Congenital clubhand: causes, symptoms, diagnosis, treatment

Congenital slanting is a combined defect caused by underdevelopment of tissues along the radial or ulnar side of the upper limb. When the wrist is diverted to the radial side, the radial armor is diagnosed (tanus valga), with the deviation to the opposite side - the elbow- born innocence (manus vara).

ICD-10 code

• Q71.4 Radial congenital slanting.
• Q71.5 Elbow Congenital Skin.

What causes innate mobility?

According to the world literature, congenital slanting is registered in 1 out of 1400-100 000 children. Radiation congenital steer is more often diagnosed. The ulnar is observed 7 times less frequently than the radial one.

There is a congenital slanting of the influence of external and internal factors, which are characteristic for other congenital anomalies of development of the upper limb. External, exogenous factors include ionizing radiation, mechanical and mental injuries, medications, contacts with infectious diseases, lack of nutrition, etc. Endogenous causes are various pathological changes and functional disorders of the uterus in pregnant women, general maternal diseases, hormonal disorders, aging of the body . In this case, the timing is important, and the most unfavorable for the mother are the first 4-5 weeks of pregnancy. No hereditary factor was detected.

How does the inherent slanting appear?

Congenital slanting is characterized by a triad: radiation deviation of the hand (can be with a subluxation and dislocation of the wrist in the articulation); underdevelopment of the bones of the forearm (primarily the radius bone); anomaly of finger and hand development.

Of the other brush lesions, hypoplasia and clinodactyly of the 2nd finger, syndactyly, flexor and extensor contractures in the metacarpophalangeal and interphalangeal joints, most pronounced in the 2nd and 3rd fingers of the hand, are possible. Suffer and wrist bones located on the radial side, while observing aplasia or konkresentsiyu with other bones.

Classification

In the classification of radiodemoral, three degrees of underdevelopment of the radial bone and four types of hand are distinguished. The basis for classification is the radiographic picture.

[1], [2], [3]

Degrees of underdevelopment of the radius

• I degree - shortening of the radius is up to 50% of its normal length.
• II degree - shortening of the radius exceeds 50% of its normal length.
• III degree - complete absence of radius.

[4], [5]

Types of Brushes

The brush is characterized by the defeat of the first ray (ray - all the phalanges of the finger and the corresponding metacarpal bone).

At type 1 hypoplasia of the first metacarpal bone and the muscles of thenar is detected, type 2 is characterized by complete absence of the metacarpal bone and hypoplasia of phalanges of the 1st finger (usually a "dangling finger" is observed). Type 3 is expressed in the aplasia of the entire first ray of the hand. Type 4 bone disorders are absent.

How is it treated?

Conservative treatment

Conservative treatment (carried out from the first months of the child's life) includes exercise therapy, massage, dressing exercises to reduce existing contractures of fingers and hands, and the provision of orthesis products. However, conservative measures do not give a lasting positive result and should be considered as a preliminary preparation for the second stage - surgical. Operative treatment is recommended to start at the age of six months.

[6]

Surgery

The choice of the method of surgical treatment depends on the type of deformation.

The smaller the degree and the younger the child, the easier it is to remove the brush from the deviation. Therefore, it is recommended to begin surgical treatment up to 2-3 years of age.

The ulnar congenital slanting is characterized by deformation and shortening of the forearm, ulnar deviation of the hand, restriction of movement in the elbow joint. To a greater degree, the development of the ulnar bone, especially its distal part, is expressed. In this zone there is usually a fibrous-cartilaginous cord connecting the ulna with the bones of the wrist. The radius of the arm is arcuately curved. Her head is most often dislocated in the elbow joint anterior and outward, which determines the contracture in the elbow joint. The axis of the forearm and hand is inclined to the elbow. Changes in the brush are very diverse. Of the pathologies of the brush, the most frequently observed aplasia of one or two, usually ulnar, rays, as well as underdevelopment of the thumb. Of the other deformations of the segment, syndactyly and hypoplasia are noted.

According to the degree of underdevelopment of the ulna, the inherent deflection of the four types is distinguished.

• The first option is moderate hypoplasia - the length of the ulnar bone is 61-90% of the radius.
• The second option is pronounced hypoplasia - the length of the ulna is 31-60% of the radius.
• The third option is the rudiment of the ulna - the length of the ulna is 1-30% of the radius.
• The fourth option is ulna aplasia (complete absence).

The goals and principles of conservative treatment are identical to the goals and principles of treatment of radiation armor.

Indications for surgical treatment of ulnar congenital armor are the impossibility or difficulty of self-serving by an abnormal limb due to contracture in the elbow joint (not eliminated by conservative methods), due to shortening of the forearm and not correctable passive ulnar deviation of the hand, and finally, because of the restriction of the brush function , first of all its two-sided grasp. Surgical intervention begins with the elimination of the most functionally significant deformation. You can operate from the first year of the patient's life.

In the postoperative period, a set of restorative measures is prescribed, including exercise therapy, massage, physiotherapy, aimed at restoring the amplitude of movements and increasing the strength of the limbs.