the phacomatoses is rare. With these exceptions, coincidence of this syndrome with other inherited diseases is usually considered to be based on chance occurrence.1

Retinitis pigmentosa associated with labyrinthine deafness (Usher's syndrome) is now believed to be transmitted as a simple recessive trait.1

The purpose of this communication is to report a case of complete Sturge-Weber syndrome associated with Usher's syndrome, retinitis pigmentosa with deafness.

Report of Case 

Present Illness.  —This 28-year-old white man was first seen at the Wilmer Eye Clinic emergency room because of pain and decreased vision in his right eye. The patient's mother stated that vision in his right eye had always been poor. About five years prior to this visit, his right eye became red, had itched and burned intermittently. It remained red and inflamed for some months,