Infantile cortical hyperostosis

Case contributed by Dr Nafisa Shakir Batta

Presentation

Irritability, mild fever, tenderness of left thigh with visible swelling. Undergoing treatment for osteomyelitis.

Patient Data

Age: 5 months
Gender: Male

On admission

x_ray

Lamellated to fusiform periosteal reaction along the shaft of left femur is evident with minimal soft tissue swelling. A subtle finding of periostitis along the right lower ribs is also seen, and child cried every time he was held due to chest pain.

Three week follow-up (new complaint jaw swelling)

x_ray

There is visible jaw swelling. The radiograph of mandible showed minimal periostitis. The radiograph of the left thigh and the chest AP view showed negligible decrease in periosteal reaction.

Case Discussion

Infantile cortical hyperostosis is a self-limiting inflammatory disorder of infants with a triad of soft tissue swelling, bone lesions on x-ray and irritability. The bone involvement is usually asymmetrical and includes predominantly mandible, ribs, clavicle and long bones like tibia, ulna, and femur.

The baby at presentation had mild fever, raised TLC and ESR and pain in the left thigh, combined with x-ray findings of periosteal reaction was initially thought to be osteomyelitis. After the antibiotic therapy, TLC continued to be mildly raised, pain and swelling subsided only minimally, with a new development of jaw swelling.

Repeat x-rays and this clinical scenario were pathognomonic of Caffey disease.

A bone biopsy was done and confirmed the diagnosis. The patient's radiographs after 6 months, at 11 months of age, were perfectly normal.

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