Case Report <250> J. Nepal Paediatr. Soc. Caffey’s Disease Affecting the Ribs Nayek K 1 , Barik K 2 , Chaki B 3 , Adhikary A 4 1 Dr. Kaustav Nayek, MBBS, MD, Professor, 2 Dr. Kanailal Barik, MBBS, MD, Associate Professor, 3 Dr. Bhusan Chaki, MBBS, PG Trainee, 4 Dr. Amit Adhikary, MBBS, PG Trainee, Department of Paediatrics, Burdwan Medical College and Hospital, West Bengal, India. Abstract Caffey’s disease is a rare disorder affecting the bones in infancy. It is characterized by irritability, fever, cortical hyperosteosis and adjacent soft tissue swelling. Here is report of an uncommon case where the ribs were significantly affected including the clavicle. Fig 1: Chest x-ray showing cortical hyperostosis of the ribs of left side. Introduction C afey’s disease is a rare disease of unknown etiology. It is characterized by cortical hyperostosis and infammation of the contiguous muscle and fascia. Mandible is the most common site involved. Here we are reporting a case which has afected the ribs including the clavicle. The Case A six month old male child was admitted to this hospital with the history of high grade intermittent fever since 26 day of age. He was treated with diferent medications (including iv antibiotics) but fever did not resolve. Patient did not have any history of contact with tuberculosis. At four months of age patient developed swelling at lower left chest. We had done the routine investigations including a chest X-ray which showed cortical hyperostosis of the ribs of left side. Hyperosteosis initially afected the lower ribs and it gradually involved the upper ribs. Hyperostosis also afected the medial end of the clavicle. Initially there was mild pleural efusion also which resolved gradually. Complete haemogram showed a decreased hemoglobin level, very high ESR, leucocytosis, and thrombocytosis. Hb was 8.2gm%, ESR 85mm, WBC count 20000/μl with Neutrophil 40%, platelet count was 722000/μl, RBCs morphology were microcytic hypochromic anemia. A provisional diagnosis of Cafey’s disease was made based on the clinical and investigational study. Patient was put on steroid (Prednisolone 1mg/kg/day) and responded dramatically. His fever subsided, swelling decreased and patient became well looking. Discussion Cafey’s disease of bone is a rare disorder of unknown etiology. It is charecterized by cortical hyperosteosis along with infammation of the adjacent muscle and fascia. Usually sporadic in nature but may be autosomal dominant or autosomal recessive 1 . A linkage to mutations of the COL1A1 gene (codes for the α 1 chain of type I collagen) has been reported 2 . It was frst reported as a disease entity by Cafey and Silverman in 1945 3 . Bones commonly afected are the mandible, rib lesions and by remissions and relapses. Clavicle, scapula and the ulna, but any tubular hones except the phalanges may also be afected. Mandible is the most common site involved 4 . The disease manifests as irritability and fever around 10 weeks of age. Soft tissue swelling may preceed the onset of bone changes. Swelling is nonsuppurative, have wood like induration with minimal warmth 1 . September-December, 2012/Vol 32/Issue 3 doi: http://dx.doi.org/10.3126/jnps.v32i3.6933