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Infantile Cortical Hyperostosis (Caffey Disease): A Review

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Purpose

Face swelling in infants may have several causes including infantile cortical hyperostosis (Caffey disease), an inflammatory process with swelling of soft tissues and periosteal hyperostosis of some bones. New insights show that this self-limited condition is collagen I-related.

Patients and Methods

Collagen I is the most important component of bone and dentine. We reviewed literature to lighten this new collagenopathy, the first one with a self-regressive course.

Results

After describing a typical case and the clinical and radiologic features of the disease, we discuss the pathogenic pathways and management care for oral professionals.

Conclusions

Oral practitioners could be asked for differential diagnosis. Surgeons could be queried for surgical correction of the bony deformity, especially of facial and mandibular asymmetry.

Section snippets

Case Report

Our patient was born October 1999 (weight, 3.31 kg; size, 49 cm; cephalic perimeter, 36 cm). Parents were nonrelated. The mother experienced 6 miscarriages, giving birth to a healthy boy in 1996. Another miscarriage occurred before our patient was born. At birth, an angulation of both legs was seen. X-rays showed important hyperostosis of the tibia and the distal part of both humerus. The cranial vault appears normal, but the mandible presents hyperostosis (Fig 1). Pain was present for 5 months

First Description

Infantile hyperostosis was described initially by Caffey and Silverman in 1945.5 They described a group of infants with tender swelling in the soft tissues and cortical thickenings in the skeleton, with onset of these findings during the first 3 months of life.

Epidemiology

It has been reported that infantile hyperostosis affects 3 in every 1,000 infants less than 6 months of age in the United States, with no predilection by ethnic origin or gender.6 Both familial and sporadic forms occur. The familial form

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