Skip to content

Member Log In

Infantile Cortical Hyperostosis

Infantile cortical hyperostosis
  1. Describe the clinical features of infantile cortical hyperostosis (Caffey's disease)
  2. List two other conditions causing periosteal reaction in the same age range as infantile cortical hyperostosis
  3. 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.

  1. Bernstein RM, Zaleske DJ. Familial aspects of Caffey's disease. American Journal of Orthopedics (Chatham, NJ) 1995;24(10):777-81.
  2. Finsterbush A, Husseini N. Infantile cortical hyperostosis with unusual clinical manifestations. Clinical Orthopaedics & Related Research 1979(144):276-9.
  3. Frana L, Sekanina M. Infantile cortical hyperostosis. Archives of Disease in Childhood 1976;51(8):589-95
  4. Fried K, Manor A, Pajewski M, Starinsky R, Vure E. Autosomal dominant inheritance with incomplete penetrance of Caffey disease (infantile cortical hyperostosis). Clinical Genetics 1981;19(4):271-4.
  5. Harris VJ, Ramilo J. Caffey's disease: a case originating in the first metatarsal and review of a 12 year experience. AJR. American Journal of Roentgenology 1978;130(2):335-7.
  6. Lachaux A, Le Gall C, Loras Duclaux I, Hermier M. [Familial infantile cortical hyperostosis (Caffey's disease) with osteolytic lesions of the skull]. Archives Francaises de Pediatrie 1992;49(6):525-8.
  7. Mikati MA, Melhem RE, Najjar SS. The syndrome of hyperostosis and hyperphosphatemia. Journal of Pediatrics 1981;99(6):900-4.
  8. Newberg AH, Tampas JP. Familial infantile cortical hyperostosis: an update. AJR. American Journal of Roentgenology 1981;137(1):93-6.
  9. Pazzaglia UE, Byers PD, Beluffi G, Chirico G, Rondini G, Ceciliani L. Pathology of infantile cortical hyperostosis (Caffey's disease). Report of a case. Journal of Bone & Joint Surgery - American Volume 1985;67(9):1417-26.
  10. Saatci I, Brown JJ, McAlister WH. MR findings in a patient with Caffey's disease [see comments]. Pediatric Radiology 1996;26(1):68-70.
  11. Talab YA, Mallouh A. Hyperostosis with hyperphosphatemia: a case report and review of the literature. Journal of Pediatric Orthopedics 1988;8(3):338-41.
  12. Thometz JG, DiRaimondo CA. A case of recurrent Caffey's disease treated with naproxen. Clinical Orthopaedics & Related Research 1996(323):304-9.
  13. Tien R, Barron BJ, Dhekne RD. Caffey's disease: nuclear medicine and radiologic correlation: a case of mistaken identity. Clinical Nuclear Medicine 1988;13(8):583-5.
  14. Turnpenny PD, Davidson R, Stockdale EJ, Tolmie JL, Sutton AM. Severe prenatal infantile cortical hyperostosis (Caffey's disease). Clinical Dysmorphology 1993;2(1):81-6.
  15. Zaleske DJ. Metabolic and endocrine abnormalities. In: Morrissy RT, Weinstein SL, editors. Pediatric Orthopaedics. Philadelphia: Lippincott-Raven; 1996. p. 137-201.
Annual Meeting

April 27 30, 2016
JW Marriott Indianapolis, Indiana

Building Orthopaedics

Find a doctor