Excessive crying and Bone disease
 
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Question :
Altaf Naseem, U N Reddy
Department of Pediatrics, Deccan College of Medical Sciences, Hyderabad

Address for Correspondence: Dr. Altaf Naseem, Assistant Professor , Department of Pediatrics, Princess Esra Hospital, Shah ali Banda, Hyderabad – 500 002, Andhra Pradesh, India. Email ID: docaltaf@rediffmail.com

A 4 month old male child presented with fever, excessive crying and swelling of the face. Plain radiographs were done as shown above. What is the diagnosis_?
Expert Opinion :
Caffey disease or Infantile cortical hyperostosis is a benign, rare, proliferating bone disease affecting infants {1}. Classically, Caffey disease occurs in the first year of life { Less than 5 months}. It is characterized by clinical triad of fever, soft tissue swelling and hyperirritability and a clinching radiograph picture of underlying cortical hyperostosis {2}. Although the etiology of caffey disease remains unclear, many clinical and pathologic features are suggestive of inflammatory process {3}. All races are affected, seen equally in boys and girls {1}. Laboratory investigation may show an elevated ESR, an elevated serum alkaline phosphatase, moderate leukocytosis, thrombocytosis and anemia {4}. Clinical course of caffey disease is highly variable, ranging from self limited to protracted illness. Clinical differential diagnosis includes Osteomyelitis, Parotitis and parotid gland abscess and bone tumors of the affected area. Management is essentially palliative aimed at pain relief but some authors claim a good response to high dose immunoglobulin {5}. Corticosteroids have been used to hasten bone remodeling and Indomethacin has been used to control flare ups.

Contributors credits: AN collected the data and drafted the article and revised the manuscript for important intellectual content. He will act as guarantor of the study. UNR helped in manuscript writing. The final manuscript was approved by all authors

Source of Funding: nil
Competing interests: nil

REFERENCES


1. Caffey J. Infantile Cortical Hyperostosis. J Pediatr. 1946` 29: 541-549
2. Carey BE. Neonatal cortical hyperostosis. Neonatal Netw. 2000` 19: 55-57
3. Pazzaglia UE, Byers PD, Beluffi G, et al. Pathology of infantile cortical hyperostosis {Caffey`s disease}. Report of a case. J Bone Joint Surg Am. 1985` 67: 1417-1426
4. Kamoun-Goldrat A, le Merrer M. Infantile cortical hyperostosis {Caffey disease}: a review. J Oral Maxillofac Surg. 2008` 66: 2145-2150
5. Berthier M, Bonneau D, Huret JL. Caffey disease responding to high-dose immunoglobulin. Eur J Pediatr. 1988` 147: 443-444

E-published: February 2010 Vol 7 Issue 2 Art No 13.

Correct Answers : yes  16%

Last Shown : Feb 2010
 
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