Caffey disease

Case contributed by Yasser Asiri
Diagnosis probable

Presentation

Right scapular swelling noticed at the age of one month.

Patient Data

Age: 5 months
Gender: Female

Initial shoulder X-ray

x-ray

The right scapula shows increased density with irregular cortical margins mostly affecting the scapular body with relative sparing of the acromion. There is extension of the irregularity to the inferior outline. Differentials may include focal bony hemangiomatous lesion versus localized infantile cortical hyperostosis of the scapula. Incidental findings of a non-displaced proximal humeral metaphyseal fracture best seen in the abducted view.

mri

MRI study demonstrates an aggressive lesion involving the right scapula with surrounding heterogeneous enhancing soft tissue component and bone marrow edema. Prominent axillary lymph nodes are seen. The imaging appearance may raise the possibility of Ewing's sarcoma, another differential diagnosis would include rhabdomyosarcoma. Proximal right humeral metaphyseal fracture with marrow edema is seen. Right lung posterior segments subsegmental atelectasis is seen. 

ct

CT study shows diffuse thick periosteal reaction and new bone formation in the right scapula with surrounding soft tissue swelling. No significant enhancing soft tissue components or destructive lesions. The findings are not typical for a malignant or sarcomatous process, findings could be related to infection versus inflammatory process like chronic osteomyelitis. Another cause of thick periosteal reaction would include Infantile cortical hyperostosis "Caffey disease".

Follow up Shoulder X-ray

x-ray

Interval significant improvement of the of the right scapula irregularity and sclerosis with only residual minimal increase density of the inferior scapula. The findings are suggestive of ongoing healing and resolution of infantile cortical hyperostosis.

Case Discussion

This patient was initially admitted as a case of right scapula suspicious mass. The shoulder radiograph and the MRI study showed aggressive features, which prompted complete tumor work up including CT guided biopsy and CT of the chest.

Reviewing the CT chest in bone window showed a better advantage in assessment of the extensive subperiosteal bone formation, cortical thickening and the surrounding soft tissue swelling involving the right scapula.

The histopathology result from the scapula bone marrow biopsy showed predominantly blood mixed with few unremarkable bone fragments and marrow elements, no signs of malignancy.

On follow up, the right shoulder swelling has subsided and the baby was doing well with normal range of motion of the right arm. X-ray of the right shoulder confirmed the resolution of the hyperostosis which is consistent with self-limiting course of Caffey disease.

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