Abstract 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 References 1. This term is used in preference to oxycephaly, since it is accepted by the Quarterly Cumulative Index Medicus. The descriptive terms turricephaly, scaphocephaly, and plagiocephaly are based on the various configurations of the cranium but designate basically the same syndrome. 2. D. M. Grieg ( Hypertelorism , Edinburgh M. J. 31:560, 1924). 3. Sherne, J.: Dislocation of the Eyeball as a Complication of Oxycephaly , Brit. M. J. 1:565, 1938.Crossref 4. Ferriman, D.: Acrocephaly and Acrocephalosyndactyly , London, Oxford University Press, 1941. 5. Gillies, H., and Harrison, S. H.: Operative Correction by Osteotomy of Recessed Malar Maxillary Compound in a Case of Oxycephaly , Brit. J. Plast. Surg. 3:123, 1950.Crossref 6. Kostečka, F.: Ein Beitrag zur Dysostosis craniofacialis , Ztschr. Stomatol. 35:113, 1937.
A.M.A. Archives of Ophthalmology – American Medical Association
Published: Mar 1, 1952