PROGRESSIVE SYNOSTEOSIS IN APERT'S SYNDROME (ACROCEPHALOSYNDACTYLY)

WITH A DESCRIPTION OF ROENTGENOGRAPHIC CHANGES IN THE FEET

¹ From the Department of Radiology, University of Nebraska College of Medicine and Hospital, Omaha, Nebraska

The feet in Apert's syndrome show a definite pattern of fusion which occurs in the following order: calcaneus with cuboid, lateral cuneiform with third metatarsal, navicular with medial cuneiform, and phalanges at the interphalangeal joints. Also present in the feet of older patients are "pseudoarticulations," well developed ossa peronei, and bony outgrowths from the phalanges.

A description of progressive synosteoses of the bones of the hands and cervical spine has also been presented. In our review of the literature of Apert's syndrome, no mention of progressive synosteoses has been noted.

Because of the generalized changes and the increasing deformities with age, acrocephalosyndactyly does not seem a particularly good descriptive term for this disease, and in its place progressive synosteosis with syndactyly is suggested. Progressive synosteosis with syndactyly is more inclusive since the acrocephaly is for the most part explained by premature synosteosis.

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