Congenital club hand: causes, symptoms, diagnosis, treatment

Congenital clubhand is a combined defect caused by underdevelopment of tissues on the radial or ulnar side of the upper limb. When the hand deviates to the radial side, the diagnosis is radial clubhand (tanus valga), when it deviates to the opposite side, the diagnosis is ulnar congenital clubhand (manus vara).

ICD-10 code

• Q71.4 Congenital radial clubhand.
• Q71.5 Congenital ulnar clubhand.

What causes congenital clubhand?

Congenital clubhand, according to world literature, is registered in 1 out of 1400-100 000 children. Radial congenital clubhand is diagnosed more often. Ulnar clubhand is observed 7 times less often than radial.

Congenital clubhand occurs as a result of external and internal factors, which are also typical for other congenital anomalies of the upper limb. External, exogenous factors include ionizing radiation, mechanical and mental trauma, medications, contact with infectious diseases, malnutrition, etc. Endogenous causes include various pathological changes and functional disorders of the uterus in a pregnant woman, general diseases of the mother, hormonal disorders, and aging of the body. In this case, the timing of exposure is of great importance, and the first 4-5 weeks of pregnancy are considered the most unfavorable for the mother. No hereditary factor has been identified.

How does congenital clubhand manifest itself?

Congenital clubhand is characterized by a triad: radial deviation of the hand (may be with subluxation and dislocation of the hand at the elbow-wrist joint); underdevelopment of the bones of the forearm (primarily the radius); anomaly in the development of the fingers and hand.

Other hand lesions may include hypoplasia and clinodactyly of the second finger, syndactyly, flexion and extension contractures in the metacarpophalangeal and interphalangeal joints, most pronounced in the second and third fingers. The bones of the wrist located on the radial side are also affected, with aplasia or concrescence with other bones observed.

Classification

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

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Degrees of underdevelopment of the radius

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

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Types of brushes

The hand is characterized by damage to the first ray (ray - all phalanges of the finger and the corresponding metacarpal bone).

Type 1 shows hypoplasia of the first metacarpal bone and thenar muscles, type 2 is characterized by the complete absence of the metacarpal bone and hypoplasia of the phalanges of the first finger (usually a "dangling finger" is observed). Type 3 is expressed in aplasia of the entire first ray of the hand. In type 4, there are no bone abnormalities.

How is congenital clubhand treated?

Conservative treatment

Conservative treatment (carried out from the first months of the child's life) includes exercise therapy, massage, redressing exercises to reduce existing contractures of the fingers and hand, and provision of orthotic devices. However, conservative measures do not provide a stable positive result and should be considered as preliminary preparation for the second stage - surgical. Surgical treatment is recommended to begin from the age of six months.

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Surgical treatment

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

The lower the degree and the younger the child, the easier it is to bring the hand out of deviation. Therefore, it is recommended to start surgical treatment before the age of 2-3 years.

Congenital ulnar clubhand is characterized by deformation and shortening of the forearm, ulnar deviation of the hand, and limited movement in the elbow joint. Underdevelopment of the ulna, especially its distal part, is expressed to a greater extent. The fibrocartilaginous strand connecting the ulna with the carpal bones is usually located in this area. The radius is arcuately curved. Its head is most often dislocated in the elbow joint forward and outward, which determines contracture in the elbow joint. The axis of the forearm and hand is deviated toward the elbow. Changes in the hand are characterized by a wide variety. Of the pathologies of the hand, the most common are aplasia of one or two, usually ulnar, rays, as well as underdevelopment of the thumb. Other deformations of the segment include syndactyly and hypoplasia.

According to the degree of underdevelopment of the ulna, congenital clubhand is divided into 4 types.

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

The goals and principles of conservative treatment are identical to the goals and principles of treatment of radial clubhand.

Indications for surgical treatment of ulnar congenital clubhand are the impossibility or difficulty of self-care of the abnormal limb due to contracture in the elbow joint (not corrected by conservative methods), due to shortening of the forearm and non-correctable passive ulnar deviation of the hand and, finally, due to limitation of the hand function, primarily its bilateral grip. Surgical intervention begins with the elimination of the most functionally significant deformation. Surgery can be performed as early as the first year of the patient's life.

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