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August 1965

Absence of Superior Recti in Craniofacial Dysostosis

Author Affiliations

Consultant in Ophthalmology, Neurological and Sensory Disease Service Program, Division of Chronic Diseases, Department of Health, Education, and Welfare (Dr. Weinstock). Department of Surgery, Division of Ophthalmology, Western Reserve University, (Dr. Hardesty).

Arch Ophthalmol. 1965;74(2):152-153. doi:10.1001/archopht.1965.00970040154003

Premature synostosis of cranial bones has prompted the description of multiple clinical entities such as craniofacial dysostosis (Crouzon's Disease), acrocephalosyndactylia (Apert's disease), oxycephaly, and many others. The main ocular findings described in these syndromes have been shallowing of the orbits with orbital divergence, exotropia without limitation of ocular movements, exophthalmos, papilledema, optic atrophy, iris and choroidal colobomas, nystagmus, congenital cataract, vitreous opacities, subluxation of the lens, and retinal detachment. Little has been reported about extraocular muscle anomalies. The most common surgical intervention has been orbital decompression to relieve severe exophthalmos and papilledema.

The craniostenoses have been described in greater detail by Howell,1 Koziak,2 Parks and Costenbader,3 Crouzon,4,5 Monthus et al,6 and Garcin et al.7

A case of craniofacial dysostosis with syndactyly (Apert's disease) is presented with unusually interesting extraocular muscle findings which have not been previously reported.

Report of Case  A boy, born in