Infantile cortical hyperostoses

Abstract

In all of the cases presented herein the disease occurred within the first six months of life. In Case v a definite swelling of the left side of the mandible was noted within the first month of life. Hyperirritability, swelling and tenderness are important clinical manifestations of this disease. The syndrome appears to be a self-limited manifestation of multiple cortical hyperostoses accompanied with a moderate leukocytosis with temperature rise some-time during the acute phase. Usually more than one area of the body was involved. The family and maternal histories were all non-contributory. The pregnancies were normal with no history of any unusual complication at the time of birth. The x-ray findings were quite consistent. They usually showed definite periosteal proliferation, marked soft tissue swelling and later irregular new bone formation. It is very interesting to note in Case i that periostitis involving the right ulna and the right humerus developed during the patient's stay in the hospital. (Figs. ia, ib and ic.) In Case n the x-ray findings are very instructive, correlating closely with the patient's clinical course. As the patient's symptoms decreased, the x-ray findings also showed definite improvement. Laboratory studies were of significance only in ruling out other known diseases. All serologie tests for syphilis were negative. In the differential diagnosis syphilis, trauma, scurvy, infection and neoplasm should be considered but the characteristic features of the syndrome, infantile cortical hyperostosis, appear to be distinct from any of these. To date no etiologic factor has been proven as causing the condition. Caffey and Silverman 2 suggest the possibility of this syndrome being a subperiosteal allergic edema. To consider that this temporary osseous response resembles those cases with temporary soft tissue edema as in angioneurotic edema is an interesting concept and one that is worthy of further consideration. Also, there may be a disturbance of the normal immunity which the infant possesses during the first year of life permitting the development of these osseous changes. It is curious that this clinical entity has not been found reported before Caffey's 1 original article in 1939, for in both orthopedic and pediatrie practice the syndrome must have been previously observed.