Camurati-Engelmann disease (progressive hereditary craniodiaphyseal dysplasia). Case report.
In a patient with Camurati-Engelmann disease, orbital and optic nerve decompression resulted in improvement of papilledema. Subsequent x-ray films of the optic canals, however, revealed reconstitution of osseous optic canals bilaterally, and papilledema has returned in one eye. Definitive treatment of this dysplastic metabolic bone disorder rests in the control of rapid abnormal bone formation.
D001848:Bone Diseases, Developmental / Q000601:surgery*; D003966:Camurati-Engelmann Syndrome / Q000150:complications / Q000000981:diagnostic imaging / Q000601:surgery*; D002675:Child, Preschool; D003399:Craniotomy; D005094:Exophthalmos / Q000209:etiology / Q000628:therapy; D006801:Humans; D008297:Male; D009915:Orbit / Q000601:surgery; D010211:Papilledema / Q000209:etiology / Q000628:therapy; D011859:Radiography