CLEIDOCRANIAL DYSOSTOSIS

A REVIEW OF 40 NEW CASES

¹ Professor and Chairman, Department of Radiology, University of Virginia, School of Medicine, Charlottesville, Virginia.

1. Forty new cases of cleidocranial dysostosis are presented in this genetic and roentgenologic evaluation.

2. Abnormalities observed previously in the disease are assessed including the following hallmark patterns: unossified fontanelles; posterior wedging of the thoracic vertebrae; delayed ossification of the pubic bones; and pointing of the terminal phalangeal tufts.

3. New aspects of the disorder recognized in this study include: distinctive segmental calvarial thickening in the temporal and occipital bones; right clavicular selectivity; high incidence of spondylolysis of the lumbar vertebrae; and characteristic lateral notching of the capital femoral epiphysis.

4. Cephalopelvic disproportion is emphasized as a potential cause of severe dystocia, necessitating cesarean sections in 5 of 14 parous women with the disorder.

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