Oxycephaly is a congenital abnormality of the bones of the skull in which the shape is that of a high dome or tower. It is often called steeple head, or Turmschadel. It is due to premature synostosis of the bones of the skull. Oxycephaly, or synostosis cranii, may further be classified as (1) scaphocephaly, narrow head with elongation from before backward, with a bulging forehead and occiput ; (2) dolichocephaly, a long narrow skull, and (3) brachycephaly, a short wide skull.
Fowler,1 referring to Rubin's2 survey of oxycephaly, listed the commoner ocular changes as optic nerve atrophy, amaurosis, exophthalmos, divergent strabismus and nystagmus. Wechsler3 stated that narrowing of the optic foramen is also present. In the case of oxycephaly I shall report on there were amaurosis, divergent strabismus, unequal exophthalmos and bilateral optic nerve myelination.
In a large percentage of oxycephalic persons the ocular defects listed were found.