Absence of Superior Recti in Craniofacial Dysostosis

Abstract

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,¹Koziak,²Parks and Costenbader,³Crouzon,^4,5Monthus et al,⁶and 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. <h3>Report of Case</h3> A boy, born in