Backbone Continuity
Introduction
Introduction to backbone continuation Diaphysealaclasis is a developmental disorder of cartilage, which is characterized by multiple exostoses and metaphyseal malformations. It is also called metaphysialaclasis. Jaffe is named hereditary multiple osteophytes, also known as osteochondroma. basic knowledge The proportion of illness: 0.002% Susceptible people: no specific population Mode of infection: non-infectious Complications: fracture, paraplegia, knee inversion
Cause
Cause of backbone continuation
The disease is a congenital malformation, is autosomal dominant, and occurs in childhood or adolescence, more male than female, about 3:1.
65 to 75% of patients have a family history, are hereditary, and the cause is unknown. The possible causes are:
1 periosteal dysplasia, thin cortical bone and incomplete periosteum, can not well limit bone outgrowth.
2 Insufficient ability of osteoclasts to act, resulting in bones not being well shaped, which explains the broad trumpet shape of the metaphysis.
3 The development of the perichondrium is defective, causing the cartilage to overgrow and transforming from cartilage to bone, because all the displaced cartilage has a tendency to malignant, which explains the tendency of exogenous osteophytes to have malignant changes.
Prevention
Backbone continuity prevention
The disease is congenital malformation, is autosomal dominant, no effective preventive measures, early detection and early diagnosis and treatment is the key to the prevention and treatment of this disease, and because of the possibility of malignant transformation of this disease, it is necessary to strictly observe the disease at the time of diagnosis. Changes in the situation should pay special attention to signs that suggest a malignant change.
Complication
Complications of backbone continuation Complications, fractures, paralysis, knee varus
If the tendon or nerve is compressed, it can cause the corresponding symptoms. The epiphysis can also be fractured, but it can heal quickly. The epiphysis of the vertebral body can produce paraplegia, such as knee varus. After the growth of mother bone stops, it generally does not continue to grow up. If the rate of growth of the epiphysis suddenly increases, or continues to increase after stopping growth, there is a possibility of malignant transformation. The incidence of malignant transformation is 2 to 10%. On the X-ray film, if the tumor presents an unclear boundary, the bone is destructive, and there are irregular spots, that is, there should be malignant changes. The bone around the hip joint, the shoulder blade and the like are easily malignant, and should be paid more attention.
Symptom
Symptoms of continuation of the backbone Common symptoms Paraplegic cartilage ossified varus
The lesions are heavy, the body is short, the limbs are not equal, and the bones have different degrees of curvature. There are hard, irregularly shaped, non-pushable masses near the osteophytes of the long bones, ie, epiphyses, mild pain, sometimes due to friction. There is a sac formed next to it, which can be accompanied by inflammation. If the tendon or nerve is compressed, it can cause the corresponding symptoms. The epiphysis can also fracture, but it can heal quickly. The vertebral body can grow parasiticly like the growth into the spinal canal. Sometimes it can be accompanied by knee varus.
Examine
Examination of backbone continuation
X-ray findings: characteristic manifestations of poor dry metaphyseal deformity, short backbone and multiple exostoses, metaphyseal into a "horn" shape, ulnar shortening, humerus bending, humeral head subluxation, sometimes To be distinguished from Madelung's malformation, there are osteophytes protruding outward at the metaphysis, the direction of which is often opposite to the joints, the number of which varies in size, and the shape is different: conical, mushroom-shaped, cauliflower-shaped, etc. The structure is consistent with the mother bone, and the cartilage cap cannot be developed. Therefore, the actual size of the mass is larger than that seen on the X-ray. The osteophytes in the forearm and calf can cause the adjacent bone to be pressure-absorbing.
Diagnosis
Diagnosis and identification of backbone continuation
The diagnosis of this disease is not difficult, there are hereditary familial features, and the presence of multiple osteophytes. The dry shape of the metaphysis is trumpet-like. It is a characteristic manifestation. It is often confused with cartilage dysplasia (Ollier disease). The latter is mainly manifested as lesions inside the bone, and it is more common in the finger bones, and there is no hereditary difference.
The disease should be differentiated from achondroplasia (ollier's disease) and renal rickets:
1, achondroplasia (ollier's disease), limbs short and unequal length and metaphysis dysplasia similar; but the lesion is not involving the metaphysis and often involving the epiphysis, no serrated pre-calcification zone, etc., no segmentation Phenomenon, the lesion range and the normal bone boundary are distinct, often with sharp marginal phase separation and metaphyseal dysplasia is progressive and obvious, in addition to the incidence of limbs, the pelvis, spine, skull can also be affected, visible in the short bones of the extremities The deformation of the symmetry "tower-like".
2. The X-ray findings of metaphyseal dysplasia and renal rickets are similar, except for the lesions of the metaphysis, the cortical density and thickness of the cancellous bone and the backbone of the metaphysis and metaphysis are normal, and renal rickets It is different.
