Orthopedics - Oleksa A.P. 2006

Pathophysiology of Joints
Skeletal Malformations and Congenital Anomalies
Arthrogryposis

Arthrogryposis multiplex congenita is a disease name introduced in 1923 by Walter and established by Stern (Stern N.G., 1923), although in literature and clinical practice the term "arthrogryposis" is most commonly used. The name originates from two Greek words: arthron, meaning joint, and grypos, meaning crooked. The condition was first described by Rosenkrantz as early as 1905 (Fig. 71).

Because arthrogryposis is rarely encountered in clinical practice, publications by numerous authors are based either on a small number of personal observations or on compiled data from various sources. This has led to differing interpretations of etiopathogenesis and various classifications of arthrogryposis.

Depending on their understanding of The Essence of this pathology, various authors even proposed their own names for the disorder: "congenital muscular aplasia" (Sheldon W., 1932); "congenital muscular dystrophy" (Middleton D.S., 1932); "multiple congenital articular rigidity" (Rocher H.L., 1954); "congenital myo-articular Dysplasia" (Rossi E., 1951); "congenital deforming myomatosis" (Kutsenok B.S. and Rabinovych O.O., 1936); "congenital arthrodyskinesia" (Bogdanov F.R., 1934). All this indicates a lack of consensus regarding the causes of the disease, the harmful Factors affecting the fetus, and the exact moment when normal fetal development begins to be disrupted. These issues have been addressed in the works of numerous researchers who have put forward A number of theories and hypotheses concerning The Nature and essence of arthrogryposis.

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Fig. 71. Arthrogryposis in a 6-year-old girl (Sturm V.A., 1968).

The mechanical theory was put forward at the beginning of the century, when it was believed that embryonic development was disrupted under METABOLISM/18.html">The Influence of various mechanical factors, primarily resulting from increased amniotic pressure due to a decreased volume of Amniotic Fluid (oligohydramnion). In such cases, the reduced volume of the Uterus tightly envelops the embryo and restricts its movements. A similar elevated pressure occurs when there is an increased amount of amniotic fluid (Hohman, 1901). However, restriction of fetal movement and the presence of extension contractures in joints are by no means compatible in arthrogryposis. Therefore, Rocher (1954) believed that increased pressure could impair the Blood supply to the fetal limbs. He also attached significance to various physical traumas.

The oxygen starvation theory was supported by Steindler A. (1949) and Sturm V.A. (1964), who asserted that this is the cause of Muscle atrophy, fatty degeneration, and fibrous degeneration of Muscles.

Badgley C.E. (1943) also believed that muscles are affected As a result of Impaired blood supply and histologically found thinned Blood Vessels in them, but these findings were not confirmed by other researchers.

The infectious theory in the Etiology of arthrogryposis has a number of proponents who suggest the possibility of an amniotic infection at the 4th to 5th week of fetal development, which penetrates the Central Nervous system. Drachman D.B. (1961) points out that maternal viral diseases (Influenza, rubella) in the first three months of Pregnancy cause Congenital Malformations in 35-100% of cases. The Role of rubella in the occurrence of congenital malformations is noted by many authors (James T., 1951; Mattner H.R., 1957; Nikiforova T.K., 1964, and others).

Stern N.G. (1923) associated arthrogryposis with intrauterine periarthritis. Nikiforova pointed out that a number of paternal diseases even before Fertilization can cause zygote inferiority, and maternal illness adversely affects egg implantation and the differentiation of cellular structures. She notes that 31% of the mothers of these children had some acute illness during pregnancy.

There is now sufficient experimental material confirming The impact of various teratogenic factors on embryonic development and THE SPECTRUM OF musculoskeletal anomalies. The earlier the harmful factor acts on the fetus, the more severe the developmental malformations tend to be.

The Theory of hereditary transmission of arthrogryposis is basically rejected, although such facts have been described by scientists. For instance, Mastella E. and Saccomany F. (1960) found 6 cases in the literature, in each of which two brothers and two sisters suffered from arthrogryposis. Similar Examples of hereditary disease transmission were described by Frischknecht, Blanchi, and Pillari (1960), when three triplet brothers were born with manifestations of arthrogryposis.

However, There is a contradiction in this theory. Hillman J.W. and Johnson J.T.H. (1952) presented two pairs of monozygotic twins, in each of which only one twin had arthrogryposis.

Rocher H.L. (1954) hypothesized that arthrogryposis is based on genodystrophy, that is, alterations in one or more genes responsible for limb development.

Summarizing the above, it can be concluded that the etiology of arthrogryposis is diverse, but The most significant role is played by the detrimental impact of teratogenic factors on the embryo, which disrupt its normal development.

Regarding the Pathogenesis of arthrogryposis, There are also a number of theories based mainly on histological studies.

The oldest was the arthrogenic theory, supported by the authors who first described arthrogryposis. Rocher H.L. (1954) convincingly argued that under the influence of infection and other intrauterine harmful factors, The Development of the ligament-capsular apparatus of the joints is delayed or halted, resulting in impaired joint function. The lack of movement in the joints causes muscle atrophy and secondary degeneration. He points out that arthrogryposis is caused by hypoplasia and mesodermal dysplasia of the entire Musculoskeletal System.

Pedrocca A. and Pavatto G.C. (1963) describe arthrogryposis as a developmental defect of the embryo with primary Changes in the joints and as a multiple congenital joint dysplasia.

As research material accumulated, the arthrogenic theory ceased to satisfy the majority of researchers, who believed that changes in the muscles are the primary cause of arthrogryposis.

As early as 1908, Howard R. performed a pathoanatomical examination of the body of a deceased boy with arthrogryposis who lived for 7 days. He revealed muscular dystrophy as well as significant degenerative changes in muscle fibers in the form of fatty and fibrous replacement of Muscle tissue. The joints remained fixed in abnormal positions even after muscle dissection, and movement in them appeared only after all ligaments and the Joint Capsule were incised. He believed that muscle lesions are caused by dysplasia, degeneration, and dystrophy of muscle tissue.

Sheldon W. (1932) considered arthrogryposis to be congenital muscular aplasia, while Middleton D.S. (1932) viewed it as a dystrophic process in muscles occurring in utero.

The myogenic theory of pathogenesis was supported by Kutsenok B.S. and Rabinovych O.O. (1936); Steindler A. (1949); Hillman J.W. (1952), and others. Bogdanov F.R. (1934) explained joint rigidity as "waxy flexibility," and their unusual stiffness as a combination of arthrogenic and myogenic factors. Banker B.Q., Victor M., and Adams R.D. (1957) anatomically and histologically examined muscles, the Brain, and the Spinal Cord at all levels in two brothers with multiple deformities of the upper and lower limbs and kyphoscoliosis. They found no pathological Changes in the central nervous system, whereas the muscles, especially of the lower limbs, showed gross pathological changes. The number of muscle fibers was reduced; they were thinner, exhibiting fibrous degeneration of fascicles that had lost their classical Structure. These fibers were enveloped in fibrous and adipose tissue. The authors believed that arthrogryposis results from acute degeneration and atrophy of formed muscle fibers rather than a delay or arrest in their development.

However, with the increasing number of pathoanatomical studies, the neurogenic theory of the pathogenesis of arthrogryposis began to prevail.

Price D.S. as early as 1932 was the first to histologically examine the BRAIN AND SPINAL cord in arthrogryposis. She discovered degeneratively altered Cells in the anterior horns of the spinal cord, along with blurred contours of nerve roots and fibers. Finding typical changes in the muscles, she concluded that in arthrogryposis, primary changes occur in the spinal cord, while muscle alterations are secondary. A reduction in the volume of the anterior horns of the spinal cord was observed by Scarella M. (1932), and Brandt even reported the absence of motor cells in the anterior horns accompanied by vacuolar degeneration and empty spaces in their place. Similar changes were described by Gilmour. They associated degenerative changes in muscles with Pathology of the anterior horns of the spinal cord.

The neurogenic theory of the pathogenesis of arthrogryposis is confirmed by pathomorphological studies of the spinal cord and brain conducted by Kanof A. (1956); Fowler W. (1959); and Drachman D.B. and Banker B. (1961). They believe that changes in muscles occur in the manner of denervation atrophy, which leads to a significant thinning of muscle fibers with the disappearance of their cross-striation and excessive proliferation of connective and intrafascial Tissues. According to Bogdanov F.R., these pathological intrauterine changes in the fetus result in congenital arthrodyskinesia.

To sum up the pathogenesis of arthrogryposis, while certain aspects remain unclear, it can be concluded that the condition may stem from anterior horn motor neuron lesions of the spinal cord in some cases, and from primary myopathy in others. However, both pathways can be triggered by embryotoxic factors, most notably teratogens.

Clinical presentation and Diagnosis.

The clinical manifestations of arthrogryposis are extremely diverse, characterized by congenital multiple rigid joint contractures and deformities combined with muscle hypoplasia. As a rule, the joints and Muscles of the upper and lower extremities are affected, whereas the back or trunk muscles are exceptionally rarely involved. In clinical practice, upper and lower limbs are most commonly affected (75% of cases), less frequently only the lower or upper limbs, and occasionally only isolated segments thereof.

We performed surgery on a 19-year-old student presenting with an affected right elbow (extensor contracture) and, to a lesser extent, the wrist joint (flexor contracture), with a normal shoulder joint.

Thus, arthrogryposis exhibits polymorphism in both the number and severity of joint lesions, as well as the degree of muscle aplasia. This has prompted clinicians to classify arthrogryposis based on these clinical manifestations.

Several classifications have been proposed. Rocher was among the first to divide patients into three groups based on the localization and pattern of contractures (Rocher H.L., 1954). The first group included patients with involvement of the upper and lower extremities fixed in extension, except for the hip joints. The second group comprised similar patients, but with knee joints fixed in flexion and hips in abduction and lateral rotation. The third group consisted of patients with extension contractures of the elbows and flexion contractures of the knees.

In the former USSR, two classifications of arthrogryposis were proposed in 1936. R. R. Vreden distinguished between typical and atypical forms of arthrogryposis, whereas Kutsenok and Rabinovych divided patients into four groups:

1. With contractures of the upper extremities only.

2. With contractures of the upper and lower extremities.

3. With contractures of the lower extremities.

4. With contractures of all extremities and the spine.

In 1949, Steindler divided patients into three groups:

1. With extension contractures manifesting as quadriplegia, paraplegia, or monoplegia.

2. With flexion contractures manifesting as quadriplegia or paraplegia.

3. Mixed forms.

According to the Classification by L. E. Rozovska and Ter-Yegiazarov (1973), patients are also categorized into three groups: severe, moderate, and mild. Severe cases involve total damage to all limb joints with pronounced muscle aplasia. The moderate group includes involvement of all limb joints except the shoulders and hips, along with corresponding muscle aplasia. The mild group comprises children with lesions restricted to the distal segments of the extremities.

Diagnosing arthrogryposis is straightforward; upon initial examination, the characteristic limb positioning with joint deformities and muscle hypoplasia is readily apparent.

The upper extremities hang limply, pressed against the trunk, medially rotated, and slender; the elbows are extended and rigid; the hands are fixed in flexion and deviated toward the elbows. Due to the Aplasia of the deltoid muscles, the acromion and clavicles appear prominent and shortened, while the scapulae are smaller and elevated. Aplasia and contracture of the pectoralis major muscle prevent arm abduction; passive abduction reveals taut Skin bridging the axillary fossa, described in the literature as "pterygium syndrome" or "pterygium axillaris". Depending on the degree of muscle aplasia, muscle contours are absent, and the arm is slender. In most patients, the forearms are fixed at an extension angle of 155–160° and in pronation. Flexor contractures occur occasionally. The range of motion in the elbow varies, depending on the severity of muscle aplasia and arthrogenic changes. During passive flexion of the forearm, the unyielding triceps muscle becomes sharply taut. The hand also exhibits characteristic alterations and positioning. Typically, it is flexed at the wrist with ulnar deviation and restricted mobility. Active wrist extension likewise depends on the extent of muscle aplasia. The fingers are clenched and inclined toward the palm. Their prehensile function is impaired due to underdeveloped finger flexors. Only in mild cases can a minimal range of motion facilitate functional adaptation in children.

Pathological changes in the lower extremities also vary. As a rule, the limbs present classic deformities with marked muscle hypoplasia. Particularly rigid contractures result from the aplasia of muscles that extend the hip, leg, and FOOT, though this also depends on the degree of muscular hypoplasia.

When the hip joint region is affected, the thigh typically assumes a flexion-abduction contracture. This thigh posture is frequently combined with knee flexion contractures and equinovarus foot deformity.

The severity of contractures depends on the degree of limb muscle hypoplasia. In severe cases, subluxations and even dislocations may occur in both the Hip and knee joints.

Extension contractures of the knee joints are most common. When attempting to flex or extend the leg, one experiences what F. R. Bogdanov termed "waxy flexibility," which is more pronounced toward extension due to the underdevelopment of the knee ligamentous apparatus.

In arthrogryposis of the lower extremities, foot deformity is invariably present and manifests in various forms. Equinovarus deformities are the most common, whereas calcaneal and flat-valgus deformities are rare.

T. S. Zatsepin considered equinovarus foot deformity in arthrogryposis to be an atypical form of Clubfoot, yet it differs from congenital clubfoot by its pronounced rigidity and resistance.

Treatment. Treating patients with arthrogryposis is a challenging problem due to multiple deformities, Skeletal Muscle aplasia of the extremities, and the involvement of adjacent tissues.

When examining a child, it is crucial to assess the severity of the condition—specifically, the number of joints involved, the degree of muscle aplasia, and long-term functional Prospects.

Treatment should begin within the first few weeks of life while the tissues are most pliable, keeping in mind the tendency of contractures to become increasingly rigid as the child grows.

The goal of treatment is to eliminate or minimize contractures as much as possible while simultaneously strengthening underdeveloped muscles across all affected limbs, enabling the patient to be mobile, self-sufficient, and capable of working. This treatment is complex and protracted, sometimes spanning many years, which can be burdensome for parents and often leads them to lose hope and discontinue therapy prematurely. It is advisable to treat such children in specialized sanatoriums and, later, in boarding schools dedicated to children with musculoskeletal disorders.

Therapy should follow proven Conservative Methods starting in early infancy. In young children, because tissues are elastic and pliable, it is easier to correct contractures and strengthen overstretched, underdeveloped muscles.

Parents must be educated about The Nature of the condition and the necessity of long-term follow-up with an orthopedist, massage therapists, and remedial gymnastics (PT) specialists.

Contractures are gradually and progressively corrected as much as possible, and the limbs are maintained in the corrected position using removable plaster splints. At the same time, muscle massage, corrective physical therapy, and thermal Procedures are administered. Paraffin-ozokerite Applications to the limbs and other warming modalities are the most accessible. Electrical stimulation of weakened muscles is essential to improve their function.

Conservative treatment is administered continuously throughout the child's first decade of life. When pursued regularly, it yields significant improvement in limb function and, in mild cases of arthrogryposis, excellent results.

Using conservative methods to achieve a functionally advantageous position of the limb segments is vital, employing serial casting, the Volkov-Oganesyan distraction apparatus, or even the Ilizarov apparatus in adolescents. However, maintaining the limb segments in a corrected position is only possible if the Functions of underdeveloped muscles are improved, which is a more challenging task. At the Turner Institute, muscle strength in arthrogryposis was graded on the five-point scale used to evaluate the sequelae of poliomyelitis. This provides a reasonably objective way to assess dynamic changes in muscle strength during the course of treatment.

Attention must be paid to strengthening the underdeveloped muscles of the thigh and leg, the shoulder abductors, and, in particular, the forearm flexors and hand muscles. Mild equinovarus foot deformity can sometimes be resolved with early conservative treatment, though the feet must be supported in the corrected position at night with splints and during the day with orthopedic footwear. In severe forms of arthrogryposis, conservative treatment is not always effective, yet it should be applied persistently for three to four years to prevent fixed rigid contractures before deciding on the necessity and feasibility of surgical intervention.

In cases of deltoid muscle aplasia, shoulder arthrodesis has been performed by N.D. Kazantseva (1953), H.L. Rocher (1954), M. Green (1964), and L.E. Rozovskaya and Ter-Yegiazarov (1973). The indication for surgery was restricted shoulder abduction in severe arthrogryposis, provided that the Functions of the trapezius, rhomboid, and anterior scalene muscles were preserved. In such patients, the deltoid muscle typically presented as a dense aponeurosis devoid of muscle fibers, and the joint capsule was thickened and nearly motionless, pressing the humeral HEAD against the scapula. The articular Cartilage retained its normal appearance. It was excised, and with the shoulder abducted to 70° and flexed to 30°, the adapted surfaces were fixed while simultaneously correcting internal rotation.

In similar cases, Steindler and Rocher limited their intervention to a derotational osteotomy of the humerus at the level of the deltoid insertion.

Surgical procedures on the elbow joint are performed most frequently. When conservative treatment fails in children older than 4 years, arthrolysis is performed. The surgery is indicated when there is a sharp extension contracture of the forearm (130°–140°) and the child cannot reach their face. Surgical outcomes depend on the condition of the biceps brachii muscle. Postoperative conservative efforts to strengthen this muscle further improve limb function.

Elbow arthrolysis has been described by F.R. Bogdanov (1934), A. Steindler (1949), V.A. Sturm (1965), and others.

We operated on a 19-year-old young man presenting with an arthrogrypotic extension contracture of the right elbow and a flexion contracture of the wrist, with well-adapted function. The active range of motion in the elbow was within 135°–155°. Flexion was blocked by an accessory lateral process of the humerus, which was clinically and radiographically identifiable anteriorly in the olecranon fossa. This can be explained by growth-related tension from pathologically altered muscles, causing it to rotate and form an angle of nearly 90° relative to the medial epicondyle. Concurrently, at the proximal end of the radius, the head was replaced by a thin (5–10 mm) plate, 15–20 mm wide, covered with cartilage-like tissue that abutted the posterolateral surface of the rotated lateral humeral process. The forearm was pronated, and supination movements were absent.

An osteotomy and partial resection of the displaced process were performed, successfully rotating it and clearing the olecranon fossa. Following capsulotomy, arthrotomy, and resection of the proximal end of the radius, intraoperative manipulation achieved elbow flexion to 45° and extension to 160°. The arm was immobilized with a plaster splint in flexion.

Subsequent treatment aimed to strengthen the biceps muscle, because for six months postoperatively, passive forearm flexion remained limited to 60°, and active flexion increased by only 20° compared to preoperative levels—an outcome that satisfied neither the patient nor us. It was recommended to continue mobilizing passive joint motion and, above all, to aggressively strengthen the biceps brachii by all available means. Supination movements remained absent due to changes in the distal radioulnar joint and surrounding muscles.

The patient declined a proposed tendon transfer to augment the biceps using the pectoralis major muscle (rated 5 out of 5 in strength).

Occasionally, elbow arthrolysis is performed via Campbell’s posterior approach combined with lengthening of the triceps tendon. The efficacy of this Procedure also depends on biceps function. More encouraging functional results can be expected when surgeries are performed during childhood.

Procedures to improve hand function are among the most technically demanding. It is well established that conservative management of wrist flexion contractures and interphalangeal extension contractures yields unsatisfactory results due to a high rate of deformity recurrence.

To correct wrist flexion, A.A. Kozlovsky (1933) and B.S. Kutsenok and O.A. Rabinovich (1936) excised the proximal row of Carpal Bones and fixed the wrist in hypercorrection, but this only exacerbated the finger deformities.

Attempts by A. Steindler (1949) to correct flexor contractures via flexor tendon lengthening proved unsuccessful; he therefore recommended waiting until age 8–10, followed by carpal bone resection combined with shortening of the extensor tendons.

For severe flexion contractures in children over 8–10 years of age, L.E. Rozovskaya and Ter-Yegiazarov (1973) performed a wedge resection of the proximal carpal row, fixed the wrist in mild hyperextension, and subsequently shortened the extensor tendons. The drawback of this approach is that it must be delayed until after the child's 8th to 10th year of life, and simultaneous one-stage correction places excessive tension on the finger flexors while weakening them.

Consequently, since 1965, these authors began utilizing wrist stabilization via lavsan (polyester) sling arthrodesis. The indication for surgery is fixed, frequently recurring contractures. The technique involves threading a lavsan tape through a bone tunnel in the radius at the junction of its middle and lower thirds, and through a second tunnel in the third metacarpal bone, tying the ends under slight wrist hypercorrection. Following surgery, the wrist is immobilized for 3–4 months, and a wrist brace is recommended for an additional 6 months. According to the authors, the surgeries were effective in the majority of cases and frequently achieved the desired outcomes.

Adduction of the thumb and flexion contractures of the other digits are caused by shortening of the skin, palmar aponeurosis, fascia, and flexor tendons. To place the thumb in a functionally advantageous position, N.G. Mead, W.C. Lithgow, and H.J. Sweeney (1958) performed palmar skin incisions and arthrodesis of the metacarpophalangeal joint.

Rozovskaya and Ter-Yegiazarov utilized palmar skin and aponeurosis incisions to release not only the thumb but also the other digits from flexion contractures, covering the resulting skin defects with free skin grafts. Good outcomes were obtained in all cases, though they recommend continuing comprehensive conservative therapy for an extended period following surgery.

As for surgical interventions on the lower extremities, they are indicated when conservative treatment, administered continuously over a prolonged period, proves ineffective. The hip joints often exhibit rigidity in severe forms of arthrogryposis. To correct rigid flexion-abduction contracture of the hip, Steindler performed a subtrochanteric osteotomy, whereas Rocher and Mattner preferred ligamentocapsulotomy in children under seven years of age. Due to the inherent tissue rigidity, ligamentocapsulotomy does not always achieve complete correction of the contracture; consequently, in the postoperative period, THE POSITION OF the limb is adjusted every 5–7 days by changing the hip spica cast. Once proper correction is attained, the limb is immobilized in a plaster cast for two months, followed by ongoing rehabilitation and conservative management.

If soft-tissue surgery fails to yield the desired outcome in children over 7–8 years of age, a corrective subtrochanteric osteotomy is performed, supplemented by tenotomy when necessary. A hip spica cast extending down the opposite thigh is maintained until bone union is achieved, after which conservative therapy is initiated to strengthen the musculature. Children are trained to walk using a walker, transitioning subsequently to crutches.

While hip involvement occurs exclusively in severe forms of arthrogryposis, knee contractures are observed much more frequently. Rigid contractures refractory to conservative treatment, as well as frequent recurrences, necessitate surgical intervention. Extension contractures of the knee are the most common; in most cases, if the hip joints are unaffected, patients retain The ability to walk.

The quadriceps muscle and knee flexors are less severely weakened than in flexion contractures, scoring at least three out of five. Range of motion in the knee joint may be minimal or even present as a flail joint. Genu recurvatum frequently develops because the flexors are weaker than the quadriceps, and this hyperextension tends to progress during ambulation. Valgus deformity at the knee can also occur when the biceps femoris is stronger than the semitendinosus and semimembranosus muscles. During the child's growth, these factors induce anatomical alterations in the femoral and tibial epiphyses, laxity of the ligaments and joint capsule, and progressive instability during unbraced walking, which ultimately leads to subluxation or occasionally dislocation of the Tibia.

Therefore, surgical management of the knee is most frequently indicated whether the limb axis remains preserved or is affected by genu valga or genu recurvata.

A. Steindler and T.S. Zatsepin performed a Z-plasty lengthening of the quadriceps tendon combined with anterior capsulotomy, immobilizing the limb in a plaster splint with the knee flexed to 90°. A similar procedure was carried out by N.D. Kazantseva (1953) and L.Ye. Rozovska with Ter-Yegiazarov (1973), who referred to it as an arthrolysis, given that the fibro-fascial bands and the proximally displaced Patella were fused into a solid conglomerate. In cases of valgus deformity, lateral capsulotomy was performed along with lengthening of the lateral collateral ligament or its reconstruction using a Dacron (lavsan) strip. To prevent excessive tissue tension and ischemic skin necrosis, the knee was flexed to only 120–130° for the first 10–12 days postoperatively, followed by gradual, staged redression to achieve greater flexion. Joint mobilization exercises and comprehensive rehabilitation were initiated 21 days after surgery and continued for many months.

Following surgery and comprehensive rehabilitation, it was possible to preserve the achieved range of motion and significantly enhance muscle strength. Patients ambulate using non-locking orthotic braces for one to one and a half years, or for a shorter duration depending on Muscle Function. Flexion contractures of the knee in arthrogryposis present a much greater therapeutic challenge. Conservative treatment rarely succeeds in eliminating the contracture and restoring a functionally advantageous limb position. Furthermore, contracture recurrences are very common because the knee flexors are quite strong and consistently overpower the weakened quadriceps. Consequently, flexor contractures require the highest number of surgical procedures on the knee to bring the limbs into a functional position.

The technical difficulty of these operations stems from the fact that in flexion contractures exceeding 140°, the neurovascular bundle is also shortened, precluding acute, single-stage straightening of the leg. Therefore, preoperative conservative preparation is carried out using serial redressions, while staged plaster casts maintain the leg in the incrementally corrected position.

In the past, surgical procedures such as posterior tenocapsulotomy of the knee joint, supracondylar femoral osteotomy with limb axis alignment, or Vreden's arthroplasty were frequently employed.

With the Introduction of the Ilizarov apparatus, and subsequently the Volkov-Oganesyan fixator, flexion contractures are now corrected without prior conservative preparation. The fixator is applied via two pairs of transfixion pins inserted through the Femur and tibia. By turning the nuts on the threaded rods by 1 mm per day, the contracture is eliminated gradually, atraumatically, and safely for the patient. Apparatus-based techniques easily allow for the reduction of tibial subluxation and even dislocation. Following removal of the fixator, passive and, wherever possible, active knee movements are developed.

The primary challenge lies in the fact that once the contracture is corrected, the weakened quadriceps muscle is incapable of maintaining the leg in extension. Subsequent treatment is therefore directed at strengthening this muscle, and patients must wear stabilizing braces until muscular strength is recovered. In the majority of cases, knee joint function remains impaired.

Lower limb arthrogryposis is invariably accompanied by Foot deformities, predominantly equinovarus. The severity of the deformity varies; if left untreated, it progresses, becomes rigid, and leads to structural bone changes and thickening of the periarticular tissues, ligaments, and tendons.

Such deformities cannot be corrected by conservative means alone. Moreover, recurrences of equinus deformity frequently occur in patients with mild forms of arthrogryposis who were treated conservatively, thus necessitating surgical intervention.

Numerous surgical techniques for foot correction have been described. The difficulties encountered during these procedures are attributable to significant fibrous-scar tissue changes that densely adhere to and tether the Tarsal Bones.

The surgical procedures performed are virtually identical to those used for congenital clubfoot.

In children aged two to five to seven years, soft-tissue surgery according to Zatsepin—namely, tenoligamentocapsulotomy—is performed. This operation is entirely rational, as the primary pathology stems from alterations in the joint capsules, ligaments, and tendons fused into a solid conglomerate (for surgical technique, see "Congenital Clubfoot").

Once the foot is placed in a functionally advantageous position, a plaster cast is applied. Following the subsidence of edema after 7–10 days, the foot is gradually manipulated into slight overcorrection. Zatsepin's procedure solely addresses the varus Displacement of the hindfoot; therefore, in the presence of forefoot and midfoot adduction, an additional release of the ligamentous-capsular apparatus along the medial border of the foot is performed. C.N. Neuman, Ch.H. Herndon, and J. Strong (1958) advocated capsulotomy of the tarsometatarsal JOINTS OF THE foot.

In mild deformities and equinus recurrences, Zatsepin's procedure is supplemented by lengthening of the Achilles tendon, posterior capsulotomy of the ankle and subtalar joints, and lengthening of the flexor hallucis longus tendon.

Because an imbalance between the foot flexors and extensors persists and equinus relapses are frequent, L.Ye. Rozovska and Ter-Yegiazarov (1973) proposed performing an ankle allodesis. The primary objective of this surgery is the Prevention of equinus. As the authors note, a prerequisite for this procedure is the passive mobility of the foot into a neutral 90° position.

The essence of the procedure involves passing a Dacron (lavsan) strip through a transverse bone tunnel in the mid-lower tibia, routing it subcutaneously, and passing it through a tunnel in the third or fourth metatarsal bone. The ends of the strip are secured to form a tensioned loop maintaining the foot in a functionally favorable position.

Lavsanodesis can be performed in children under 9 years of age, when bone surgeries are still premature, yet ambulation is impaired due to equinus that persists even after Zatsepin's operation. Following this combined procedure, the duration of cast immobilization is four months. Subsequently, children must wear orthopedic footwear or an orthotic brace.

It is crucial to continue massage and stimulation of the foot extensors, bearing in mind that pathologically altered muscles lag in growth as the child develops, which precipitates relapses. Therefore, ongoing corrective physical therapy (PT) should be implemented, supplemented when necessary by staged corrective plaster or plastic casts.

In neglected clubfoot and recurrences in children aged 10–12 years, bony procedures—such as wedge osteotomies—are performed, with M.I. Kuslik (1931) advocating for crescentic resection. Wedge resection entails the excision of a wedge from the talar head and the anterior border of the calcaneus, specifically at the level of the Chopart joint, to reposition the distal foot segment. This osteotomy must span the entire width of the joint so that the bones are anchored solely by soft tissues on the plantar aspect, thereby establishing optimal conditions for correction.

A.Ye. Frumina (1936) observed no instances of secondary shortening of the foot during growth following wedge resections for congenital clubfoot in children aged 5–10 years.

If arthrogrypotic clubfoot is accompanied by an absence of ankle joint mobility, bone resection must be executed in a manner that allows the foot to be positioned at an angle of 100–105°, representing a slight equinus position.

In advanced cases of clubfoot, some orthopedists performed astragalectomy and achieved satisfactory outcomes. It was believed that the feet acquired a correct shape and became painless during walking, and that such surgical intervention did not affect subsequent foot growth. Therefore, it was suggested that these operations be performed even in children aged 4 years and older.

Summarizing the above, it can be concluded that foot deformities in arthrogryposis respond poorly to conservative treatment; however, it must be initiated in the first weeks of the child's life, similarly to the treatment of deformities in other joints. This prevents their progression, and in mild cases, even allows achieving satisfactory functional results, whereas in moderate and severe forms of arthrogryposis, conservative treatment serves as a preparation for surgery and facilitates its execution.

Treatment outcomes depend on the functional capacity of the muscles; therefore, prolonged conservative treatment must be continued after surgery, and sometimes repeated operations are required.