[MIM 114 000]

(infantile cortical hyperostosis, Caffey-Silverman syndrome, Toni-Caffey disease)

Rare. Unknown cause: association of genetic (collagen I anomaly?) and environmental (viral infection) factors ?  It affects primarily young infants: pain with sensitivity to pressure, localized swelling of soft tissues and sometimes redness of one or more parts of the body; fever may be present. Radiography: lamellar periosteal reaction with thickening of the soft tissues, progressively leading to cortical thickening (doubling of the cortical diaphysis). The most frequently affected bones are: tibia, mandible, iliac wing, wrist, tarsus, clavicle, ribs. Severe pain can cause pseudoparalysis. Sometimes, dysphagia and nasal obstruction are observed.

Differential diagnosis: osteomyelitis, osteogenesis imperfecta, hypoparathyroidism.

Usually spontaneous resolution occurs in a few months (before 2 years of age), but relapses are possible.

Symptomatic treatment of pain: NSAIDs, sometimes immunoglobulin or corticosteroids

Anesthetic implications:

careful positioning, management of a child under corticotherapy

References : 

-        Hall C. Caffey disease. Orphanet Encyclopedia. Feb 2005. www.orpha.net/data/patho/GB/uk-caffey05.pdf

Updated: November 2019