Clubhand after a fracture surgery 21 5. Underdevelopment or complete absence of the radius

Congenital clubhand is a consequence of congenital shortening of the muscle tendons and ligaments of the palmar-radial side, as well as underdevelopment or absence of the radius and ulna. Depending on the side of the deviation - deviation of the hand, patients are divided into two groups with ulnar and radial clubhand. Taking into account the severity of hand deviation and limitation of range of motion in the wrist joint and rotational movements, three degrees of severity are distinguished. Grade I severity includes children with radial or ulnar deviation of the hand up to 160 degrees, limited palmar flexion to 60-70 degrees, dorsal flexion up to 50-60 degrees. Rotational movements in children with I degree clubhandedness are 60-70 degrees. Grade II severity includes patients with radial and ulnar deviation of the hand up to 110 degrees, limitation of palmar flexion to 40-60 degrees, dorsal flexion to 30-50 degrees. Rotational movements are limited to 45-50 degrees. Grade III severity includes children with radial deviation of the hand below 110 degrees, limited palmar and dorsal flexion to 20 degrees, and rotational movements to 10-30 degrees. In patients with congenital radial clubhand due to aplasia and hypoplasia of the radius as a result of anomalies of the musculoskeletal system, the following were identified: Clinical signs(fig.): the affected upper limb is sharply shortened compared to the healthy one and is arched in the forearm area (mainly due to the forearm, ranging from 2 to 9 cm); the hand is deflected to the radial side at an angle from 85 to 109 degrees to the axis of the forearm and is pronated, often the first finger is underdeveloped in the form of a rudiment, hanging on a skin pedicle or absent, the fingers are bent at the interphalangeal joints. The affected arm is atrophic (1-3 cm) compared to the healthy one, this is especially pronounced in the forearm area. Sometimes four fingers are observed. Palpation of the head ulna protrudes in the form of a hemispherical protrusion, covered with calloused skin. The ulna is arched and shortened throughout the entire palpation; in most cases, in place of the radius, the muscles of the radial side are palpated, which are significantly atrophied. In some cases, it is possible to palpate the rudimentary radius bone closer to the elbow joint. Movement in the wrist joint is limited due to dorsal and palmar flexion, within 10-20 degrees. With congenital underdevelopment of the distal end of the radius with a bilateral lesion, underdevelopment of the first finger is noted in the form of a rudiment hanging on a skin pedicle, the side with a more pronounced lesion was shortened by 1 cm compared to the other, the forearm and hand were slightly pronated, movements in the wrist joint were limited account for palmar flexion of the hand by 20 degrees and dorsiflexion by 30 degrees, in elbow joint- not impaired, hand grip function is satisfactory.

In patients with congenital ulnar clubhand, the deformity may be caused by exostosis, Ollier's disease, etc. In such children, soft tissue atrophy and shortening of the forearm from 3 to 5 cm are observed. The function of the elbow and wrist joints is impaired, the forearm is varus curved. Behind the external condyle humerus the dislocated head of the radial bone is palpated, due to which movements in the elbow joint are limited.

Also, in the lower third of the forearm, on the projection of the ulna, growths of dense consistency are detected. Treatment of children with congenital clubhandedness should begin from the first weeks of the child’s life, at the beginning of redressal with staged plaster casts, with maximum use of the entire arsenal of thermal physiotherapeutic procedures. Possibilities conservative treatment when eliminating deviation of the hand in congenital clubhandedness, they are limited due to the extreme severity of the lesion and are considered as a stage of preoperative preparation. Despite various reasons occurrence of both radial and ulnar clubhand, the following principles of surgical treatment of this group of patients can be outlined. The first surgical treatment of all types of clubhand should be as early as possible so that the tendon-muscular system and neurovascular bundle easily lengthened when hand deviation was eliminated. Second: it is necessary to move the hand along the axis of the forearm by eliminating secondary deformation, i.e. to lengthen an underdeveloped hand or in case of total defects, it is necessary to restore the integrity of the “fork” of the wrist joint, as well as eliminate dislocation, subluxation in the joints of the affected limb. Treatment method for congenital radial clubhand. In patients with congenital radial clubhand with absence or subtotal defect of the radial bone, the technique schematically depicted in the figure is used. The technique consists of two stages: the first involves distraction and stretching of soft tissues, eliminating radial deviation of the hand dislocation; on the second - splitting the ulna and creating a “fork” of the elbow joint. After matching the hand with the end of the ulna, without removing the Ilizarov apparatus, an incision is made in the skin and soft tissue along the dorsal-outer lower third of the forearm, 4-5 cm long, exposing the distal third of the ulna in sagittal plane. Then, through each split halves, knitting needles with a thrust platform are passed in a horizontal plane; at the same time, in order to cut through the split half of the ulna bone with a knitting needle in inner surface It is laid with two allobone planks. The spokes are fixed with special rods with a side notch to the beams, connected to the rings of the apparatus. Postoperative wound sutured tightly. 2-3 days after the operation, a “fork” of the elbow joint is created by gradually separating the split halves of the ulna by pulling the knitting needles with thrust pads. The space between the split halves of the ulna is gradually replaced by bone regenerate. Thus, the distal end of the ulna thickens, creating bone support for the hand on the radial and ulnar sides. After consolidation of the regenerate in the area of the created “fork” of the Ilizarov apparatus, a removable gypsum bandage for 1-2 months. At the same time, the forearm and hand are massaged, movements are developed in the interphalangeal, metacarpophalangeal joints, warm baths followed by wearing acetone splints for 6 months.

Radial deviation of the hand is eliminated by advancing the lengthening on the side of the clubhand. After equalizing the length of both bones of the forearm, self-reduction of the dislocation or subluxation of the head of the ulna occurs. 1-1.5 months after X-ray control, the Ilizarov apparatus is removed and a removable plaster splint is applied. Subsequently, patients receive physical therapy and, in parallel, physical therapy, aimed at restoring impaired functions of the wrist joint and fingers.

Insufficient beam length of the radial side, everyone is affected soft fabrics and a skeleton. Most often, violations relate to the forearm, wrist, and hand. Radiation dysplasia is usually combined with hypoplasia of the first finger. EXCEPTION: Thrombocytopenia - absent radius syndrome with normal first digits.

Prevalence

1 in 30,000 to 1 in 100,000 live births
Boys > girls
Right > Left
In half of the cases there is bilateral damage
An anomaly of the radius is present in 50% of patients with hypoplasia of the first digit.

Causes

Sporadic
Syndromic
Teratogenic

Thalidomide (tranquilizer)
Valproic acid
Radiation

Combination diseases

They are common and can be syndromic or non-syndromic

Blood diseases

Fanconi anemia

Autosomal recessive inheritance
Anemia, thrombocytopenia, leukopenia
Congenital anomalies of the heart, kidneys, digestive tract
Lag mental development in 20% of cases
Underdevelopment/absence of the first finger in 80% of cases
Radiation dysplasia in 20% of cases

Thrombocytopenia - absent radius (TAR) syndrome

Autosomal recessive
Thrombocytopenia and leukocytosis
Bilateral radial aplasia, but first digit present
Abnormalities of the heart, digestive tract and skeleton
Normal intelligence in 90% of cases

Heart diseases

Holt-Oram syndrome

Autosomal dominant inheritance
Atrial septal defect in a third of patients
Triphalangeal first finger
Radiation dysplasia
Radioulnar synostosis

Diseases of the maxillofacial area

Cleft lip occurs in 7% of cases of radiation dysplasia.

Spinal diseases

VACTERL (VATER)

V spinal anomalies
And anal atresia
With abnormalities of the cardiovascular system
T tracheoesophageal fistula
R kidney abnormalities
L limb defects

Non-syndromic diseases

Syndactyly

Triphalangeal first finger
Radioulnar synostosis
Sprengel's disease
Scoliosis
Congenital hip dislocation
Clubfoot

Neurological

Hydrocephalus
Deafness

Heartfelt

Patent ductus ductus
Ventricular septal defect

Gastrointestinal

Esophageal-tracheal fistula
Anal atresia

Classification

Vaupe and Klug classification of radial dysplasia

Type 1 Radius shorter than ulna
Type 2 Hypoplasia distal section radius
Type 3 Missing part of the radius
Type 4 Complete absence of the radius

Prevalence

Type 4 (66%)
Type 1
Type 3
Type 2

Symptoms and signs

Bent wrist with supination and radial deviation of the hand.

Skeleton

Insufficiency of the radius from moderate hypoplasia to complete absence, depending on the type.
The fibrous “bud” is a strand of fibrous tissue represented by the mesenchymal remnant of the distal radius, which can extend from the wrist to the proximal forearm, causing progressive deformity.
Shortening of the forearm with the length of the ulna about 60% of normal.
Bent ulna in types 3 and 4.
The humerus may be shortened with a defect in the distal part, which causes a decrease in the range of motion in the elbow joint.
Absence of the wrist joint. The articulation of the ulna with the carpal bones is impaired and the triangular fibrocartilaginous complex is absent.
Hypoplasia of the wrist, with the absence or underdevelopment of the trapezius and scaphoid bones.
Hypoplasia or absence of the first finger.
Abnormal stiffness of the three-phalangeal fingers with improvement from the radial to the ulnar direction.

Muscles

Muscle abnormalities depend on the degree of underdevelopment of the radius

Absent or underdeveloped muscles:

Forearm: pronator teres, brachioradialis
External flexors: flexor carpi radialis, flexor pollicis longus
External extensors: extensor carpi radialis longus and brevis, extensor of the little finger, extensor of the second finger
Extrinsic muscles of the thumb: abductor pollicis longus, extensor pollicis longus and brevis
Eminence muscles thumb: abductor pollicis brevis, flexor pollicis brevis, opponensus

The flexor digitorum superficialis often fuses with the flexor digitorum profundus. Possible absence of the deep flexor of the second finger. Flexor carpi ulnaris and ulnar nerve, innervating the intrinsic muscles of the hand, are usually present.

Nerves

The ulnar nerve is usually normal
The musculocutaneous nerve is usually absent, median nerve innervates the muscles of the anterior shoulder sheath
The radial nerve usually ends at the level of the elbow joint
The median nerve usually provides sensation to the radial side of the hand and forearm.

Vessels

The brachial artery is usually highly divided
Ulnar artery is usually present
The radial artery is usually absent.

Treatment

Consultation with a pediatrician to assess the condition for concomitant pathology.

There are three treatment methods, the choice depends on the patient and the nature of the deformity

Do not treat

List of contraindications for surgical treatment see below.
In type 1, improvement is achieved in most cases by stretching and splinting.

Teaching parents about stretching and splinting

Type 1 and 2
Treatment should begin in the neonatal period.

Operation

Wrist correction for severe types 2, 3 and 4.
Distraction lengthening of the forearm in some cases of type 2, and types 3 and 4.
If necessary, osteotomy of the ulna, for types 3 and 4.

Contraindications for surgery

Moderate anomaly
Heavy combined systemic diseases with a poor prognosis
Untreated adults (one who has grown and adapted to the deformity)
Loss of elbow flexion:

Straightened hand does not reach mouth or perineum
For bilateral lesions with stiffness in the elbow joint, correction of only one hand is performed

Severe soft tissue contracture, with limited possibilities of correction by nerves or blood vessels.

Principles of surgical treatment

Correction of deformity
Reduction of radial and volar wrist displacement
Wrist stabilization
Move the hand to the ulna while maintaining wrist movements

Increasing forearm length

Avoid additional damage to maintain growth potential (already reduced).

Improved hand function

Improving grasping of objects, fist grip and pinch grip.

If necessary, pollicization is performed only after wrist correction.

External pitchfork

With all the meaning appearance it should not be considered more important than function, especially in untreated adults.

Splinting

When performing all operations, splinting is necessary postoperative period.

Operation execution time

Early

Up to 6-9 months

Wrist correction for types 3 and 4
Preoperative distraction
Centralization/radialization.

9-18 months

Pollicization or other method of reconstruction of the first digit

Late

If necessary, revision of wrist correction
Distraction lengthening of the ulna
Osteotomy of the ulna
Transposition of tendons to restore the opposition of the first finger.

Surgical interventions

Preoperative distraction

Correction is possible in cases of failure of stretching and splinting. It may be necessary to mobilize the bud when applying the device. Possibility of wrist correction without tension. Additional lengthening reduces pressure on the ulnar epiphysis (with less growth disturbance) in severe cases. Reduces bone removal requirements for wrist correction.

Centralization

Resection of the central carpal bones to create a socket for the ulna
Removal of cartilage from the distal end of the ulna while preserving the growth plate
Placement of the ulna in the bed and fixation with a rod
Restoring the balance of the ulnar flexor and extensor carpi muscles
Impairs wrist growth.

Realization

Complete passive wrist correction is required before surgery

Sometimes preoperative distraction is required.

Carpal bones are not resected
The second metacarpal bone is positioned along the same axis with ulna, moving the wrist to the radial side with fixation with a rod.
If necessary, the balance of the tendons is restored.
Limb length and wrist range of motion can be maintained

There is less injury to the wrist and the growth area of the ulna.

Distraction ulnar lengthening can be used to correct ulnar length and restore wrist alignment.

Osteotomy of the ulna, performed simultaneously with wrist correction or later. The distraction lengthening technique can also be effective for correcting ulnar curvature.

Elbowcarpal arthrosis is performed at complete or almost complete skeletal maturity in untreated patients. In case of recurrence of deformity after a previously performed operation.

Complications

Early

Rod infection/migration
Fixator fracture

Late

Recurrence of radial deviation of the wrist
Reduced growth of the wrist and forearm
Stiffness in the wrist.

27.12.2014

Congenital clubhand is a persistent deformity, as a result of which the hand deviates to the ulnar or radial side from the longitudinal axis of the forearm. Occurs in the prenatal period. It is a consequence of underdevelopment of one of the bones of the forearm or the tendons associated with these bones. Manifested by curvature upper limb– the hand is located at an angle to the forearm.

Persistent deformation, as a result of which the hand deviates to the ulnar or radial side from the longitudinal axis of the forearm. Occurs in the prenatal period. It is a consequence of underdevelopment of one of the bones of the forearm or the tendons associated with these bones. It manifests itself as a curvature of the upper limb - the hand is located at an angle to the forearm. It can be combined with underdevelopment of the metacarpal bones, phalanges of the fingers, the absence of one or more fingers, fusion of the fingers, contractures, subluxations or dislocations in the elbow and wrist joints.

Congenital clubhand is an anomaly in which the hand is located at an angle to the forearm. This type limb deformities. It occurs due to underdevelopment of the bones or tendons of the forearm during the prenatal period. It is a rare pathology; according to the literature, radial clubhand is detected in one out of 55 thousand newborns, ulnar clubhand in one out of 220-550 thousand newborns. Can be one-sided or two-sided. Often combined with others birth defects development. Recommended surgery V early age.

Causes of development of congenital clubhandedness

Congenital clubhand is formed as a result of exposure to a number of external and internal factors. External (exogenous) factors that can cause the development of this pathology include maternal malnutrition, infectious diseases, taking certain medications, and ionizing radiation. Among the internal (endogenous) reasons are: late pregnancy, hormonal disorders, severe somatic diseases of the mother, functional disorders And pathological changes uterus The exposure time is of great importance - critical period The first 5 weeks of pregnancy are considered. Genetic predisposition not identified.

Classification of congenital clubhandedness

There are two types of congenital clubhand: radial and ulnar. Radial clubhand is formed with underdevelopment or absence of the radius bone and associated tendons, ulnar clubhand - with underdevelopment or absence of the ulna bone and the corresponding tendons. Each type of congenital clubhand is divided into several subtypes, taking into account the degree of bone underdevelopment.

Ulnar clubhand:

Moderate hypoplasia. The ulna is shortened by 10-29% compared to the radius.
Severe hypoplasia - the ulna is shortened by 30-69% compared to the radius.
Rudiment of the ulna - the ulna is shortened by 70-99% in comparison with the radius.
Aplasia of the ulna - the ulna is completely absent.

Radial clubhand:

Grade 1 – the radius is shortened by no more than 50% of normal.
Grade 2 – the radius is shortened by more than 50% of normal.
Grade 3 – the radius is completely absent.

In addition, with radial clubhand, orthopedic experts distinguish 4 types of hands:

Type 1 – hypoplasia of the first metacarpal bone and thenar muscles (the elevation located between the first finger and the wrist joint).
Type 2 – the first metacarpal bone is absent, underdevelopment of the phalanges of the 1st finger is revealed.
Type 3 – the first metacarpal bone and the first finger are absent; the absence of fingers II and III may also be observed.
Type 4 – the bones of the hand are developed normally.

Symptoms of congenital clubhandedness

With radial clubhand, the hand is in a pronated position; an angle is formed between the hand and forearm, open towards the radial surface (thumb side). The dorsal surface reveals a proximal displacement of the hand relative to the head of the ulna. The brush rotates around the head like a weather vane. Most patients with congenital clubhand have various hand defects: absence of the first finger, absence of the first metacarpal bone, absence of the first, second and third fingers. The first finger may be shortened, consist of one phalanx, or, due to the absence of the first metacarpal bone, hang on a skin pedicle. The fifth and fourth fingers are developed normally. Due to underdevelopment and contractures of the fingers, the functionality of the hand is sharply limited, and grasping objects is often impossible.

The forearm is usually shortened, the head of the ulna protrudes. Due to the deformation of the ulna, an arched curvature of the forearm occurs with a convexity facing the ulnar side (the side of the V finger). The forearm muscles are underdeveloped. The pronators and supinators of the forearm, the long muscles of the first finger and the brachioradialis muscle are often absent. The long head of the biceps brachii may also be absent. With aplasia of the radius, the absence of the radial artery is also observed.

With ulnar clubhand, the hand is in a supinated position; an angle is formed between the hand and forearm, open to the ulnar side (the side of the fifth finger). The fifth and fourth fingers are often absent or underdeveloped. The forearm is shortened. Due to the deformation of the radial bone, an arched curvature is formed, convexly facing the radial side (side of the first finger). Movement in the elbow joint is limited due to dislocation of the radial head. The muscles of the forearm on the ulnar side are underdeveloped or absent. The hand function is less impaired than in radial clubhand.

Diagnosis of congenital clubhandedness

Diagnosing congenital clubhand is not difficult due to visually visible deformations and obvious dysfunction of the limb. To accurately assess the degree of underdevelopment of bone structures and clarify the further treatment plan, x-ray of the forearm bones and x-ray of the hand bones. To assess the condition of soft tissues, it is prescribed MRI of the forearm.

Treatment of congenital clubhand

Treatment of congenital clubhandedness begins from the first days of life. Infants under 6 months of age are prescribed conservative therapy, aimed at “stretching” soft tissues, reducing and preventing the development of contractures. Patients are referred for massage and exercise therapy and wearing orthoses. Operations are recommended to be performed before the age of 1 year, the optimal period is 6-9 months. Indications for surgical intervention are unremovable contractures in the wrist and elbow joints, uncorrectable deviation of the hand in relation to the forearm and limitations in hand function. Depending on the degree and type of deformation, intervention can be either one-stage or multi-stage. At the first stage, the most functionally significant deformation is eliminated; at subsequent stages, minor deformities and, if possible, cosmetic defects are corrected.

In case of radial clubhand, surgical interventions are performed to restore the “fork” in the area of the wrist joint. For severe contractures, bone intervention is performed in combination with lengthening of the wrist flexors. With aplasia of the radius, radialization or centralization of the hand is carried out. Within 1-2 months. the brush is brought into correct position, using a distraction device (Ilizarov apparatus), and then stabilize it on the ulna. A pronounced arched curvature of the forearm is an indication for corrective osteotomy.

For ulnar clubhand, excision of the fibrous cord connecting the underdeveloped ulna to the wrist joint is performed in combination with corrective osteotomy of the radius. If the length of the ulna is more than 50% of normal, the bone is lengthened using the Ilizarov apparatus. Sometimes the intervention has to be carried out in two stages: at the first stage, the bone is brought into the correct position and the dislocation of the radial head is eliminated, at the second stage a single-bone forearm is formed.

Finger fusion with ulnar and radial clubhand is usually eliminated at the final stage, after correction of more significant deformities. Toes are transplanted from the foot to replace missing toes. In the postoperative period, exercise therapy, massage and exercise therapy are required to prevent contractures and secondary deformities.

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Start of activity (date): 12/27/2014 23:01:00
Created by (ID): 645
Keywords: deformation, hand, in utero

– this is a stable deformation, as a result of which the hand deviates to the ulnar or radial side from the longitudinal axis of the forearm. Occurs in the prenatal period. It is a consequence of underdevelopment of one of the bones of the forearm or the tendons associated with these bones. It manifests itself as a curvature of the upper limb - the hand is located at an angle to the forearm. May be combined with other congenital defects of the limb. The diagnosis is made based on X-ray picture and inspection data. Treatment is surgical, carried out at an early age (usually before 1 year). After the operation, exercise therapy, massage and physiotherapy are prescribed.

ICD-10

Q71.4 Longitudinal shortening of the radius

General information

Congenital clubhand is an anomaly in which the hand is located at an angle to the forearm. This type of limb deformity occurs due to underdevelopment of the bones and/or tendons of the forearm during the prenatal period. It is a rare pathology; according to the literature, radial clubhand is detected in one out of 55 thousand newborns, ulnar clubhand in one out of 220-550 thousand newborns. Can be one-sided or two-sided. Often combined with other congenital malformations. Treatment is carried out by pediatric orthopedists. Surgery at an early age is recommended.

Causes

Congenital clubhand is formed as a result of exposure to a number of external and internal factors. External (exogenous) factors that can cause the development of this pathology include maternal malnutrition, infectious diseases, taking certain medications, and ionizing radiation. Internal (endogenous) causes include late pregnancy, hormonal disorders, severe somatic diseases of the mother, functional disorders and pathological changes in the uterus. The time of exposure is of great importance - the first 5 weeks of pregnancy are considered the critical period. No genetic predisposition has been identified.

Classification

Ulnar clubhand:

Moderate hypoplasia. The ulna is shortened by 10-29% compared to the radius.
Severe hypoplasia - the ulna is shortened by 30-69% compared to the radius.
Rudiment of the ulna - the ulna is shortened by 70-99% in comparison with the radius.
Aplasia of the ulna - the ulna is completely absent.

Radial clubhand:

Grade 1 – the radius is shortened by no more than 50% of normal.
Grade 2 – the radius is shortened by more than 50% of normal.
Grade 3 – the radius is completely absent.

In addition, with radial clubhand, specialists in the field of orthopedics and traumatology distinguish 4 types of hands:

Type 1 – hypoplasia of the first metacarpal bone and thenar muscles (the elevation located between the first finger and the wrist joint).
Type 2 – the first metacarpal bone is absent, underdevelopment of the phalanges of the 1st finger is revealed.
Type 3 – the first metacarpal bone and the first finger are absent; the absence of fingers II and III may also be observed.
Type 4 – the bones of the hand are developed normally.

Symptoms of congenital clubhandedness

Most patients with congenital clubhand have various hand defects: absence of the first finger, absence of the first metacarpal bone, absence of the first, second and third fingers. The first finger may be shortened, consist of one phalanx, or, due to the absence of the first metacarpal bone, hang on a skin pedicle. The fifth and fourth fingers are developed normally. Due to underdevelopment and contractures of the fingers, the functionality of the hand is sharply limited, and grasping objects is often impossible.

Diagnostics

Diagnosing congenital clubhand is not difficult due to visually visible deformations and obvious dysfunction of the limb. To accurately assess the degree of underdevelopment of bone structures and clarify the further treatment plan, radiography of the bones of the forearm and radiography of the bones of the hand are performed. To assess the condition of soft tissues, MRI of the forearm and electromyography are prescribed.

Treatment of congenital clubhand

Treatment begins from the first days of life. Infants under 6 months of age are prescribed conservative therapy aimed at “stretching” soft tissues, reducing and preventing the development of contractures. Patients are referred for exercise therapy, massage and wearing orthoses. Operations are recommended to be performed before the age of 1 year, the optimal period is 6-9 months.

Indications for surgical intervention are unremovable contractures in the wrist and elbow joints, uncorrectable deviation of the hand in relation to the forearm and limitations in hand function. Depending on the degree and type of deformation, intervention can be either one-stage or multi-stage. At the first stage, the most functionally significant deformation is eliminated; at subsequent stages, minor deformities and, if possible, cosmetic defects are corrected.

In case of radial clubhand, surgical interventions are performed to restore the “fork” in the area of the wrist joint. For severe contractures, bone intervention is performed in combination with wrist flexor lengthening and skin grafting. With aplasia of the radius, radialization or centralization of the hand is carried out. Within 1-2 months. the hand is brought into the correct position using a distraction device (Ilizarov apparatus), and then stabilized on the ulna. A pronounced arched curvature of the forearm is an indication for corrective osteotomy.

Syndactyly (fusion of fingers) with ulnar and radial clubhand is usually eliminated at the final stage, after correction of more significant deformities. Toes are transplanted from the foot to replace missing toes. In the postoperative period, physiotherapy, massage and exercise therapy are required to prevent contractures and secondary deformities.

Congenital clubhand is a combined defect caused by underdevelopment of tissue on the radial or ulnar side of the upper limb. If the hand deviates to the radial side, a diagnosis of radial clubhand is made. (tanus valga), when deviated in the opposite direction - ulnar congenital clubhand (manus vara).

ICD-10 code

Q71.4 Radial congenital clubhand.
Q71.5 Ulnar congenital clubhand.

What causes congenital clubhand?

Congenital clubhand, according to world literature, is registered in 1 out of 1,400-100,000 children. Radial congenital clubhand is more often diagnosed. The ulnar one is observed 7 times less often than the radial one.

Congenital clubhand occurs due to the influence of external and internal factors, which are also characteristic of other congenital anomalies development of the upper limb. External, exogenous factors include ionizing radiation, mechanical and mental trauma, medications, contacts with infectious diseases, lack of nutrition, etc. Endogenous causes - various pathological changes and functional disorders of the uterus in a pregnant woman, general diseases mothers, hormonal disorders, aging of the body. Wherein great importance have a duration of exposure, 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 joint); underdevelopment of the bones of the forearm (primarily the radius); abnormal development of fingers and hand.

Other lesions of the hand 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 of the hand. The bones of the wrist located on the radial side also suffer, and aplasia or concrescence with other bones is observed.

Classification

In the classification of radial clubhand, there are three degrees of underdevelopment of the radial bone and four types of the hand. The basis for classification is the x-ray picture.

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 radius bone.

Types of brushes

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

In type 1, hypoplasia of the first metacarpal bone and muscles is detected thenar, type 2 is characterized by the complete absence of the metacarpal bone and hypoplasia of the phalanges of the first finger (in this case, a “dangling finger” is usually 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 a child’s life) includes exercise therapy, massage, retraining exercises to reduce existing contractures of the fingers and hand, and provision of orthotic products. However, conservative measures do not provide lasting positive result and they should be considered as preliminary preparation for the second stage - surgical. Surgical treatment is recommended to begin at six months of age.

Surgery

The choice of surgical method depends on the type of deformity.

The lower the degree and the younger child, the easier it is to get the brush out of deviation. Therefore, it is recommended to start surgery up to 2-3 years of age.

Ulnar congenital clubhand is characterized by deformation and shortening of the forearm, ulnar deviation of the hand, and limited movement in the elbow joint. The underdevelopment of the ulna, especially its distal part, is more pronounced. In this area there is usually a fibrocartilaginous cord connecting the ulna to the carpal bones. The radius is arched. Its head is most often dislocated in the elbow joint anteriorly and outward, which determines contracture in the elbow joint. The axis of the forearm and hand is deviated to the ulnar side. Changes in the hand are characterized by great diversity. Among the pathologies of the hand, the most commonly observed 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.

Based on 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 rudiment of the 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.

The indication for surgical treatment of ulnar congenital clubhand is the impossibility or difficulty of self-care with the abnormal limb due to contracture in the elbow joint (not eliminated conservative methods), due to shortening of the forearm and passively non-correctable ulnar deviation of the hand and, finally, due to limitation of the function of the hand, primarily its bilateral grip. Surgical intervention begin with the elimination of the most functionally significant deformity. The operation can be performed from the first year of the patient’s life.

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

It is important to know!

Clubhand is a severe deformity, the main symptom of which is a persistent deviation of the hand towards the missing or underdeveloped bone of the forearm: in the absence of the radius - radial clubhand (manus vara), in the absence of the ulnar - ulnar clubhand (manus valga).