Widening of the proximal end of the femur

Introduction The predilection sites of hereditary malformation of cartilage are followed by the femur, the proximal and distal ends of the humerus and the proximal end of the tibia. Typical X-ray findings are: bone formation defects and skeletal deformities, hip valgus and thickening of the proximal femur, and the ulnar deflection of the wrist is relatively shortened.

Cause

It is a congenital skeletal dysplasia, often with a family history, and has been reported in 4 or more generations. It is generally considered to be a hereditary bone disease and is an autosomal dominant hereditary disease. The cause of the disease is still unclear, and may be: 1 congenital embryo defect; 2 misplaced displacement of the epiphyseal plate; 3 residual immature cells in the periosteal inner layer or metaplastic chondrocytes gradually grow to form osteophytes. At present, the understanding of this disease has not been completely unified. Some scholars believe that the disease is a skeletal dysplasia, which is more common in children up to the age of 20 years old, male: female about 3:1. The general clinical manifestations are palpable bony masses, which are deformed due to shortening and bending of the bones, and the activity near the joints is limited, often symmetrical.

an examination

Related inspection

Limbs and limbs, bones and joints, bone marrow, bone marrow, bone density, bone density, bone and joint, soft tissue, CT examination

X-ray examination showed: proximal femur and femoral neck thickening; two distal femurs, proximal and distal humerus, proximal humerus, multiple epiphyses at the distal ends of the humerus, and the affected bones were enlarged and thickened; The distal part of the left tibia is compressed by the bone and the cortex is thin.

Differential diagnosis

Generally diffuse, symmetrical onset, all bones of cartilage bone can become the site of osteochondroma, the metaphyseal end of the growing bone, the soft bone tumor is multiple and large, especially adjacent to the knee, shoulder, hip, wrist The area of the sputum, but it occurs at the elbow and is rare and light. Multiple hereditary osteochondromas are also common in the trunk bone, occurring near the ossification center of the second or osteogenesis, in the scapula, mainly near the spine, inferior horn, shoulder, glenoid and condyle; in the pelvis, Mainly sputum; in the spine, mainly in the spinous processes and transverse processes; in the ribs, more common in the osteochondral junction or the posterior end. In addition to the calcaneus with the second nucleus, multiple hereditary osteochondromas do not occur in the carpal and humerus because these bones occur in the same way as the epiphysis.

It can be seen and touched a fairly symmetrical multiple osteophyte. In more severe cases, its performance is characteristic, and diagnosis can be made by physical diagnosis alone. In more severe cases, limb shortening can occur, sometimes very pronounced and diffuse, so that it resembles achondroplasia. However, multiple hereditary osteochondromas are not characterized by achondroplasia and cartilage growth is normal. In fact, limb shortening is not caused by abnormal cartilage growth, but because the proliferative potential of cartilage is consumed by external branches (osteochondroma) and is not used for the extension of bone. Because the external dispersion is unevenly distributed on the circumference of the backbone and the bones of the upper limbs and the lower legs, the limb shortening often merges with the deformity, for example, there may be knees, ankles, elbows, wrists, or valgus, most typical. The deformity occurs in the forearm and chest. Because the osteochondroma that occurs in the wrist is more than the elbow, the bone extension of the bone 3/4 occurs at the distal end, and the bone extension of the tibia 4/5 occurs at the proximal end, so the ulna is always shorter than the humerus. Malformation, that is, the humerus is convex to the lateral and dorsal sides, the hand is biased, and the humeral head is dislocated.

X-ray examination showed: proximal femur and femoral neck thickening; two distal femurs, proximal and distal humerus, proximal humerus, multiple epiphyses at the distal ends of the humerus, and the affected bones were enlarged and thickened; The distal part of the left tibia is compressed by the bone and the cortex is thin.

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