Cranial CT findings in sclerosteosis.

ABSTRACT

Sclerosteosis or Van Buchem's disease is a rare genetic craniotubular hyperostosis that becomes evident in early childhood and is associated with progressive involvement of the skull. The pathologic changes in the cranium noted on CT are described in three cases. Although the disease is incurable, CT is useful to display the morbid anatomy of the cranium before palliative surgery.