Short hands

Introduction Multiple epiphyseal dysplasia is mainly characterized by short hands, thick fingers and short stature. Multiple epiphyseal dysplasia (MED) features characteristic osteophyte stagnation: flat, fragmented, well-known osteophyte formation, short stature and early onset osteoarthritis, dominant gene inheritance, mutations can be found On chromosome 19, Danish scholars reported a prevalence of 9.0/100,000. Multiple epiphyseal dysplasia, also known as Fairbank disease or multiple osteophyte osteogenesis imperfecta, is a rare hereditary cartilage development defect with hereditary and family history. Men are more common than women. The age of onset is young children and adolescents. It is more common in hips, shoulders, and ankles, followed by knees, wrists, and elbow joints.

Cause

The cause of this disease is unknown, and it is a rare hereditary dysplasia.

The pathological changes in this disease include the ossification center of the developing (bone) tendon. Intrachondral ossification is the division of the epiphyseal cartilage cells that are irregular. Articular cartilage is initially normal, but the patient becomes deformed during the course of the disease because of the underlying bony support. The deformity of the joint is permanent, showing degenerative changes in adults and osteoarthritis at an early stage. Diagnosis can not be made until the late childhood. The child complained of joint stiffness, pain, and lameness like a duck step. Such children are short and have short limbs. Usually the elbows and knees are flexed and contracted, but the intelligence is unaffected.

an examination

Related inspection

CT examination of bone and joint and soft tissue

[clinical manifestations]

Mainly manifested as pain in the hip joint and knee joint, limited mobility, difficulty walking, and a swinging gait. Shoulder joint activity is also limited. The bone ends are often large, and a small number of patients have joint flexion deformities or joint relaxation. The hand becomes shorter, the fingers become thicker, and the figure is short. Because the disease affects the long bones of the extremities more than the spine, it is characterized by a certain degree of short-legged dwarfs, in addition to knee varus, valgus, unequal length of both lower extremities and posterior kyphosis.

diagnosis

The diagnosis of this disease mainly depends on the X-ray.

X-ray findings: the symmetry osteophytes center appears late, develops slowly, and the time delay of fusion with the backbone. The characteristic manifestation is irregular ossification and abnormal density of the ossification center. It is often spotted or mulberry-like, with many small scattered ossification centers surrounding the large ossification center, but not as many as the skeletal epiphyseal dysplasia. These multiple ossification centers make the bone ends larger. The epiphysis at the lower end of the humerus is inclined from the inside to the outside, the depth is reduced, and the tibia is elongated. The talus morphology was changed to accommodate tibial bone deformation (50%). The long backbone is shorter than normal, and the skull and teeth are normal. Can be divided into two types:

1Riing type (soft type). There are multiple epiphyseal dysplasia, flattened bones, and minor bone invasion in the hand;

2Fairbank type (heavy), small callus, irregular carpal bone delayed ossification, metacarpal and phalanx changes.

Differential diagnosis

The deformity of the hand is a clinical manifestation of acromegaly in involuntary movement. "" deformity: the interosseous muscle appears in the hand in the late stage of the iliac muscle atrophy, and the large and small intermuscular muscles atrophy, forming a deformity of the hand. Peronial myoatrophy, also known as Charcot-Marie-Tooth disease (CMT), is the most common group of peripheral neuropathies, accounting for approximately 90% of all hereditary neuropathies. The common features of this group of diseases are the onset of children or adolescents, chronic progressive sacral muscle atrophy, and the symptoms and signs are relatively symmetrical. Most patients have a family history. Because of the main clinical features of iliac muscle atrophy, it is also known as peroneal myoatrophy. According to neurophysiological and neuropathological findings, CMT is classified into type I and type II, CMTI type is called hypertrophic type, and type CMTII is called neuronal type.

Boxing hand deformity is a series of deformed severely distorted or deformed deformities after hand burns: twisted, fist-shaped and crippled. Loss of hand function. The back side of the hand is weaker, so the burns are deeper, often occurring at the same time as the face, or as part of a multi-site or large-area burn. Treatment should pay great attention to early treatment, in an effort to preserve hand function. Advanced treatments are organized according to the specific conditions of tissue transplantation, in an effort to maximize the recovery of the hand's labor function.

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