Hockey stick player

Introduction The hockey stick is a rare congenital malformation, but it is more common than the ulna. The onset is often bilateral, and unilateral is not uncommon. There are more male patients than females, with a ratio of about 3:2. In the neonatal period, the forearm is bent to the temporal side, the thumb is turned to the inside, and the forearm is bent at 90°.

Cause

The real cause of congenital absence of the humerus remains unclear. According to Gegenbauer theory, the upper limb consists of one trunk and four secondary rays. The first ray is composed of the humerus, the scaphoid, the large angle bone, the first metacarpal and the thumb. When the development of the first pair of rays is inhibited, congenital absence of the humerus occurs, and the thumb is often absent. Recently, it has been thought to be related to the inhibition of the growth of the cervical 7 nerve root or the phrenic nerve.

classification

1. This deformity can be divided into three types, and type A is dysplasia of the tibia. The distal part of the humerus is missing. The ossification of the epiphysis was delayed, the distal end of the humerus was short, and the styloid process was the same level as the ulnar styloid process. The proximal humerus epiphysis has a normal relationship with the elbow joint. The humerus shortened lightly, the scaphoid and Lei multi-angle bones were dysplasia, the sacral finger was small or absent, and the wrist joint was partial, but it was stable. Type B is absent in the humerus (Fig. 1A). The distal end of the humerus is not developed, and the proximal humerus is normal to maintain a certain degree of stability of the elbow joint. The humerus is underdeveloped and merges with the ulna to form a type of ulnar and ulnar fusion. Sometimes the tibia and the humeral head merge. The ulna shortens and thickens and bends to the cheekbones. The wrist is unstable and the hand is skewed to the temporal side. Type C is completely absent of the tibia (Fig. 1B). This type is the most common, accounting for about 50% of the original distortion. Because the wrist has no tibial support, the soft tissue of the forearm is severely contracted, and the hand and forearm form a 90° or more 90° deviation. When the elbow flexes, the hand is even parallel to the upper arm. The temporal ray is completely absent, including the scaphoid, the large angle bone, the first metacarpal and the thumb bone. If the thumb is present, there are often hypoplasia or floating fingers. The humerus is short, the humeral head is underdeveloped or absent, and the humerus of the distal radius is delayed.

2, the wrist between the wrist is a fiber connection, no articular cartilage coverage, the wrist joint dislocation to the temporal and volar side, the forearm temporal soft tissue contracture is obvious. If the deformity is not treated, the deformity is aggravated as the bone grows. The metacarpophalangeal joint is overextended and the flexion is limited. Closed interphalangeal joint fixed flexion deformity. The proximal interphalangeal joint and metacarpophalangeal joint X-ray showed normal. Joint stiffness may be associated with abnormalities in the extensor digitorum. The knuckle contraction is gradually reduced from the ulnar side to the ulnar side.

In 1/4 of the cases, the elbow joint was stiff and straight. If the elbow joint is straight and contracted, it can be regarded as a contraindication for centralization of the wrist.

Muscles are also affected. Defects or hypoplasia of the extensor tendon, fibrosis or fusion. The anterior muscle of the anterior muscle, the long and short wrist of the temporal extension, the diaphragm and the supinator muscle are often absent. The long flexor hallucis longus, the long extensor pollicis longus and the abductor abductor muscle and the large fish muscle are also often absent. However, the interosseous muscles, the sacral muscles and the small intermuscular muscles are generally not affected. The extensors of the wrists and fingers are normal, but often merge together. The flexor digitorum has a large variation and can be hypoplasia, fibrosis or fusion with the flexor digitorum.

The biceps brachii lacks long heads and short heads, but the abnormal points are often attached to the joint capsule or the remaining humerus or humerus. The chest, small muscles, and deltoid muscles are present, but the endpoints are abnormal or merge with the deltoid or diaphragm muscles.

3, nerve involvement: the phrenic nerve and ulnar nerve are normal, the musculocutaneous god is often absent, the phrenic nerve often terminates the elbow, and the sacral side of the hand is dominated by the median nerve. The median nerve is thicker than normal and is located under the deep fascia of the forearm. The median nerve is thicker than normal, located under the deep fascia of the forearm, and should be avoided during surgery.

4, vascular involvement: the presence of the ulnar artery, often become the main blood vessels supplying the forearm and hands. The interosseous artery is well developed. Abnormalities of the radial artery and the palmar artery, and the degree of vascular involvement is associated with dysplasia of the temporal side. The radial side of the forearm is dominated by the interosseous anterior artery, starting from the ulnar artery and accompanying the median nerve. The radial artery is degenerated or absent.

an examination

Related inspection

Doppler echocardiogram electrocardiogram chromosome

There are several methods for examining this disease:

(1) Ultrasound examination:

Congenital defects of the humerus occur at the distal end of the humerus, and the muscles that innervate the wrist are also contracted and deformed, resulting in shortening of the forearm, bending of the ulna to the tibia, and deformation of the wrist and handcuffs. Some scholars call it a "abnormal hand." Ultrasonography can show a specific deformity image of the absence or shortening of the tibia, short and curved forearm of the residual limb, and hand deflection.

(2) X-ray inspection:

Clinically, the bilateral lateral humerus and the orthotopic position of both sides of the humerus can be found. The humerus of the affected side can be found to be absent. At the same time, other bone deformities, such as the ulnar bones or the metacarpal and phalanx defects, can be found. It is skewed to the temporal side and forms a vertical shape with the ulna.

Differential diagnosis

Differential diagnosis of hockey sticks:

1. The deformity of the hand: The deformity of the hand is a clinical manifestation of the hand and foot hyperkinesia in involuntary movement.

2, "hands" deformity: skeletal muscle atrophy in the hand of the interosseous muscle, large and small intermuscular muscle atrophy, the formation of hand deformity. 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.

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