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Year : 2004  |  Volume : 14  |  Issue : 2  |  Page : 185-186
Pictorial essay : Infantile cortical hyperostosis (Caffey's disease)

Radio Diagnosis, IGMC and Mayo Hospital Nagpur Amarjyoti x-ray and Sonography Clinic, Wardha Road, Dhantoli Nagpur-440012, India

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Keywords: Infantile Cortical Hyperostosis

How to cite this article:
Phatak S V, Kolwadkar P K, Phatak M S. Pictorial essay : Infantile cortical hyperostosis (Caffey's disease). Indian J Radiol Imaging 2004;14:185-6

How to cite this URL:
Phatak S V, Kolwadkar P K, Phatak M S. Pictorial essay : Infantile cortical hyperostosis (Caffey's disease). Indian J Radiol Imaging [serial online] 2004 [cited 2021 Mar 1];14:185-6. Available from:

   Introduction Top

Infantile cortical hyperostosis is usually self limiting disease begins in early infancy is characterized by unusual irritability, soft tissue swelling and cortical hyperostosis in multiple bones of skeleton, [1],[2]. The condition has been diagnosed in utero, usually however babies are well for several weeks before the onset. The average age of onset is 9 weeks and no case has started after the age of five months [3].

   Discussion Top

Hallmark of the disease is periosteal new bone formation and cortical thickening that underlies the soft tissue swelling [1],[2],[4] When the tubular bones are involved only the diaphysis is involved causing spindle shape of bones, the process sparing bone ends and metaphysis. Massive cortical thickening, widening of bones, bridging of bones across interosseous membrane and enlargement, marginal hyperostosis and sclerosis of flat bones occur. [2] Distribution of lesions is characteristic. The mandible, clavicles and ulnae are the bones most frequently involved [1],[2],[4] The long bones, the ilia, lateral ribs and skull are often affected. Scapula usually shows unilateral involvement. All the bones of the body are implicated except phalanges, vertebral bodies and cuboidal bones, [2] Ever-sole, Holman and Robinson made the most comprehensive microscopic studies of biopsy specimens both in early and late stage in the disease. Early lesion is confined to periosteum, actually intraperiosteal. The swollen, mucoid periosteum looses its peripheral limiting fibrous layer and blends with contiguous overlying fascia, muscles, tendons and disappears temporarily as an identifiable structure. Osteoid trabeculae appear throughout the swollen acutely inflamed periosteum and invades juxtaperiosteal soft tissue, muscles and connective tissue. In the sub acute phase the periosteum reestablishes as an entity with a peripherally limiting sheet of fibrous tissue beyond the new bone that has formed from ectopic osteoid trabeculae. In the late stage the extra peripheral bone is gradually removed [5]. Occasionally the active disease may persist and recur intermittently for years with crippling deformities in the extremities and markedly delayed muscular and motor development. In long standing case the hyperostosis appear to be reamed out from inside producing a thin walled bone with large medullary cavity. When excessively large hyperostosis affect parallel neighbouring bones such as ribs or radius and ulnar pressure may kill the contiguous periosteum with local fusion of cortical walls that act as interosseous bridges. [5] Differential diagnosis is usually not difficult. Some conditions to be included are : trauma, hypervitaminosis A, scurvy, osteomyelitis, syphilis, parotitis and malignancy. The majority of these conditions can be ruled out by age group, demonstrating mandibular involvement, and observing triad of irritability, swelling and osseous lesions. Parotitis does not produce bone changes. If mandible is not involved its recognition may not be easy. The late manifestations of infantile cortical hyperostosis (Expansion of marrow cavity and thinning of cortex) may also be observed in some cases of Hunter Hurler disease but the presence of other findings both clinical and laboratory will readily differentiate these conditions [1].

   References Top

1.Daniel Wilner Infantile cortical hyperostosis in Radiology of bone tumors and allied disorders First edition WB Saunders company Toronto 1982 volume 2:1793-1807.  Back to cited text no. 1    
2.George B Greenfield; Radiology of bone disease Lippincott company, Philadelphia, 4th edition 1986:531.  Back to cited text no. 2    
3.David Sutton A text book of Radiology reprint, E & S Livingstone Edinburgh and London 1971:57  Back to cited text no. 3    
4.Grainger and Allisons diagnostic radiology A text book of medical imaginag Churchill Livingstone, vol 2, Third edition 1997;1748.  Back to cited text no. 4    
5.John caffey Pediatric x ray diagnosis vol 2, seventh edition year book medical publishers INC Chicago, London 1978;1430-1442.   Back to cited text no. 5    

Correspondence Address:
S V Phatak
Radio Diagnosis, IGMC and Mayo Hospital Nagpur Amarjyoti x-ray and Sonography Clinic, Wardha Road, Dhantoli Nagpur-440012
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Source of Support: None, Conflict of Interest: None

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[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5]

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