Caffey Disease

Caffey disease, also known as infantile cortical hyperostosis, is a benign proliferating bone disease. The typical radiographic picture is cortical hyperostosis and involvement of the adjacent fascia, muscles, and connective tissues.

Caffey disease has prenatal and infantile forms. The prenatal form of Caffey disease is congenital and more severe. There is major angulation of the long bones, generalized symmetrical involvement of the skeleton, and polyhydramnios.

The more common infantile form has different radiographic appearances depending on the phase of the disease. The early phase is characterized by thickening of the periosteum that can extend into adjacent soft tissues. Cortical resorption may be present during this phase. In the subacute phase, there is an ossifying periostitis with production of layers of immature subperiosteal lamellar bone. There may also be deposition of bone in adjacent soft tissues. The late phase is characterized by removal of peripheral bone, beginning along the inner surface and extending outward.

Differential diagnosis:

• Hypervitaminosis A: There is periosteal new bone formation that may be simliar to Caffey disease.
• Healing scurvy: Subperiosteal new bone formation during healing phase Uncommon before age 4 months, irregularity of the metaphysis, presence of subperiosteal hemorrhage, decreased alkaline phosphatase levels; marked osteopenia
• Healing rickets: Stripelike density that parallels the outer cortical margin of long bones, resembling a periosteal reaction Splaying and irregularity of the metaphysis, slower resolution of clinical and radiographic findings
• Trauma: Especially child abuse.
• Osteomyelitis: Not usually polyostotic.