Cranio-facial melorheostosis: case report and review of the literature

Melorheostosis is a rare condition principally affecting the bones of the appendicular skeleton, producing characteristic radiographic features of flowing “candle wax” subperiosteal bone and streaky endosteal bone formation in the diaphysis and epiphysis, which may affect only one side of long bones within a limb. Radiographic appearances form the basis of the diagnosis, as clinical and histological findings are non-specific.Our patient, an elderly woman, was admitted from the Casualty Department following a fall. Radiographs of her pelvis and left elbow were obtained (Figs 1 and 2) showing the typical longitudinal distribution of flowing subperiosteal and endosteal new bone. The left wrist and right elbow were similarly affected, but to a lesser extent. Skull radiographs showed (Fig. 3) bony obliteration of the left maxillary antrum without expansion of its outline, thought to be due to melorheostosis and similar in appearance to a previously described case. Thickening of the vault may be partially due to hyperostosis frontalis interna, but is asymmetrical, and melorheostosis was suspected in addition.Examination revealed no skin changes, vascular abnormalities or joint deformity and the patient had experienced no pain in the affected areas.Over 200 cases have now been reported. The classic pattern of subperiosteal bone deposition may be monostotic, polyostotic, monomelic or affect multiple limbs and the trunk (Morris et al, 1963). Extra-osseous bone deposition can occur.

Duke Scholars

Author John Wiley Williams Jr. Medicine, General Internal Medicine