Answer of May 2009

Clinical History:

A 5-months-old boy presented with progressive left facial swelling with mild skin erythema for one week. There was associated increased irritability and mild drooling of saliva. No associated fever, injury or feeding problem detected. The condition was not improved with oral antibiotics prescribed by the general practitioner. Radiographs and CT scan of the mandibles were performed.

                               Fig 1. AP                                                                   Fig 2. R Oblique

                            Fig 3. L Oblique                                         Fig 4. Axial CT

Diagnosis:

Caffey's Disease (Infantile Cortical Hyperostosis)

Discussion:

Radiographs of the mandible show solid undulating periosteal reaction and subperiosteal new bone formation over left side of mandible associated with adjacent soft tissue swelling. CT of the mandible shows thick solid periosteal reaction and subperiosteal new bone formation over left side of mandible associated with swollen left masseter muscle more clearly. Caffey's Disease, also called infantile cortical hyperostosis is an uncommon self-limiting proliferative bone disease of infancy. Caffey and Silverman first reported this disease as a distinct entity in 1945. The exact etiology is unknown. Clinical triad consists of fever, soft tissue swelling and hyperirritability. Presentation is usually before 5 months of age with no sexual or racial predilection. Mandible being the most common site of involvement (75%), followed by clavicle, rib, scapula and skull. The clinical course is highly variable, ranging from self-limited to protracted, with the self-limited course being the most common pattern. Laboratory investigations may show leukocytosis, elevated erythrocyte sedimentation rate (ESR), elevated C-reactive protein (CRP), elevated alkaline phosphatase level (ALP), thrombocytosis and iron deficiency anemia. Plain radiographs may show soft tissue swelling, periosteal reaction and cortical hyperostosis. CT will shows similar findings but is generally avoided because of its high radiation burden. MRI adds little important additional information for the clinical evaluation of Caffey's Disease, but is useful when infection or neoplastic cause is considered. Overall, the age of onset, clinical signs, laboratory results and typical radiographic features are clues for the correct diagnosis of Caffey's Disease. There is no specific treatment for Caffey's Disease. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to alleviate the symptoms. Corticosteroids may be helpful in severe cases, but these agents do not have any effect on the bone lesions.