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July 1950


Author Affiliations

From the Department of Ophthalmology, University of Nebraska College of Medicine.

Arch Ophthalmol. 1950;44(1):37-46. doi:10.1001/archopht.1950.00910020040003

CRANIOFACIAL dysostosis, a rare deformity, produced by premature synostosis of certain craniofacial sutures, characteristically shows frontal bosses, prognathism, exophthalmos, exotropia, optic nerve atrophy and maxillary hypoplasia and, as will be shown, is a dominant hereditary character. Three cases of familial occurrence are described, and a preliminary report is given on a case in which the recommended surgical correction was made in early childhood.

CLASSIFICATION  Twenty-four divisions under the general condition of premature synostosis of the cranial sutures were found in the literature,1 but the most popular and plausible classification, based on morphologic similarities, is that which follows:Cranial stenoses (after Faber2)

  • Scaphocephaly, or boat skull: The sagittal suture closes prematurely, giving a long skull in the anteroposterior diameter.

  • Brachycephaly, or broad skull: Premature closure of the coronal sutures produces a short (anteroposterior), but wide, skull.

  • Acrocephaly (oxycephaly), or tower skull: As a result of premature closure of the

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