Definition
Infantile cortical hyperostosis, Caffey's disease; Caffey's
syndrome; familial subperiosteal bone formation over many bones,
especially the mandible and clavicles and the shafts of long bones;
it follows fever, usually appearing before 6 months of age and
disappearing during childhood.
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Radiographic Appearance
The typical radiological feature is a marked periosteal
new bone formation surrounding the diaphysis of long bones. The new
bone becomes lamellated as the activity of the condition subsides
and the bone becomes normal. It is a self limiting condition and no
treatment is indicated.
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Pathology
1) Infantile cortical hyperostosis is a self limiting disease of
unknown etiology occurring in infants. It can simulate a systemic
infection with fever and leucocytosis and is characterised by soft
tissue swellings over the clavicle, mandible and tender with no
features of inflammation and they do not suppurate. When some
swellings subside others may appear. The bone may thicken
enormously.
2) Disease of unknown etiology affecting skeleton & adjacent
tissues;
Ccharacterized by fever, rirritability, swelling of soft tissues, &
cortical bone thickening;
thickening of jaw & forearm are the most common sites, but
occassionally lesion is diffuse;
mandibular involvement is characteristic;
in extremities, the ulna is frequently invovled;
with the exception of the mandible, single bone involvement is
suggestive of trauma rather than Caffey's Disease;
Iatrogenic cause:
may result from intravenous prostaglandins used to treat neonatal
congenital heart defects.
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Treatment:
The disease is self limiting and treatment is symptomatic. |
Image 1
Infantile Cortical Hyperostosis of the femora.
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Useful Link
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