Infantile cortical hyperostosisObjectives
- Describe the clinical features of infantile cortical hyperostosis (Caffey's disease)
- List two other conditions causing periosteal reaction in the same age range as infantile cortical hyperostosis
- Discuss the natural history of infantile cortical hyperostosis
Infantile hyperostosis was initially described by Caffey in 1957, and is known by both Caffey's disease and infantile cortical hyperostosis. A considerable literature has ensued, which can be confusing. The incidence of the disorder is said to be decreasing. The onset is at about 6 weeks of age, often with a febrile illness. The infant is irritable, with an elevated sedimentation rate and elevated alkaline phosphatase. The soft tissue and periosteum overlying the affected bone are swollen. The mandible is characteristically affected. A case report demonstrated the lack of cortical bone in the diaphysis. Several authors have described an autosomal dominant pattern with incomplete penetrance. The general outlook is for spontaneous resolution, but some recurrences of activity have been reported. Based on the association of hyperostosis with administration of prostaglandin E to maintain patent ductus arteriosis, naproxen given as a prostaglandin inhibitor was successful in treating one case of recurrent infantile cortical hyperostosis. A variety of case reports illustrating unusual presentations of infantile cortical hyperostosis are published. There is also a neonatal variant described, which is generally fatal.
Conditions which may resemble infantile cortical hyperostosis include trauma, osteomyelitis, and congenital syphilis. Zaleske includes physiologic periosteal reaction of the newborn, characterized by thin, even symmetric periosteal reaction along femora, tibiae, and humeri.
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