Infantile cortical hyperostoses; a review of cases with a case report.

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The syndrome of “infantile cortical hyperostoses” was defined and first reported by Caffey and Silverman (1) in July 1945. Since that time, 24 cases have been recorded in the literature. Clinically this disease is manifested by onset in the early part of the first year of life, irritability and fever, tenderness and swelling of the face and jaws, scapula, or extremities. Radiographically there is evidence of cortical hyperostosis at the above-mentioned sites. A compilation of the findings in the 24 reported cases is given in Tables I and II. As is shown, the average age of onset was in the twenty-fifth week, with a sex ratio of 14 males to 10 females. The health of the mother was good, except in one instance, and the diet was adequate in all cases. The feeding history was satisfactory in all but 2 infants, one of whom had a low vitamin C intake. Fever and irritability were present in the majority of cases, as was a leukocytosis. Brawny edema of the face occurred in 66 per cent of the children. The alkaline phosphatase was elevated in every instance in which it was determined. The tuberculin test and serologic tests for syphilis were negative in all cases in which they were performed. Serologic tests were done on four mothers and all were negative. Sedimentation rates were obtained in 11 infants and with a single exception were found to be elevated. The red cell count and hemoglobin were below normal in the majority of cases. Infection of various types was present in 7 of the 24 patients. The duration of acute symptoms, as is shown in Table II, varied from one to ten months. The observations on periosteal involvement are compiled in Table II. As is shown, the most common site is the mandible, followed by the clavicles, upper extremities, lower extremities, ribs, and scapulae, in the order named. Tenderness over the involved bone was present in all but 4 cases. All the patients recovered. Case Report W. P. B., a 3-month male Sioux Indian, was admitted to Creighton Memorial St. Joseph's Hospital on Aug. 19, 1948, with a questionable diagnosis of bone tumor or congenital syphilis. The chief complaint was swelling of the extremities. Except that the mother's diet had been poor, the gestation period had not been remarkable. Delivery was normal, and the weight of the child at birth was 7 pounds and 6 ounces. Bottle feedings of evaporated milk were taken well. No vitamin supplements were included in the formula. Both mother and father were well, but the father had received antisyphilitic therapy twelve years earlier. There was a history of tuberculosis in one sibling. Six weeks before admission, the mother noticed a swelling of the child's right upper arm. This progressed for two weeks, after which time it remained stationary. Four weeks prior to admission a similar swelling appeared in the left thigh. The child was not irritable and no fever was observed at this time.