Infantile Cortical Hyperostosis


Introduction

Infantile Cortical Hyperostosis is an unusual disease of an unknown etiology. It is also known as Caffey's disease or Caffey-Silverman disease after the persons who discovered it. The main feature of the disease is a peculiar cortical thickening of certain bones like the mandible, the clavicles, and the shafts of long bones. Several other diseases like syphilis, scurvy, rickets and traumatic injury also produce cortical thickening in bones, but as yet researchers have been unable to find any link between these diseases and infantile cortical hyperostosis.

Pathology

This is usually a self-limiting condition. There are several theories as to the pathology of Infantile Cortical Hyperostosis.

One theory proposes that the condition occurs due to a localized defect during bone formation when blood supply to a particular area is disturbed or deficient. Another theory suggests that Infantile Cortical Hyperostosis is an inherited disorder wherein the small blood vessels that carry blood and nutrients to the particular area of the bone (called arterioles) get necrosed and hence result in the peculiar features of the disease.

Yet another theory proposes that allergy is responsible to cause this disease, but definite proof is not available. An inflammatory focus can also not be ruled out as the cause of this condition.

Some cases of autosomal dominant inheritance have been documented in the early 1980s.

The condition can mimic like an infection with fever and on blood test can show a raised white cell count. There is usually a tender soft tissue swellings over the collar bone, lower jaw bone and tender. However on pathological examination there are no features of inflammation and they do not go on to form pus like a classical inflammation. When a swelling in one place subside another swelling may appear

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