On some late skeletal changes in chronic infantile cortical hyperostosis

J Caffey - Radiology, 1952 - pubs.rsna.org
J Caffey
Radiology, 1952pubs.rsna.org
Infantile cortical hyperostosis is usually a benign disease in which the clinical manifestations
subside after a few weeks and the skeletal changes, demonstrated roentgenographically,
disappear within a few months (Caffey). More than 50 such cases have been reported
during the last six years and scores of others have been recognized in all parts of the United
States and in many parts of the world. The benign features of the disorder and the good
prognosis have become well known. The chronic type of infantile cortical hyperostosis with …
Infantile cortical hyperostosis is usually a benign disease in which the clinical manifestations subside after a few weeks and the skeletal changes, demonstrated roentgenographically, disappear within a few months (Caffey). More than 50 such cases have been reported during the last six years and scores of others have been recognized in all parts of the United States and in many parts of the world. The benign features of the disorder and the good prognosis have become well known. The chronic type of infantile cortical hyperostosis with late crippling residuals in the skeleton has been observed in only a few cases and is generally little appreciated.
This report is a description of clinical and roentgen findings in a patient who has suffered from infantile cortical hyperostosis for more than two years and who is still deformed and disabled by late residuals in the skeleton. In the evolution of the late bone lesions, the early changes—external thickening of the cortical walls with a normal-sized medullary cavity—were later converted into thinned walls with dilated medullary cavity. Bone bridges between each ulna and radius and between contiguous ribs, found in this patient, are the first lesions of this type to be reported in infantile cortical hyperostosis. Persistent ventral bowing of the tibias (Smitham and Palmer; Kitchin), which did not occur in this patient, is discussed because it promises to be an important and not infrequent sequel of the disease.
Case Report
B. L., a Negro girl, was born of a healthy, primiparous mother. She weighed seven pounds, and during the first three months thrived on a cow's milk formula, with daily supplements of one-half ounce of orange juice and eight drops of a concentrate of vitamins A and D.
When the baby was three months of age, the father had chtckenpox, to which the infant was intimately exposed. Seven days later, large water blisters were said to have appeared on both her wrists and then spread, during three or four days, over the arms, neck, face, legs, and feet. The physician who examined her at home stated that the rash was not characteristic of chtckenpox. With its onset the child became feverish and hyperirritable and ate and slept poorly. Five days after the cutaneous lesions appeared, the left forearm became swollen and tender, and then the jaws, entire right arm, and the entire right leg. These swellings enlarged slowly during a period of seven days. After admission to the Willard Parker Hospital, a city hospital for contagious diseases, roentgenograms disclosed thickenings of the bones which led to the diagnosis of infantile cortical hyperostosis. No signs of chickenpox were observed at that institution.
When four months of age, the child was admitted to the New York Hospital with a fever of 37.5° C.
Radiological Society of North America