infantile cortical hyperplasia
Introduction
Introduction to infantile cortical hyperplasia Infantile cortical hyperostosis, also known as Caffey disease, is a disease that invades bones and muscle fascia during infancy. It is characterized by the formation of new subperiosteal bone in long tubular and flat bones, as well as swelling and pain in the affected area. Most of the men were 6 months old. However, it has recently been reported that this disease can occur in children 11 years of age. basic knowledge The proportion of the disease: the incidence of infants is about 0.003% Susceptible people: children Mode of infection: non-infectious Complications: pleural effusion
Cause
Causes of infantile cortical hyperplasia
(1) Causes of the disease
The cause and pathogenesis of this disease are unclear. Most cases have long-term low fever and increased erythrocyte sedimentation rate. Therefore, some people suspect that the bone is mildly infected, but the bacterial culture in the diseased part is aseptically grown, because the disease can occur in the fetus, but Most of them occur within half a year after birth, and can occur in a family of siblings or twins. Therefore, some people think that they may be autosomal dominant hereditary diseases. Because of the better response to adrenocortical hormones, sometimes the disease can occur due to food. Consider allergic factors.
(two) pathogenesis
In the early stage, there were obvious inflammatory changes in the periosteum and its surrounding soft tissues. Subsequently, the inflammation gradually subsided, leaving the hypertrophic periosteum and subperiosteal new bone, and the intimal hyperplasia of the small artery in the diseased bone and surrounding soft tissue changed, causing hypoxia to be reactive. The cause of periosteal hyperplasia.
The predilection site is the mandible and ulna, followed by the humerus, clavicle, scapula and rib. If the bilateral lesions are bilateral, they are often asymmetrical. Many of the 12 cases treated by Beijing Children's Hospital are multiple lesions, affecting 10 cases of the humerus. There were 5 cases of ulna and radius, 4 cases of femur, 4 cases of clavicle, 3 cases of scapula, 3 cases of mandible, 3 cases of ribs, and no bioinflammation of submucosal new bone. The bone hypertrophy was Normal immature slab bone is formed, the medullary cavity is rich in blood vessels, there is fibrosis, and there is also osteoclast activity, no trace of periosteal hemorrhage.
Prevention
Infant osteocortical hyperplasia prevention
The disease can be self-healing, the prognosis is good, no special treatment is needed. Application of hormones can improve symptoms in the acute phase and has no significant effect on bone repair. The clinical symptoms disappeared within 1 month, and the laboratory examinations returned to normal. Generally, the bones gradually recovered within 6 to 9 months.
Complication
Complications of infantile cortical hyperplasia Complications pleural effusion
Individual lesions with severe or recurrent episodes will leave limb deformities, pleural effusion, exophthalmia and diaphragmatic paralysis. There are many reports on the complications of this disease, such as enlarged bone marrow cavity and thin cortical bone; Ribs, ulna and humerus fusion; humeral head dislocation, humerus or femur anterior curvature, lower limb unequal length, facial asymmetry, etc.
Symptom
Symptoms of infantile cortical hyperplasia Common symptoms ESR increases fast soft tissue swelling irritability
The most common clinical manifestations are easy irritability and local tumors. The beginning of the tumor may have tenderness, but the local temperature is not high, and it is not red. At the beginning of the disease, every fever, erythrocyte sedimentation rate increases, serum alkaline phosphatase increases. And anemia, the typical X-ray is seen as a large number of new bone under the periosteum, the diseased bone becomes thick, the main symptoms are three:
1. General performance: There may be pale, anemia, irritability with mental symptoms, and local lymph nodes do not increase.
2. Deep soft tissue swelling: hardening and tenderness lesions can be multiple or single. In the same sick child, the degree of lesions of each affected bone can be expressed in different degrees. The swelling appears before the X-ray film shows the lesion, and the regression is also better than the X-ray. The on-chip lesion disappeared early, the local is not red or hot, never purulent, but can relapse without cause, in the original or in another part.
3. Cortical bone hypertrophy: found in long tubular bone and flat bone, such as the jaw, shoulder blade, frontal bone, tibia and ribs.
In addition, there may be symptoms such as pseudospasm and pleurisy. Most of the sick children can heal within 6 to 9 months after the onset of the disease, and individual cases can be repeated until childhood.
Examine
Examination of infantile cortical hyperplasia
1. Peripheral blood examination: increased white blood cells, decreased hemoglobin and red blood cell count.
2. Blood biochemical examination: ESR increases, serum alkaline phosphatase increases when the disease is high.
The tubular and flat bones of the whole body are characterized by thickening and sclerosis of the cortical bone. Common tendon sites are the mandible, scapula, ribs, clavicle and long bones of the extremities. The periosteal hyperplasia begins to appear on the lateral side of the cortical bone below the swollen soft tissue. Layered thickening, and finally combined with the cortical bone to thicken the cortical bone, dense and hard, when the lesion invades the tubular bone, only a thick layer of subperiosteal new bone shadow is seen around the bone, sometimes surrounded by a tube The backbone, the edge can be irregularly wavy, but does not involve the epiphysis and metaphysis. Due to the neoplastic hyperplasia, the medullary cavity can be narrowed or disappeared. In the early stage of the lesion, the soft tissue around the bone is thickened and swollen, and the affected area is thickened. Absorption and recovery to normal, later than clinical symptoms and soft tissue swelling.
Diagnosis
Diagnosis and diagnosis of infantile cortical hyperplasia
According to clinical manifestations and laboratories, auxiliary examinations, except for infectious diseases, can be diagnosed.
The disease can be confirmed from the symptoms of the sick child and X-ray film. Osteomyelitis, old fractures, bone tumors and vitamin A poisoning should also be excluded at the time of diagnosis.
