A 3-week-old afebrile infant developed fussiness with feeds, a shoulder mass and decreased left upper extremity motion. Left shoulder radiograph (Fig. 1) demonstrated diffuse scapular periosteal reaction, which was confirmed on CT along with bilateral scapular and lower mandibular cortical thickening (Fig. 2). The constellation of clinical, laboratory (elevated C-reactive protein), and imaging findings suggested Caffey disease. Indomethicin was administered with clinical improvement after 2 weeks and complete resolution of CT abnormalities by 10 weeks.
Infantile cortical hyperostosis (Caffey disease) is a rare, self-limited disorder of early infancy characterized by hyperirritability, acute inflammation with swelling of soft tissues, and massive subperiosteal new bone formation [1]. Involvement is often asymmetrical, affecting diaphyses of long bones, mandible, clavicle, ribs and scapula. Refusal to eat and pseudoparalysis can occur secondary to pain. Both sporadic and familial cases, some with mutations of type I collagen, have been reported [2].
References
Kamoun-Goldrat A, le Merrer M (2008) Infantile cortical hyperostosis (Caffey disease): a review. J Oral Maxillofac Surg 66:2145–2150
Cho T, Moon HJ, Cho DY et al (2008) The c.3040C > T mutation in COL1A1 is recurrent in Korean patients with infantile cortical hyperostosis (Caffey disease). J Hum Genet 53:947–949
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Parnell, S.E., Parisi, M.T. Caffey disease. Pediatr Radiol 40 (Suppl 1), 39 (2010). https://doi.org/10.1007/s00247-010-1869-2
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DOI: https://doi.org/10.1007/s00247-010-1869-2