Caffey Disease eMedicine Radiology

Description: Caffey disease, or infantile cortical hyperostosis, is a benign, rare, proliferating bone disease affecting infants. Caffey and Silverman first reported this disease as a distinct entity in 1945. Classically, Caffey disease occurs in the early part of the first year of life (<5 mo). It is characterized by a clinical triad (fever, soft-tissue swelling, and hyperirritability) and a clinching radiographic picture of underlying cortical hyperostosis (thickening or bony expansion). In addition to the skeleton, the adjacent fascia, muscles, and connective tissues are also involved. Some have suggested that Caffey disease has a predilection for patients with immunodeficient disorders. Two forms of Caffey disease have been described: prenatal and infantile. The prenatal form is rare and has a poor prognosis. The prenatal form has been described as a more severe, congenital form of Caffey disease that is probably inherited as an autosomal recessive trait. Patients present with major angulation of the long bones, generalized symmetrical involvement of the skeleton, and polyhydramnios. Because the prenatal form is a rare presentation of Caffey disease, the remaining discussion in this article, except in the differential diagnostic section, pertains to the more common infantile form.
Synonyms and related keywords: infantile cortical hyperostosis, Caffey's disease, Caffey-Silverman syndrome, prenatal Caffey disease, infantile Caffey disease, prostaglandin E, sporadic Caffey disease, familial Caffey disease
Davis & Asrat 2007