infantile cortical hyperplasia
Introduction
Introduction to infant cortical hyperplasia Infant cortical hyperplasia, also known as Caffey's disease, is a temporary condition of hyperplasia of the baby's cortex, which may also be affected by muscles and fascia adjacent to the bone. It is characterized by congenital bone development disorder and is an autosomal dominant inheritance. But Fairbank believes the disease may be an infectious disease caused by a virus. The cause of the disease is still unclear. It is a congenital bone development disorder, mainly characterized by periosteal lesions. The cells are mostly mitotic, with mucinous edema and mucinous edema. The fibrous tissue of the outer layer of the periosteum disappears and adheres to adjacent muscles, fascia, and tendons. As the disease progresses, the outer layer of the periosteum reappears with fibrous tissue and leads to new subperiosteal bone. The bone marrow is a typical fibrotic change and is an autosomal dominant inheritance. But Fairbank believes the disease may be an infectious disease caused by a virus. basic knowledge The proportion of illness: 0.003% Susceptible people: children Mode of infection: non-infectious Complications: anemia, dyskinesia
Cause
Causes of infant cortical hyperplasia
The cause is still unclear. It is a congenital bone development disorder, mainly characterized by periosteal lesions. The cells are mostly mitotic, with mucinous edema and mucinous edema. The fibrous tissue of the outer layer of the periosteum disappears and adheres to adjacent muscles, fascia, and tendons. As the disease progresses, the outer layer of the periosteum reappears with fibrous tissue and leads to new subperiosteal bone. The bone marrow is a typical fibrotic change and is an autosomal dominant inheritance. But Fairbank believes the disease may be an infectious disease caused by a virus.
Prevention
Infant osteocortical hyperplasia prevention
The disease can be self-healing, the prognosis is good, no special treatment is needed, and the application of hormone can improve the symptoms in the acute phase, and has no obvious effect on bone repair. The clinical symptoms disappear within one month, and the laboratory examination also returns to normal, generally 6~ 9 months of bone repair gradually.
Complication
Infant osteocortical hyperplasia complications Complications, anemia, dyskinesia
A small number of anemia, more leukocytosis, erythrocyte sedimentation rate, a small number of patients can be extended to several years, called chronic infant cortical hyperplasia, can leave limb deformities, dyskinesia.
Symptom
Infant cortical hyperplasia symptoms common symptoms persistent fever irritability soft tissue swelling
Often started within 10 weeks, the baby first showed irritability and fever, and later there was pain in the affected area, accompanied by local swelling, swelling of soft tissue was diffuse, not red or hot, local lymph nodes were not swollen, no depression Hard, the most vulnerable part of the disease is the mandible, up to 75%. Therefore, the symptoms of the face are the most common and most obvious. If the long bone is involved, the limb can cause false sputum due to pain, and some infants can be accompanied. Have anemia.
X-ray findings: firstly for mild cortical bone thickening, and later obvious subperiosteal new bone formation, the whole body bone team finger and toe outside, can be affected, the most common is the mandible, followed by ribs, clavicle, ulna , humerus, scapula, humerus and humerus, the most obvious part of long bone lesions is the backbone, while the epiphysis and metaphysis are often invaded, bones are bent, limbs grow, and in some cases, when new bone formation is too much, it can be misdiagnosed as malignant. Tumors, most patients can heal themselves after a few months without leaving any traces, but in a few people, there are traces of minor lesions and limbs that are too long.
The age of onset of this disease is early in the baby, and the diagnosis is generally not difficult, but individual cases can recur. At this time, the child is old enough, and can even be extended to adulthood, and the symptoms are more and more frequent. At this time, the medical history of the infancy should be inquired in detail. .
Examine
Examination of infant cortical hyperplasia
Biochemical examination: anemia, increased white blood cells, increased erythrocyte sedimentation rate and increased AKP.
Imaging examination: X-ray, the first manifestation of mild cortical bone thickening, and later obvious subperiosteal new bone formation.
Diagnosis
Diagnosis and diagnosis of infant cortical hyperplasia
Differential diagnosis
(1) Infant osteomyelitis: There is a clinical course of acute infection. The local soft tissue has red, swollen, hot and painful inflammatory manifestations. The disease of the long bones of the extremities is often misdiagnosed. Follow-up observation is very important.
(2) scurvy: more common in artificially fed infants, X-ray manifestations of subperiosteal hematoma calcification, surrounded by the entire backbone and metaphysis end of the encapsulation, dry sputum endemic scurvy symptoms, osteophytes "ring sign."
(3) Vitamin A poisoning: Some symptoms and X-ray signs of this disease have similarities with infant cortical hyperplasia, and this disease has a history of taking excessive vitamin A for a long time.
(4) Trauma, small infants are more common in birth trauma, bleeding due to muscle damage, swelling, gradual calcification and ossification after 2-3 weeks.
In addition, it should be differentiated from congenital syphilis, bone disease after fracture, traumatic subperiosteal hemorrhage, cellulitis, bone tumor, lymphadenitis, progressive dysplasia.
