bubble_chart Overview

Also known as Caffey's disease. It is a temporary condition of infantile cortical hyperostosis, where the muscles and fasciae adjacent to the affected bones may also be involved.

bubble_chart Etiology

The cause of the disease is unknown. It is a congenital bone developmental disorder, belonging to autosomal dominant inheritance. However, Fairbank believes that this disease may be an infectious condition caused by a virus.

bubble_chart Pathological Changes

The primary lesion is in the periosteum, with cells exhibiting numerous nuclear divisions, accompanied by mucinous edema. The outer fibrous tissue of the periosteum disappears and adheres to adjacent muscles, fasciae, and tendons. As the condition progresses, fibrous tissue reappears in the outer layer of the periosteum, leading to the formation of new bone beneath the periosteum. The bone marrow shows typical fibrotic changes. During the convalescence stage, the proliferated new bone beneath the periosteum gradually disappears, the thickened cortical bone thins progressively from the inside outward, and the medullary cavity returns to normal.

bubble_chart Clinical Manifestations

The onset typically occurs within 10 weeks, with infants first exhibiting dysphoria, restlessness, and fever. Subsequently, pain in the affected area appears, accompanied by localized swelling. The soft tissue swelling is diffuse, without redness or heat, and the local lymph nodes are not enlarged. The swelling is firm to the touch and does not indent. The most common site of involvement is the mandible, accounting for up to 75% of cases, making facial symptoms the most frequent and prominent. If long bones are affected, the limbs may exhibit pseudoparalysis due to pain. Some infants may also present with anemia.

X-ray findings initially show grade I cortical thickening, followed by the gradual development of obvious subperiosteal new bone formation. Almost all bones except the fingers and toes can be involved, with the mandible being the most commonly affected, followed in order by the ribs, clavicle, ulna, radius, scapula, tibia, and fibula. In long bones, the most pronounced changes occur in the diaphysis, while the epiphysis and metaphysis are usually spared. The bones may become curved, and the limbs may lengthen. In rare cases, excessive new bone formation may lead to misdiagnosis as a malignant tumor. Most patients recover spontaneously within a few months without any residual traces, though a minority may retain mild signs of the disease or limb overgrowth.

bubble_chart Auxiliary Examination

Anemia, elevated white blood cell count, increased erythrocyte sedimentation rate, and elevated AKP.

bubble_chart Diagnosis

The onset of this disease occurs in early infancy, and diagnosis is generally not difficult. However, individual cases may recur. By this time, the child is older and may even reach adulthood, with symptoms appearing and disappearing intermittently. In such cases, a detailed history of the infant period should be obtained.

bubble_chart Treatment Measures

The disease tends to heal on its own. It usually resolves within a few months, so no special treatment is required. Some also recommend hormone therapy.