Scintigraphic findings in progressive diaphyseal dysplasia

A 14-yr-old white male with a severe form of progressive diaphyseal dysplasia (Engelmann-Camurati disease) was referred to our institution for evaluation of splenomegaly, which is not usually associated with the disease. Our studies included bone-marrow, and liver-spleen scans. These scintigraphic findings, along with the probable cause for splenomegaly, are discussed.