Caffey Disease

Definition

Definition of Caffey Disease
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, 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.

Symptoms

Symptoms of Caffey Disease
Among the very striking signs of Caffey's disease is the presence of some soft tissue swellings deeply attached to the bone, along with some prominent cortical thickening of the affected bone. Such swellings would typically appear within the first 90 days of the infant from birth. Facial swellings as other soft tissue swelling may be observed. Other symptoms include hyperirritability, fever, pleurisy, pseudoparalysis, and dysphagia.

Causes

Causes of Caffey Disease
The cause of this medical condition is still not identified to date.

Diagnosis

Diagnosis of Caffey Disease
The clinical symptoms suggest a diagnosis of Caffey's disease, which can be confirmed by X-rays. This may not be apparent during the early phase of the disorder and may simply begin as swellings. Laboratory findings will also show an abnormal increase of enzyme alkaline phosphate levels, which is normally associated with this medical condition.

Treatment

Treatment of Caffey Disease
The disease is self limiting and treatment is symptomatic.

Prognosis

Prognosis of Caffey Disease
Consult with your doctor.

Prevention

Prevention of Caffey Disease
Consult with your doctor.