Abstract

Recognition of the association between ocular manifestations and pathologic changes as well as malformations in distant parts of the body belongs to one of the fastest growing chapters in ophthalmologic knowledge. Ocular changes accompanying osseous dystrophies form well circumscribed entities. Optic nerve atrophy in oxycephaly, exophthalmos and ophthalmoplegias in Apert's syndrome (acrocephalysyndactyly), optic nerve atrophy in dystrophia craniofacialis (Crouzon), optic nerve atrophy and divergent strabismus with synostosis of the major and minor alae of the sphenoid bone, blue sclera, deafness and osteoporosis causing spontaneous fractures (Lobstein's disease) are but a few illusstrations. In another group of ocular changes, linkage with anomalies of the fingers is characteristic. Retinitis pigmentosa occurs with polydactyly, microcornea with brachydactyly, aniridia with dystrophy of the hands and feet or with malformation of the hands in the form ofdes pinces de homardand, finally, congenital dislocation of the lenses with arachnodactyly (Marfan's syndrome). The exact clinical

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