Progressive diaphyseal dysplasia: Camurati-Engelmann's disease

The authors have studied two cases of progressive diaphyseal dysplasia (Camurati Engelmann's disease). In one case their investigations included bone marrow pressure tests, phlebography and blood gas analysis of the medullary blood. The BMP was raised, and the phlebogram showed venous stasis. The blood gas analysis revealed reduced oxygenation of the medullary blood. On the hypothesis that the vascular change could be of pathogenetic significance, the authors proceeded in both cases to open the medullary canal. This produced immediate remission of the pain, which still persists in one case after six years, while it lasted for three years in the second case.