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Cheryl L. Keys, MD; Joel Sugar, MD; Mahmood F. Mafee, MD

Arch Ophthalmol. 1990;108(12):1720-1723.

Abstract
• Familial congenital trigeminal anesthesia as an isolated abnormality is an unusual disorder. To our knowledge, only one family has previously been reported. We report here a family with three affected members demonstrating facial anesthesia, bilateral corneal changes, and nasal septal damage secondary to self-traumatization. Magnetic resonance imaging demonstrated hypoplasia of gasserian ganglia and trigeminal nerves in the affected father of two affected sons. The pathogenesis of this disorder appears to be congenital hypoplasia of the trigeminal nerves and gasserian ganglia that is inherited in a dominant fashion.

Author Affiliations
From the Department of Ophthalmology, Eye and Ear Infirmary (Drs Keys and Sugar), and the Department of Radiology (Dr Mafee), University of Illinois at Chicago College of Medicine.

Footnotes
Accepted for publication July 6, 1990.

Reprint requests to Department of Ophthalmology, UIC Eye Center, University of Illinois at Chicago College of Medicine, 1855 W Taylor St, Chicago, IL 60612 (Dr Sugar).

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