Introduction

Arthrogryposis is a descriptive term that describes an individual with congenital contractures of three or greater joints. Arthrogryposis is a congenital disorder of formation within the neuromuscular axis. Classification of arthrogryposis can include: classic, distal, and syndromic arthrogryposis. Classification helps us understand the extent of the disability.

By definition, arthrogryposis is congenital contractures of three or greater joints in multiple body areas. It is non-progressive. Its incidence is 1 in 3,000–5,000 live births. Arthrogryposis is not a specific diagnosis, but rather a clinical finding. It is a characteristic that is seen in over 300 different disorders. An isolated congenital contracture affects only a single area of the body, such as seen in congenital club foot, which occurs in 1 of every 500 live births. This is distinctly different than arthrogryposis, which affects two or more different joints of the body. Treatment is based on functional disabilities and is aimed at improving functional abilities by improving limb position, strength, and mobility. The primary long-term goals of treatment are to improve use of adaptive patterns to allow for walking and independence with activities of daily living.

Classification

As shown in Fig. 23.1, congenital contractures can be divided into isolated congenital contractures, such as club foot, or multiple congenital contractures which are termed arthrogryposis [1]. Hall (1983 reference) has classified arthrogryposis as limb only, limb and viscera, or limb and CNS (Table 23.1). Clinically, this presents as three distinct types: classic (amyoplasia), distal, and syndromic.

Fig. 23.1
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Classification of types of arthrogryposis [1]

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Table 23.1 Types of arthrogryposis as classified by Halla
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Classic arthrogryposis is also known as amyoplasia, or arthrogryposis multiplex congenital (AMC). This is a distinct form of arthrogryposis with characteristic clinical findings. Amyoplasia refers to a = no, myo = muscle, plasia = growth. In this condition, the shoulders are usually internally rotated and adducted, the elbows are extended, the wrists are flexed and ulnarly deviated, the fingers are stiff, and the thumbs are in the palm (Fig. 23.2). If there is lower extremity involvement, the hips may be dislocated, the knees are extended, and the feet often have severe equinovarus contractures. Many patients have a mid-facial hemangioma. Associated conditions can exist. In one series, 10 % of children had gastroschisis or bowel atresia [2]. In most clinical series, symmetrical involvement of the upper and lower extremities occurs. Other variations include upper extremity only, lower extremity only, or asymmetric involvement. In Hall’s original description of 135 patients with amyoplasia, all cases were sporadic; however, there was an increased prevalence in twins and it occurred more commonly in conditions that would lead to decreased intrauterine limb movement, such as a bicornuate uterus, oligohydramnios, or intrauterine crowding [3].

Fig. 23.2
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A baby with classic arthrogryposis shows the typical features of internally rotated shoulders, extended elbows, flexed and ulnarly deviated wrists, stiff fingers, and the thumbs are in the palm. Additionally, her hips are dislocated, her knees are extended, and her feet have severe equinovarus contractures

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Distal arthrogryposis includes ten distinct types as seen in Table 23.2. As described in the Online Mendelian Inheritance in Man® (OMIM®) [4], distal arthrogryposis includes what was previously called Freeman–Sheldon syndrome, Sheldon–Hall syndrome, Gordon syndrome, and multiple pterygium syndrome. Specific diagnostic criteria are necessary to make a diagnosis of a distal arthrogryposis. In the upper limb, major diagnostic criteria include camptodactyly, hypoplastic or absent flexion creases, overriding fingers, and ulnar deviation of the wrist (Fig. 23.3). This is commonly referred to as “the windblown hand.” For the lower limb, major diagnostic criteria include talipes equinovarus, calcaneovalgus deformities, congenital vertical talus, and/or metatarsus adductus. To be affected, an individual must exhibit two or more major criteria; however, when a first degree family member meets diagnostic criteria, other family members only need one major criterion to be affected.

Table 23.2 Distal arthrogryposis syndromes
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Fig. 23.3
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Clinical features of distal arthrogryposis are seen in this father and son. The autosomal dominant disorder shows camptodactyly in the digits, with mild ulnar deviation of the wrists. Dislocated radial heads are noted by the prominence in the lateral elbow for the son

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Syndromic arthrogryposis includes multiple CNS disorders or neuromuscular diseases, which include multiple congenital contractures. Developmental abnormalities that affect the forebrain, such as microcephaly, are sometimes associated with arthrogryposis. Genetic peripheral neuropathies with an onset during fetal life are rare causes of arthrogryposis. Neuromuscular junction blockade in fetuses carried by mothers with myasthenia gravis or autoantibodies against fetal acetylcholine receptors can result in arthrogryposis [5].

Etiology

Multiple congenital contractures appear to have a final common pathway. In the normal fetus, joint formation occurs by cavitation between 26 and 52 days post-fertilization. In order for normal joint development to occur, there must be adequate space, nerve supply, and muscle activity to promote normal joint formation. A disruption in any of these elements will lead to loss of normal joint movement, causing congenital contracture [1, 5]. Restricted movement can occur through fetal crowding with multiparous births, or uterine abnormalities such as a bicornuate uterus. Maternal illness can cause restricted movement, such as myasthenia gravis. Abnormal muscle or nerve development additionally leads to congenital contractures. Oligohydramnios has a known association with multiple congenital contractures. Classic arthrogryposis (amyplasia) is not known to have a specific genetic cause.

Distal arthrogryposes are a group of autosomal dominant disorders that mainly involve the distal aspects of the limbs, characterized by primary hand and foot involvement, limited involvement of proximal joints, and variable expressivity [6]. Mutations in at least five genes (TNN12, TNNT3, TPM2, MYH3, and MYH8) that encode components of the fast twitch contractile myofibers have been associated with distal arthrogryposis [7–9]. For example, in approximately 90 % of cases of distal arthrogryposis type 2, mutations are found in MYH3, a gene that encodes embryonic myosin. Mechanisms by which altered contractility leads to congenital contracture are not known.

Syndromic arthrogryposis is commonly most severe and includes many CNS and muscular diseases [1]. CNS malformations that are associated with diminished corticospinal activation of spinal cord motor neurons, such as hydranencephaly or microcephaly, most likely contribute to fetal hypomobility and development of congenital contractures [10]. Congenital neuropathies, myopathies, and muscular dystrophies may similarly lead to multiple congenital contractures due to lack of normal fetal movement.

Historical Perspective

Adolph Wilhelm Otto first described an infant with multiple congenital contractures noted at autopsy in 1841. He described this as “a monster with inwardly curved extremities.” This has been credited as the first written description of arthrogryposis.

Clinical Manifestations of Arthrogryposis

The most common presentation to the hand surgeon includes classic arthrogryposis and distal arthrogryposis. Many children with syndromic arthrogryposis that includes limb and viscera are not surgical candidates. Patients with limb and CNS involvement have a lethal presentation as stillborn.

Classic Arthrogryposis (Amyoplasia)

Patients with classic arthrogryposis (amyoplasia) most commonly have lack of formation of normal musculature. The lack of normal muscles leads to multiple congenital joint contractures in the upper extremity. The most common pattern of deformity in the upper extremity is internal rotation of the shoulder with weak or absent shoulder girdle muscles; extension contracture of the elbow with weak or absent biceps and brachialis muscles; pronated, flexed, and ulnarly deviated wrists, with weak or absent wrist extension; and rigid digits with thumb and palm deformity. The degree of stiffness and weakness ranges from mild to severe and is not progressive.

The goal of treatment for children with arthrogryposis is to improve their quality of life by facilitating functional independence. At birth, nonoperative measures are initiated, with range of motion exercises, muscle and joint stretching, and splinting of specific joints to improve passive range of motion. Treatment to improve the function of the upper limb requires comprehensive planning with simultaneous assessment of shoulder, elbow, wrist, forearm, and hand function.

Nonoperative management is initiated at birth, and most commonly carried out for at least 12 months. Improvement of joint mobility is common, particularly at the elbow and wrist. The elbow is most critical in terms of achieving passive mobility to gain hand-to-mouth function. If after nonoperative treatment functional independence is still not possible, consideration for surgical treatment is explored [11]. Possible surgical treatment options are shown in Table 23.3.

Table 23.3 Surgical treatment options in amyoplasia
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Clinical Features of Amyoplasia

The joints of the upper and lower extremities are stiff in varying degrees. The skin is smooth over the joints, with reduced or absent skin creases. Oftentimes at large joints, particularly the shoulders, skin dimples are seen. Reduced mass of the muscles is visualized, and palpation shows an increase of firm tissue with an increase in fibrous tissue. A similarity in facial appearance is notable; intellectual development is usually normal.

Treatment of the Shoulder

In most patients, shoulder internal rotation is an integral part of their ability to perform bi-manual skills as the shoulder internal rotation helps bring their hands to midline and cross over to assist with grasp, as shown in Fig. 23.4. However, in some children, if the internal rotation contracture is severe and actually interferes with function, an external rotational osteotomy of the proximal humerus can be performed to improve function.

Fig. 23.4
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De-rotation osteotomy of the shoulder can be indicated for severe internal rotation positioning of the limb causing significant dysfunction. Care must be taken with judicious use of this operation as most children with amyoplasia use the shoulder internal rotation to assist with bimanual hand use as shown in this figure

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Treatment of the Elbow

Nonoperative management is initiated at birth, and most commonly carried out for at least 12 months. Improvement of joint mobility is common, particularly at the elbow and wrist. The elbow is most critical in terms of achieving passive mobility to gain hand-to-mouth function. If after nonoperative treatment elbow flexion is insufficient to allow passive mobility of hand to mouth, surgical treatment would be indicated. Specifically, if less than 90° of flexion is achieved and the hand cannot be brought to the mouth passively, a posterior elbow capsulotomy with triceps lengthening would be indicated [11]. Indications for surgery are less than 90° of passive elbow flexion and an inability to reach the hand to the mouth.

Surgical Technique

Posterior elbow capsulotomy with triceps lengthening is performed with the patient in a lateral decubitus position. A sterile tourniquet is used, at least through initial dissect to allow identification and protection of the ulnar nerve. The posterior aspect of the elbow is identified by palpation. Caution should be taken that oftentimes the limb is so internally rotated that the medial epicondyle can be mistaken for the olecranon. A curvilinear incision is made down the posterior aspect of the elbow. In arthrogryposis, significance of cutaneous tissue with minimal tissue planes is a common pathological finding. The ulnar nerve is identified as it passes through the inner muscular septum and through the cubital tunnel. Osborne’s fascia is released, and the ulnar nerve is protected with a vessel loop. The sterile tourniquet then can be removed to allow greater proximal dissection and triceps mobilization once the ulnar nerve has been identified and protected, particularly in the small child. The tricep is isolated on its insertion at the olecranon. Dissection is carried out medially and laterally, isolating the triceps tendon back to the level of the musculotendinous junction as shown in Fig. 23.5. A Z-lengthening or V-Y advancement is performed; at least doubling the length of the tendon will be necessary to provide appropriate elbow flexion.

Fig. 23.5
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Posterior elbow capsular release with triceps lengthening is performed through a posterior incision. The triceps is isolated, as shown here, and is subsequently lengthened using a Z-lengthening technique

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The posterior aspect of the capsule is then incised, exposing the joint surface. The arthrotomy is extended medially and laterally to allow maximum elbow flexion with gentle passive stretch. It is important to be careful about increasing the joint mobility, as physeal fractures can occur if excessive force is used. Dissection most commonly needs to be carried out at least to the mid-axial line and may include the posterior aspects of the medial and lateral ligaments. Full flexion of the elbow is the goal of the posterior capsular release. The triceps is then repaired in an elongated position with use of a non-absorbable or reinforced suture. The skin is closed, and a light dressing applied. The limb is then placed in a hinged elbow brace or a long-arm cast in at least 90° of flexion. Passive range of motion to allow joint mobility is initiated as soon as tolerated by the patient. Therapy is advanced to include hand-to-mouth activities with passive flexion. During the first month, this is limited to 90° to protect the triceps lengthening, and advanced thereafter to full passive flexion. Use of a splint to maximize flexion during the day is possible with a hinged splint and use of rubber bands anteriorly as shown in Fig. 23.6. If an elbow flexion contracture ensues, alternative nighttime flexion splinting alternated with extension splinting can be initiated.

Fig. 23.6
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Use of a splint to maximize flexion during the day is possible with a hinged splint and use of rubber bands anteriorly as shown. During the first month, this is limited to 90° to protect the triceps lengthening, and advanced thereafter to full passive flexion

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Surgical Outcomes

Several series examining results of posterior capsular release with triceps lengthening report excellent results. For example, Van Heest et al. [11] reported on a study group of 23 children treated between 7 months and 13 years of age with an average follow-up of 5.4 years. Prior to the surgery, the average arc of passive motion was 32°, with an average of 38° of flexion. An arc of at least 90° of passive flexion was achieved in all children intraoperatively. At an average follow-up of 5.4 years, 22 of the 23 children were able to feed themselves with the hand on the operated side. Twenty-one of the children with less than grade three elbow flexion strength required the use of passive assistance. No further muscle transfers were performed in these children, as adaptive mechanisms, as shown in Fig. 23.7, allowed independent activities of daily living.

Fig. 23.7
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Nonoperative intervention for active elbow flexion requires the use of passive elbow flexion, and adaptive maneuvers such as tabletop push (a), swinging of the arms (b), or contralateral arm use (c, d) to bring the hand to the mouth. Many children are quite creative in being able to passively achieve hand-to-mouth function, so that operative treatment with muscle transfer is not necessary

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Operative Outcomes with Muscle Transfer

Several options exist for muscle transfers. First, if passive range of motion has been achieved either operatively or nonoperatively, passive adaptive maneuvers can be performed by the child for functional use of the elbow. Such is described by Van Heest et al. [11]. Nonoperative intervention for active elbow flexion requires the use of passive elbow flexion, and adaptive maneuvers such as tabletop push (see Fig. 23.7a), swinging of the arms (see Fig. 23.7b), or contralateral arm use (see Fig. 23.7c, d) to bring the hand to the mouth. Many children are quite creative in being able to passively achieve hand-to-mouth function.

Operative measures to improve active elbow flexion include transfer of the flexor pronator origin (Steindler) [12, 13]; transfer of the pectoralis muscle; transfer of the triceps muscle; free muscle transfer of the grascilis; or, most recently, transfer of a single head of the triceps on its separate neurovascular pedicle. One review of the results of surgical treatment of arthrogryposis with tendon transfer surgery examined 18 tendon transfers in 14 children with an average follow-up of 4 years [14]. Using functional outcome criteria, six of nine transfers provided good function, one provided fair, and two provided poor. The most common reason for downgrading was development of an elbow flexion contracture, which precluded active and passive elbow extension after triceps transfer. Subsequent studies have similarly shown severe elbow flexion contractures and, most commonly, triceps to biceps tendon transfer is no longer recommended [15]. The pectoralis transfer can be used as a unipolar [16], partial bipolar [17], or complete bipolar transfer [18]. The advantage of the pectoralis transfer is that additional muscle mass is added to the hypoplastic limb. The disadvantage is the extensive dissection necessary. It may also be contraindicated for use in females because of the chest wall deformity; lack of predictability of strength is common as well. The third available option for transfer is the latissimus dorsi muscle. Muscle mass is added from the chest wall to the hypoplastic limb without significant loss of function. However, in many children with arthrogryposis, the latissimus dorsi muscle is underdeveloped and insufficient for transfer. Several authors have recommended pre-operative evaluation by MRI scan or intraoperative assessment of muscle quality prior to transfer. Additionally, due to its shape as a long muscle, extension is difficult to assess. The Steindler transfer, as described by Goldfarb et al. [12], is a less invasive elbow flexion transfer. The medial epicondyle origin of the flexor pronator muscle is divided and transferred to the anterior portion of the humerus. This transfer has been shown to improve initiation of elbow flexion, but has difficulty with achieving the full arc of elbow flexion for hand-to-mouth function. Additionally, critics have been concerned about enhancing the Steindler effect in requiring simultaneous wrist and elbow flexion in children who already have a wrist flexion contracture. Lastly, transfer of a single head of the triceps has recently been described by Ezaki [19]. Isolation of a single head of the triceps would allow transfer of one head while maintaining the other two heads as an antagonist elbow extensor. Theoretically, this would avoid the elbow flexion contractures seen after triceps to biceps transfer. The difficulty with the muscle transfers described above is that most children with amyoplasia have weak muscles, and transferring a weak muscle does not provide significant strength; thus, most of the outcomes of muscle transfer surgery are only good, not excellent.

Radial Head Dislocations

Some children with arthrogryposis will present with radial head dislocations. On physical examination, prominence of the radial head may be seen or palpated (Fig. 23.8); radiographs will reveal a radial head dislocation. If this occurs, assessment of the effect of loss of range of motion must be conducted. For example, an anteriorly dislocated radial head can block terminal flexion. Resection of the radial head can, in some cases, restore or improve function [20].

Fig. 23.8
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A dislocated radial head can be diagnosed on physical examination by prominence of the radial head at the lateral head as seen here. Motion of the radial head can be palpated during pronation and supination

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Treatment of the Wrist

Nonoperative management of the wrist includes passive range of motion and splinting. Most commonly, a wrist hand orthosis is worn at night to improve passive extension of the wrist and fingers (Fig. 23.9). During the day, wrist splints are avoided because movement of the wrist is already limited in these stiff joints, and further splinting most commonly does not enhance function.

Fig. 23.9
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Wrist hand orthosis worn at night to improve passive extension of the wrist and fingers. During the day, wrist splints are avoided because movement of the wrist is already limited in these stiff joints, and further splinting most commonly does not enhance function

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The most common treatment of the wrist is dorsal carpal wedge osteotomy. Dorsal carpal wedge osteotomy was first described by Ezaki in 1993 [19]. Surgical indications for dorsal carpal wedge osteotomy include excessive wrist flexion contracture deformity which limits upper extremity function, having failed nonoperative treatment. Of particular note is that some children with severely stiff upper limbs do use their wrist flexion posturing in order to achieve hand-to-mouth function or to assist in crawling and standing up (Fig. 23.10). If this is the case for a child, straightening the wrist would worsen their abilities. Only in children with adequate elbow flexion should wrist extension osteotomies be performed.

Fig. 23.10
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Some children with arthrogryposis have severely stiff upper limbs and they use their wrist flexion posturing in order to achieve hand-to-mouth function or to assist in crawling and standing up, as shown here. If this is the case for a child, straightening the wrist would worsen their abilities. Only in children with adequate elbow flexion should wrist extension osteotomies be performed

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Surgical Technique

Dorsal carpal wedge osteotomy is performed using a dorsal approach to the wrist; the digital and wrist extensor tendons are isolated and protected. A dorsal capsulotomy is then performed. At the level of the midcarpus (Fig. 23.11), a dorsal wedge osteotomy is made sufficient to correct the wrist flexion deformity to at least a neutral position, taking care that noteworthy finger flexor tightness is not produced by tenodesis. If ulnar deviation correction is required as well, the dorsal carpal wedge can resect more bone on the radial side to provide biplanar deformity correction. This position is held in place with two cross K-wires. In addition, tendon transfer of the extensor carpi ulnaris (ECU) to the extensor carpi radialis brevis may be performed to correct the ulnar deviation deformity or wrist extension weakness, or both, if the ECU tendon is noted to have sufficient excursion intraoperatively. After the procedure, the patient is placed in a cast for 1 month. If radiographs show healing of the osteotomy, the cast is removed and the K-wires are pulled. The patient is given a wrist splint for protection and begins to participate in occupational therapy activities for wrist range of motion, particularly wrist extension, and hand function. Removable night splints are indicated on a case-by-case basis if needed for further improvement of wrist extension.

Fig. 23.11
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Dorsal carpal wedge osteotomy is performed using a transverse dorsal incision and dorsal capsulotomy. A dorsal wedge of carpus is excised through the midcarpal joint (a, b), which is often synostostotic. Preservation of the radiocarpal joint is essential to maintain the limited arc of motion present. Pinning in a position of extension (c, d) with cast for 4–6 weeks to allow for bone healing is recommended. Concomitant centralization of the ECU tendon can improve long-term results for maintaining wrist extension

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Surgical Outcomes

An evaluation of 20 wrists in 13 children with an average 4 years follow-up revealed a mean improvement of 43° of wrist extension with a loss of 35° of wrist flexion [21]. No significant change in the arc of motion was seen; however, extension was relocated into a more functional extended position. In one review [21] children older than 7 years of age at the time of surgery had significantly greater extension improvement than those less than 7 years of age. Additionally, patients who had a concomitant ECU tendon transfer at the time of dorsal carpal wedge osteotomy had a greater improvement in wrist extension. Dorsal carpal wedge osteotomy can significantly improve wrist extension while at the same time preserving the arc of motion (Fig. 23.12).

Fig. 23.12
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This patient presents after a left wrist dorsal carpal wedge osteotomy (DCWO) requesting that the right wrist be treated. Part (a) shows a clinical picture of the post-op DCWO on the left wrist and a pre-op DCWO on the right wrist. Part (b) shows the lateral radiograph of the post-op DCWO on the left wrist and a pre-op DCWO on the right wrist. Prior to the surgery, the left wrist had similar deformity to the right wrist

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Treatment of the Hand

Syndactyly releases are most commonly a partial syndactyly and can be performed using local flaps with or without skin graft. The patterns in the hand with amyoplasia are similar to those with distal arthrogryposis and will be discussed together.

Distal Arthrogryposis

The second type of arthrogryposis commonly seen by hand surgeons is distal arthrogryposis. Features shared by all distal arthrogryposes include a consistent pattern of hand and foot involvement, limited proximal joint involvement, and variable expressivity. Ten different types of distal arthrogryposes have been described to date (see Table 23.2). Most commonly in these types of arthrogryposis the “windblown hand” is seen. The windblown hand includes ulnar deviation of the digits through the metacarpophalangeal (MCP) joint, stiff digits, and thumb-in-palm. The digits can be stiff in flexion, such as seen in camptodactyly, or stiff in extension, with side-to-side intrinsic grasp patterns. The thumb is typically flexed across the palm with adduction of the ray through the carpometacarpal joint, as well as flexion of the MCP joint. Simple incomplete syndactyly is common (Fig. 23.13).

Fig. 23.13
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Simple incomplete syndactyly is common in arthrogryposis and can be treated with local flaps or full thickness skin grafts if functionally limiting finger or thumb use

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Treatment of the Hand

The mainstay of treatment for the windblown hand is nonoperative management with splints for improved positioning and passive range of motion of the joint, starting as an infant when the diagnosis is first made. In the early school-age child, if positioning has not improved then surgical management can be considered.

Surgical management in the windblown hand would include release of contractures. Release of camptodactyly has been disappointing, so that stiff digits are most commonly treated nonoperatively.

Treatment of the Thumb-in-Palm Deformity

Treatment of thumb-in-palm deformity involves repositioning of the thumb through osteotomies, fusions, or tendon transfers. Release of the first web can include a dorsal rotation flap, a Z-plasty, or volar skin grafting. Release of the thumb adductor is performed as described by Matev [22], with release of the origin of the thumb adductor from the third metacarpal, thus preserving its pinch power through preserving its nerve supply. This is important in children who are already weak when maximum thumb pinch strength needs to be preserved. If posturing across the palm is severe, consideration of an MCP fusion to position the thumb MCP joint in greater extension can be considered in the older child. In the younger child, release of the volar capsule and augmentation of the dorsal capsule with pinning for 4–6 weeks to allow healing can be considered. Augmentation of the extensor pollicus brevis tendon through transfer from the extensor indicis proprius has been used as shown in Fig. 23.14. Transfer of the extensor carpi radialis longus tendon, if present, to the first ray can improve abduction of the ray itself. Large series are not available for either of these surgical techniques. Adduction of the first metacarpal with contracture of the first web and volar skin is often accompanied by contracture of the thumb adductor and deficient thumb extension. Thus, a thumb reconstruction would include release of the first web, with possible skin grafting on its volar aspect.

Fig. 23.14
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Thumb-in-palm deformity is a common feature of both amyoplasia and many types of distal arthrogryposes (a). Surgical treatment with Z-plasty of the contracted volar skin, MP dorsal capsulodesis, and augmentation of thumb extension with transfer of the EIP tendon to EPB can improve thumb position and function (b)

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In some cases, children with arthrogryposis will present with hyperextension deformity through the MCP joint as shown in Fig. 23.15. Most likely this will be due to adduction of the first metacarpal across the palm, with secondary stretching of the volar capsule in hyperextension, which can lead to dislocation of the MCP. Release of the first ray using the Matev procedure [15] with a volar capsulotomy as described by Tonkin et al. [23] has been conducted. Surgical operations for the windblown hand reviewed by Wood [24] concluded that the most common procedure was Z-plasty of the thumb, followed by release of the thumb adductor, extensor indices proprius transfer to extensor pollicis longus or extensor pollicis brevis, with dorsal rotation flap or skin grafting. In three cases lengthening of the flexor pollicis longus tendon was necessary.

Fig. 23.15
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Thumb-in-palm can on occasion cause adduction of the first ray with secondary hyperextension of the MCP joint. In cases such as these, volar capsulodesis would be necessary as part of surgical treatment. In the older child, MCP joint fusion may be an option

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Summary

In summary, arthrogryposis is a disorder of joint formation of the neuromuscular axis leading to multiple congenital contractures. Classification as classic arthrogryposis (amyoplasia), distal arthrogryposis, and syndromic arthrogryposis helps us understand the extent of disability and its treatment. Amyoplasia is the most common arthrogryposis that is treated surgically. Elbow capsular release with triceps lengthening, dorsal carpal wedge osteotomies, and thumb-in-palm correction are the most common surgical procedures. Treatment is based on functional positioning and use of the limb. The goal of management of the child with arthrogryposis is to increase independence by improving joint position and mobility.