Infantile cortical hyperostosis of the mandible.

Infantile cortical hyperostosis (Caffey's disease) is a self-limited disease. It usually occurs during the first 6 months of life and presents with periosteal new bone formation. The mandible is the most frequently affected bone, and its involvement is virtually pathognomonic. (1) Most reported cases of Caffey's disease were published between 1945 and 1968; since then, there has been a gradual but significant decrease in the number of reports. (2)

The etiology of Caffey's disease is still unknown, but several theories have been advanced. Among the proposed causes are infections, immunologic defects, allergic reactions in collagen tissues, vascular abnormalities, and genetic anomalies. The theory of genetic transmission by an autosomal-dominant inheritance mechanism is supported by two reports of familial cases, (2,3) but this is not the most common scenario.

The clinical presentation includes the triad of hyperirritability, soft-tissue swelling, and the appearance of painful, firm soft-tissue masses. Some patients develop fever, anemia, an increase in erythrocyte sedimentation rate, and an elevated serum alkaline phosphatase level. In most cases, patients experience regression of radiographic findings and a complete recovery in weeks to months. (2)

The mandible is involved in approximately 75 to 80% of cases, either alone or with the involvement of other bones. (4)

Pathologically, an acute inflammatory reaction is seen in the periosteum along with infiltration of the connective tissues by polymorphonuclear leukocytes. The process then extends to the adjacent soft tissues and muscles, resulting in collagen hyperplasia and fibrinoid degeneration. Osteoid trabeculae are formed and, during a subacute phase, the periosteum re-forms around both the old and new bone posteriorly, and it calcifies and appears subperiosteally. During the final stage, remodeling, the peripheral hyperostotic bone disappears by resorption. (4,5) Imaging findings include an ill-defined increase in bone density with marked cortical thickening and a wavy contour. During the healing phase, a laminated periosteal reaction may be present. (4) This is best demonstrated on computed tomography (CT) (figure).

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Radiologic characteristics on magnetic resonance imaging include thickening of the periosteum, a low to intermediate signal intensity on T1-weighted sequences, and a high signal intensity on T2-weighted sequences in the adjacent soft tissues (including muscles) secondary to edema. (3,6)

References

(1.) Caffey J, Silverman W. Infantile cortical hyperostoses. Preliminary report on a new syndrome. Am J Roentgenol Radium Ther 1945;54: 1-16.

(2.) Yousefzadeh DK, Brosnan P, Jackson JH, Jr. Infantile cortical hyperostosis, Caffey's disease, involving two cousins. Skeletal Radiol 1979;4:141-7.

(3.) Newberg AH, Tampas JP. Familial infantile cortical hyperostosis: An update. AJR Am J Roentgenol 1981;137:93-6.

(4.) Langer R, Kaufmann HJ. Case report 363: Infantile cortical hyperostosis (Caffey disease ICH) lilac bones, femora, tibiae and left fibula. Skeletal Radiol 1986;15:377-82.

(5.) Kocher MS, Kasser JR. Radiologic case study. Infantile cortical hyperostosis (Caffey's disease). Orthopedics 1999;22:712, 707-8.

(6.) Harris VJ, Ramilo J. Caffey's disease: A case originating in the first metatarsal and review of a 12 year experience. AJR Am J Roentgenol 1978;130:335-7.

From the Department of Radiology, Louisiana State University Health Sciences Center, New Orleans.