THE CLINICAL picture of acrocephaly1 or related craniostenoses includes a characteristic pattern of facial deformity, consisting primarily of exophthalmos, aplastic, recessed maxilla, and hypertelorism.2 Ptosis and strabismus are often present. The underdeveloped maxilla is responsible for the flatness of the anterior facial region and the recessed floor of the orbit. The nose is frequently described as wide and curved or as, in the French literature, becque de perroquet. The palate is usually contracted and high-vaulted. If the alveolar process is recessed with the rest of the maxillary compound, the protrusion of the normally developed mandible (relative prognathism) adds another factor to the facial malformation. The same facial appearance is also characteristic of craniofacial dysostosis (Courzon's disease), which many authors consider identical with acrocephaly.
Acrocephaly has always attracted the attention and interest of ophthalmologists. Next to mental deficiency, optic nerve atrophy, leading to blindness, is the most serious symptom
LEWIN ML. FACIAL DEFORMITY IN ACROCEPHALY AND ITS SURGICAL CORRECTION. AMA Arch Ophthalmol. 1952;47(3):321–327. doi:10.1001/archopht.1952.01700030329006
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