Melorheostosis associated with arteriovenous malformation of the ear

Background. Melorheostosis is a rare bone dystrophy that may be associated with various vascular malformations. We report a case of arteriovenous fist ulae of the ear associated with melorheostosis limited to the same side of the body. Case report. A 13 year-old boy presented a congenital port-wine nevus of th e right side of the head complicated by an arteriovenous fistulae and angio matous nodules of the ear. He was treated by laser, surgery of the nodules, arterial embolisations and sclerotherapy. in 1999, he had a benign trauma of the right hand. The X-ray showed hyperostosis resembling wax flowing dow n a candle reaching the carpus and some of the metacarpals and the phalange s of the right hand, typical of melorheostosis. The complete radiographic c heck-up showed the same characteristic appearance on the right side of the skull and the long bones of the right upper limb. Except a deformation of t he right fingers, there were no others symptoms. Discussion. Melorheostosis is a rare, sporadic and benign bone dysplasia th at may be localized to a single limb or disseminated. The diagnosis is usua lly made in late childhood. Pain, stiffness, deformation of a limb are the main clinical manifestations. The skin may be erythematous and sclerotic. T he radiographic appearance is characteristic with hyperostosis on one side of the bone resembling wax flowing down a candle. A vascular abnormality is present in 17 P. 100 Of cases (hemangiomas, aneurysms, renal artery stenos is...). In these cases, melorheostosis is usually limited to the same side of the vascular lesion. We report the first case of arteriovenous fistulae of the ear associated with melorheostosis, on the same side of the body. Th e physiopathology of melorheostosis is still unknown but the association wi th a homolateral vascular abnormality suggests a localized defect in embryo genesis of the vascular and skeletal systems.