Striated hypertrophy of limbs
Introduction
Introduction to limb striated hypertrophy Melorheostosis is also known as bone candle disease, single-arm candle-like hyperosteogeny, and Leri osteoporosis candlebone. For a rare shape of the localized bone hyperplasia, from the X-ray film, it seems that the molten wax flows down the backbone. It is believed that congenital malformation is a kind of periosteal bone dysplasia caused by submucosal telangiectasia. The incidence begins from children, and men and women are equal. Most of them invade single limbs, and lower limbs are more common in upper limbs. basic knowledge The proportion of illness: 0.005%-0.007% Susceptible people: no specific population Mode of infection: non-infectious complication:
Cause
Limb striated hypertrophy
Etiology
The etiology of this disease is unknown, and it is believed that multiple congenital malformations are a kind of periosteal bone dysplasia caused by submucosal telangiectasia. In the literature, there was a report accompanied by familial brittle osteopetrosis, so it is thought that it may be caused. The second mutation of the isolated familial fragility gene site, no hereditary and family history, the incidence of children from the beginning, men and women are equal, most of the violation of single limbs, lower limbs are more common in the upper limbs.
[pathological changes]
The epithelium and endosteal hyperplasia are irregularly hardened. Under the microscope, the bone hyperplasia area is a tight lamellar bone, arranged in disorder, Harvard's tube is distorted, immature and mature bone tissue interweave, osteoblast activity Increased, osteoclast activity decreased, so new bone formation, other structures are normal.
Prevention
Limb striate hypertrophy prevention
The disease is a congenital disease, so there is no effective preventive measure, the lesion is progressive, the disease progresses rapidly during the developmental period, and it is slow after adulthood and does not affect life.
Complication
Limb striate hypertrophy Complication
The disease is a rare congenital disease, and its complications include the following:
1, the foot valgus.
2, knee valgus.
3, bone bending.
4, knee joint swelling and so on.
In the advanced stage, ectopic hyperplasia can protrude into the joint or even form a bone bridge across the joint, causing joint movement disorders.
Symptom
Limb striated hypertrophy symptoms Common symptoms Shoulder joint activity constrained joint swelling medulla hyperplasia limbs cold dull pain joint swelling foot valgus hypoxic ischemic nerve injury
The clinical manifestations of this disease mainly include the following:
(1) Pain is the most common, accounting for about 1/2, and the older the age, the more obvious the pain is, usually it is dull or dull, and the activity is aggravated.
(2) The joint activity of the affected limb is limited, which is caused by bone hyperplasia around the joint and bone deposition in the soft tissue, which is rarely caused by the destruction of the joint surface.
(3) Bone deformity.
(4) Local palpation can touch the surface of the bone, which is uneven, hard as a stone, often with valgus, knee valgus, bone bending, and swelling of the knee joint.
(5) The affected limb may develop swelling, chills, sweating and induration. There are also patients with scleroderma, which are sympathetic and ischemic, sometimes with hyperplastic bone.
Examine
Limb striate hypertrophy
The auxiliary method used in this disease is mainly X-ray examination:
X-ray performance: It occurs in the long bones of the extremities. It is more common in single limbs. There is irregular bone hyperplasia on one side of the backbone. The shape of the backbone is destroyed, just like the melting wax of the burning candle flows down from the side. The bone has no structure, and the epiphysis and short bone often appear as spots. It can surpass the joint to invade the distal bone, but does not invade the joint surface. The pelvis and scapula also show increased density and spots. The skull, spine and ribs are rare. .
Diagnosis
Diagnosis and differentiation of limb striated hypertrophy
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
For typical cases, because of their special performance, diagnosis is not difficult, and some cases need to be differentiated from the following diseases.
1. Osteosclerosis (marble osteosis).
2, bone spot disease (fragile osteosclerosis).
3, bone fiber structural disorders.
4, inflammatory bone sclerosis and so on.
