RT Journal Article
SR Electronic
T1 Cranial CT findings in sclerosteosis.
JF American Journal of Neuroradiology
JO Am. J. Neuroradiol.
FD American Society of Neuroradiology
SP 505
OP 511
VO 7
IS 3
A1 S C Hill
A1 S A Stein
A1 A Dwyer
A1 J Altman
A1 R Dorwart
A1 J Doppman
YR 1986
UL http://www.ajnr.org/content/7/3/505.abstract
AB 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.