CORNEAL OPACITIES IN SPONDYLOEPIPHYSEAL DYSPLASIA TARDA

Spondyloepiphyseal dysplasia tarda is an inherited skeletal dysplasia involving the spine and epiphyses of long bones with onset in childhoo d, giving rise to disproportionate short stature and degenerative spin e and hip disease. Associated ocular disease is not commonly recognize d. We report a patient with spondyloepiphyseal dysplasia tarda and a u nique pattern of corneal opacities. Bilateral, irregularly shaped, nod ular, deeply posterior opacities confined to the peripheral cornea wer e noted in this patient. A central stromal granularity was also seen. Minimal visual loss was associated with these findings. An X-linked in heritance pattern is presumed but could not be confirmed.