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13 March 2026: Review Articles  

Management of Lower-Extremity Deformity in Arthrogryposis Multiplex Congentia: A Narrative Review

Yongwei Shang DEF 1, Jianjie Xu ABC 1*

DOI: 10.12659/MSM.951581

Med Sci Monit 2026; 32:e951581

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Abstract

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ABSTRACT: Arthrogryposis multiplex congenita (AMC) is a highly heterogeneous constellation of disorders defined by non-progressive congenital multiple joint contractures, typically manifesting across multiple limbs. The pathology results in significant functional impairment, including restricted range of motion, chronic arthralgia, and secondary musculoskeletal deformities like scoliosis. Given that AMC is an umbrella designation encompassing over 300 distinct etiologies, the profound clinical variability poses substantial diagnostic and therapeutic challenges. The relative rarity of AMC and the absence of consensus-based, longitudinal treatment protocols create a critical void in standardized clinical management across the lifespan of patients. This comprehensive narrative review synthesizes the contemporary literature and clinical evidence to establish a structured, life-course management paradigm, extending from neonatal screening through to adult care. We advocate for an evidence-based approach that recalibrates therapeutic goals to emphasize maximal functional capacity and societal participation rather than strict anatomical normalization. Key aspects addressed include early-life neuroplasticity, the principles of staged and minimally- invasive surgical correction, and the need for seamless, lifelong, multidisciplinary care coordination. Furthermore, the review critically examines persistent clinical dilemmas concerning hip and knee contracture management, and proposes algorithmic pathways for addressing recurrent foot deformities. By integrating the latest advancements in molecular genetics, surgical innovations, and rehabilitative science, this work serves as an authoritative resource, offering clinically applicable strategies to optimize long-term outcomes for individuals living with AMC.

Keywords: Arthrogryposis, Surgical Procedures, Operative, Rehabilitation, Ilizarov Technique, Limb Deformities, Congenital

Introduction

Arthrogryposis multiplex congenita (AMC), a term first introduced in 1923, encompasses a spectrum of congenital disorders characterized by non-progressive contractures in 2 or more body areas. Its etiology is diverse, involving genetic, parental, environmental, and fetal developmental factors. Clinical presentation includes limited joint mobility with or without muscle weakness. While contractures do not spread to unaffected joints, their manifestation is dynamic and can change with growth and therapeutic intervention [1]. Arthrogryposis multiplex congenita (AMC) is a condition characterized by the presence of congenital multiple joint contractures at birth, typically involving multiple limbs and occasionally accompanied by abnormalities in other body parts and/or neurological involvement. According to international studies, its estimated prevalence ranges from 1 in 3000 to 1 in 5000 live births. Due to aberrant joint development, affected individuals may exhibit restricted active and passive movement of limb joints, arthralgia, scoliosis, and neurological abnormalities [2]. Arthrogryposis is associated with over 400 medical conditions and 350 known genes, demonstrating considerable phenotypic variability. The primary pathogenic mechanism is believed to be reduced fetal movement during development. Familial aggregation has been observed in some cases. Current genetic studies indicate that variants in genes such as TTN, CHRNG, RYR1, and ECEL1 account for 27.7% of AMC cases. Disorders presenting primarily with multiple joint contractures include both autosomal dominant inheritance patterns (eg, DA, CPSFS1A, and DAIPT) and autosomal recessive forms (eg, CPSFS1B) [3].

AMC is a heterogeneous group of syndromes rather than a single disease entity, uniformly characterized by the presence of multiple joint stiffness and contractures at birth [4]. Clinical manifestations vary widely but typically involve 2 or more joints in a symmetric distribution, with predominant involvement of the lower extremities. A hallmark feature is severely restricted active and passive range of motion, often resulting in fixed deformities such as flexion, extension, adduction, or abduction. Common presentations include hip dislocation with mild flexion, knee hyperextension, and talipes equinovarus. Limb muscles are frequently hypoplastic, contributing to a characteristic cylindrical appearance of the extremities. It is important to note that AMC itself is non-progressive, although some underlying conditions leading to AMC (eg, muscular dystrophies) may exhibit progressive deterioration [5].

AMC is generally categorized into 3 subtypes: amyoplasia, distal arthrogryposis (DA), and syndromic forms [6]. Among these, distal arthrogryposis is the most common subtype and comprises a heterogeneous group of disorders characterized primarily by contractures affecting the hands and feet, with relative sparing of major proximal joints, without a primary neurological and/or muscle disease. Many cases of DA are linked to specific genetic mutations in genes encoding proteins integral to the contractile apparatus. The mode of inheritance is most frequently autosomal dominant, although X-linked mutations have also been documented [7]. The 10 identified forms of distal arthrogryposis are organized into a hierarchical classification, which systematically groups them by their common characteristics [8]. In contrast, amyoplasia is defined by multiple symmetric limb contractures accompanied by muscular hypoplasia and fibrofatty replacement of affected muscle tissue. Intellectual function is typically preserved in these individuals [9]. The clinical spectrum of AMC is broadly divided into non-syndromic forms, such as amyoplasia and distal arthrogryposis, and syndromic forms. The latter is a common and often severe category, which can be further categorized by etiology into prenatal neurogenic disorders and myopathic/neuropathic disorders. A definitive diagnosis requires comprehensive neurological and musculoskeletal evaluations. AMC is not a single disease entity, but rather an umbrella term encompassing over 300 disorders with diverse etiologies. It can arise from various causes, including neurological, muscular, or connective tissue abnormalities, as well as intrauterine space constraints [10]. This considerable heterogeneity leads to significant variability in clinical presentation, severity, and prognosis among affected individuals.

The management of AMC is prolonged and typically requires long-term, multidisciplinary collaboration involving rehabilitation therapy, orthotic devices, and multiple surgical interventions. As a rare disease, clinical research on AMC is often constrained by small sample sizes, fragmented studies, and a lack of high-quality randomized controlled trials, resulting in inconsistent treatment standards and variable outcome evaluations. To address these challenges, this article provides a narrative (non-systematic) review of the management of lower limb deformities in AMC. It is important to note that this review did not employ PRISMA-style screening, duplicate study selection, or a formal risk-of-bias appraisal of the included literature. While recent international consensus and seminal reviews (eg, van Bosse [11], Dahan-Oliel et al [12]) have defined the etiological landscape and general rehabilitation principles for AMC, a granular synthesis of surgical strategies for specific anatomical regions is still lacking. This narrative review addresses this gap by establishing a deformity-by-region management framework focused on the lower extremities. Unlike broader guidelines, our work integrates surgical outcome updates and evidence-based advancements from 2020 to 2025, providing a life-stage rehabilitation synthesis directly mapped to orthopedic decision-making for the hip, knee, and foot. By bridging the divide between generalized care and specialized surgical techniques, we provide clinicians with a practical roadmap to optimize long-term functional autonomy in patients with AMC.

Methods

This study utilized a narrative review methodology. The literature search was intended to be selective rather than exhaustive, aiming to identify representative evidence and key clinical advancements rather than to comprehensively retrieve all available studies. In August 2025, a selective literature search was performed in the Medline database. The search strategy incorporated a combination of keywords pertaining to arthrogryposis multiplex congenita (AMC), such as “arthrogryposis,” “amyoplasia,” and “multiple congenital contractures,” together with terms related to lower-limb deformities (“hip,” “knee,” “foot”) and rehabilitation. These terms were connected using Boolean operators (“AND” or “OR”). The timeframe covered included literature published from the inception of the database to August 2025, with a specific focus on the period between 2020 and 2025 to ensure the inclusion of contemporary practices.

Additionally, the reference lists of the selected articles were reviewed to identify further relevant publications. The search was restricted to articles available in English to ensure the feasibility of detailed data synthesis by the authors. The most clinically relevant and recent literature was selected, spanning from randomized controlled trials to case series, to provide a narrative overview of the current management landscape.

Management Principles

Arthrogryposis multiplex congenita (AMC) is a complex condition requiring a comprehensive and individualized approach to treatment. It is crucial to understand that AMC is a spectrum of conditions marked by multiple congenital joint contractures, rather than a single disease entity [13]. This heterogeneity precludes a uniform treatment protocol and underscores the need for tailored interventions. Contemporary management is centered on a multidisciplinary team (MDT) approach, integrating expertise from various specialists, including orthopedic surgeons, neurologists, physiatrists (rehabilitation physicians), physical and occupational therapists, and orthotists (bracing specialists) [14]. Collaborative MDT efforts facilitate the development of holistic treatment plans aimed not merely at correcting individual joint deformities, but at optimizing overall functional independence and quality of life [15]. This paradigm represents a substantial shift from earlier reductionist models, reflecting a deeper understanding of the systemic nature of the condition.

Initial management prioritizes non-surgical interventions, which serve as the foundation of treatment. Physical therapy, initiated within the first weeks of life, is critical for preserving and improving joint mobility and muscle strength. This is commonly combined with serial casting and splinting to gradually correct deformities. These conservative measures are not merely preliminary; they can substantially reduce the need for surgery or the extent of later operations [16]. When surgery is necessary, the modern philosophy has shifted from aggressive, one-time interventions to a more sequential and planned approach. Instead of tackling a single “problem joint”, the team assesses all affected areas, such as the hips, knees, ankles, and feet, and plans a series of procedures at optimal times. This might involve early, aggressive soft-tissue releases in infancy (age 3–12 months), with more complex bony osteotomies planned for later in the toddler and childhood years as the child’s bones mature. This thoughtful, phased approach considers the entire lower extremity, ensuring that corrections lead to long-term ambulatory function rather than isolated, short-term solutions [17]. The ultimate goal is to empower children with the tools needed to achieve maximum mobility and independence as they grow.

Hip deformity and Management

Hip involvement is a common finding in children with AMC, with contractures (18–51%) occurring more often than dislocations (15–30%) [18]. A key distinction in AMC is that hip dislocations are typically congenital, unlike in other neuromuscular disorders in which they develop over time. A core principle of treatment is the early and aggressive correction of these deformities, particularly maintaining hip flexion contractures below 30°. This approach is critical for achieving and preserving functional ambulation into adulthood

Hip contractures in AMC commonly present as multiplanar deformities involving flexion, abduction, and external rotation. For mild-to-moderate isolated flexion contractures, a percutaneous anterior release is a minimally invasive option. This procedure involves a small incision to section the conjoined tendon and fascia lata, followed by casting and physical therapy [19]. When a multiplanar contracture leads to a poorly positioned, non-functional hip, a proximal femoral reorientational osteotomy may be necessary. This surgery realigns the lower extremity with the body by creating a wedge-shaped intratrochanteric osteotomy, while leaving the hip joint in its original position [20]. Despite a high rate of recurrence, studies show that these interventions can improve mobility and function [21]. For instance, a study found that all patients achieved deformity correction, and the majority had improved mobility, with many achieving independent or assisted ambulation at follow-up [22].

AMC hip dislocations occur earlier in gestation than idiopathic dislocations, notably in contrast to developmental dysplasia of the hip (DDH). This earlier onset results in stiffer joints and more severe displacement. The primary treatment goals are to achieve a stable, plantigrade posture and functional mobility. Unilateral dislocations are generally recommended for open reduction, especially in infants under age 12 months, to prevent secondary complications like pelvic obliquity and scoliosis. Manual closed reduction is typically unsuccessful due to the joint stiffness [18]. While an anterolateral approach provides clear surgical exposure, it carries a higher risk of bleeding and heterotopic ossification. Conversely, the medial approach offers better cosmesis and less blood loss but poses a greater risk of neurovascular injury [23]. Surgical stability can be enhanced with ligamentum teres reconstruction or temporary pinning, followed by a 6-week spica cast. Miao et al [24] suggested open reduction with a modified Smith-Petersen approach should be performed before the age of 12 months, reducing the need for revision surgery when performed early. It is important to note, however, that open reduction in AMC patients is associated with a higher rate of proximal femoral growth disturbance and re-dislocation compared to DDH, reflecting the challenging prognosis [25].

Bilateral dislocations of AMC, which are often high, stable, and symmetrical, frequently result in a balanced pelvis and functional gait. Historically, many surgeons have advised against reduction due to the high rate of complications, including re-dislocation, stiffness, and avascular necrosis, with complication rates exceeding 70% [26]. However, recent studies suggest that in selected patients with good hip motion and ambulatory potential, surgical reduction can lead to improved functional outcomes, including independent or assisted ambulation [27,28]. Surgical reduction should be considered only in selected patients with good hip motion and ambulatory potential [29]. Aydin reported that the short-term results of early open reduction of the hips were not promising, as most of the patients required additional hip surgeries [30]. Over the long term, if painful joint dysplasia and degeneration develop, total joint replacement (TJR) may be a viable option to alleviate pain, although it may not alter ambulatory status [31].

In the management of hip deformities associated with AMC, contractures (occurring in 18–51% of cases) are more frequent than dislocations (15–30%), and both often present congenitally [18]. The core treatment objectives are to achieve hip stability and preserve functional ambulation, with particular emphasis on maintaining hip flexion contractures below 30° [1]. Mild-to-moderate flexion contractures may be addressed with percutaneous anterior release followed by casting [32]. For multiplanar deformities involving flexion, abduction, and external rotation, proximal femoral reorientational osteotomy is recommended to improve mechanical alignment [21]. In cases of unilateral dislocation, early open reduction – ideally before walking age – using either an anterolateral or medial approach is advised to mitigate pelvic obliquity and secondary scoliosis; this is often supplemented with temporary stabilization and spica casting. Bilateral dislocations, if symmetric and stable, can be managed non-operatively, though reduction may be considered in selected ambulatory patients with preserved hip motion, despite risks including re-dislocation, stiffness, and avascular necrosis (exceeding 70%) [25,26]. Long-term monitoring is essential to detect recurrence or degenerative changes. Total hip arthroplasty can be effective for painful joint degeneration in older patients but does not improve ambulatory function [31]. Treatment must be highly individualized, emphasizing early intervention and functional outcomes [19].

Knee Deformity and Management

Knee deformities are the second most common lower-extremity issue in children with arthrogryposis, presenting as either flexion or extension contractures [32]. In severe cases, these can lead to anterior and proximal tibial dislocations. The primary objectives of treatment are to reduce these contractures and promote functional ambulation. It is important for families to recognize that although these deformities are most pronounced at birth, they are amenable to substantial improvement through early and proactive intervention. This potential for correction stems from the heightened plasticity and adaptability of neonatal connective tissues, which respond to sustained mechanical tension by facilitating gradual joint remodeling. Consequently, the initiation of treatment in the immediate postnatal period is critical to optimizing long-term mobility and functional outcomes [11].

Knee flexion contractures, a common finding in arthrogryposis, are often classified by severity: mild (<30°), moderate (30–60°), and severe (>60°) [33]. Early intervention is crucial, as some knee dislocations and flexion deficits can be manually reduced shortly after birth through a combination of joint distraction and progressive stretching. These conservative measures are often followed by serial casting. For mild and moderate contractures, non-operative treatments are typically preferred. When surgery is required, modern techniques like growth guidance with femoral anterior distal hemiepiphysiodesis are highly effective. This method, which involves the use of transphyseal screws, has shown great promise in correcting genu recurvatum (hyperextension). Close radiographic monitoring and timely hardware removal are essential to prevent complications such as neurovascular compromise [34]. Nei [35] reported a new surgical technique of femoral anterior distal hemiepiphysiodesis with transphyseal screws, which shows great potential for correcting genu recurvatum.

For moderate knee flexion contractures in arthrogryposis, a combination of posterior knee releases and other procedures is often required. In children aged 4 to 10 years, the application of 8-plates on the anterior distal femoral physis has shown success in correcting deformities, particularly those under 45°, and improving walking ability. These growth guidance techniques can be effective and less invasive than more extensive osteotomies. Mohamed et al [33] explored innovative methods for gradual correction, demonstrating the efficacy of a cast-rod construct for correcting fixed-knee flexion deformities in young children. Their study reported a significant reduction in contracture, with the median angle decreasing from 54.5° to 10° after treatment. It is important to recognize the significant impact of residual contracture on a patient’s mobility. A study of 51 patients with AMC found that a residual knee flexion contracture greater than 30° was strongly associated with a non-ambulatory status [36]. While surgical options like percutaneous extensor osteotomy of the distal femur can provide initial correction, a retrospective study by Marcus et al highlighted a high rate of recurrence, underscoring the challenge of maintaining long-term correction in these complex cases [37].

For severe knee flexion contractures in arthrogryposis, a surgical approach using an external fixator is often necessary, as hemiepiphysiodesis alone is unlikely to provide sufficient correction. This method allows for a gradual and controlled extension of the joint [38]. A common technique involves a knee-spanning external fixator combined with a posterior knee release. For example, Qin et al utilized a modified Ilizarov external fixator, surgically installed with the knee in its maximum extended position. Gradual distraction (slow stretching) commenced a few days after the operation at a rate of 2 to 3 mm per day, which was later adjusted to 2 mm per day. This process continued until full extension (0 degrees) was achieved. This technique has been shown to be a simple, safe, and effective method for correcting severe knee flexion deformities [39]. Proper placement of the fixator hinge, ensuring it aligns with the knee’s rotational axis, is crucial to prevent subluxation. Furthermore, initial joint distraction can help avoid impingement as the knee extends.

For skeletally mature patients with AMC, distal femoral extension osteotomies can provide a complete and immediate correction. However, this procedure is best reserved for those near skeletal maturity due to a high risk of recurrence from physeal remodeling in growing patients [40]. Similarly, while a supracondylar osteotomy with femoral shortening can be an option, it does not increase the total range of motion and has a high recurrence rate in growing children. In older children with severe contractures, a Judet quadricepsplasty, potentially combined with a femoral shortening osteotomy, may yield better results [41]. A notable advancement in this area is a combined surgical approach that includes peroneal nerve decompression, posterior knee release, and proximal femoral shortening. Studies have shown this novel strategy can increase the total arc of motion, significantly reduce flexion contracture, and improve ambulatory function [41]. For extension contractures without dislocation, the timing of surgical intervention is more controversial. Since these deformities typically don’t interfere with ambulation, many surgeons opt for a more conservative or delayed approach. [42]

Christine’s long-term study of the functional outcomes of knee contracture release surgery in arthrogryposis revealed a decline in patient-reported and functional mobility scores over an average of 11.9 years, despite initial improvements. This suggests that while surgery can offer short-term benefits, it may not provide a durable solution. The findings highlight the critical role of long-term support, often necessitating lightweight orthotics to maintain stability, mobility, and support during weight-bearing activities [43]. Similarly, other research on total joint replacement (TJR) in AMC patients shows mixed results. While TJR significantly reduces pain and improves joint-specific function, it does not consistently lead to improvements in a patient’s ambulatory status or overall walking ability [31].

Knee deformities – most commonly flexion or extension contractures – significantly impair mobility in children with AMC [44]. Treatment should begin neonatally, when tissues are most responsive. Mild-to-moderate flexion contractures (<60°) often respond to serial casting, hemiepiphysiodesis (eg, anterior 8-plating), or posterior soft-tissue releases. Severe contractures (>60°) typically require gradual correction using external fixation (eg, Ilizarov) with or without posterior release [11,13,42,45]. In skeletally mature patients, distal femoral extension osteotomy or quadricepsplasty with femoral shortening can improve arc of motion and function, but recurrence remains common [40]. Long-term use of orthotics is often necessary to maintain stability and function. Total joint replacement may reduce pain but does not improve ambulation [31]. Surgical intervention should be individualized, with attention to physeal status, joint congruence, and ambulatory potential. Recurrence is common, especially in growing children, and close monitoring is essential [11].

Foot Deformity and Management

In children with AMC, foot deformities are common and often challenging to manage. The most common deformities are rigid clubfoot (78–90%), followed by congenital vertical talus (CVT) (3–10%), with less common presentations including isolated equinus, cavovarus, or metatarsus adductus [45]. The primary objective of treatment is to achieve a plantigrade, pain-free, and functional foot that supports ambulation. Unlike typical clubfoot, the arthrogrypotic variant is exceptionally rigid and prone to recurrence, largely due to inherent muscular imbalances and continued growth [46]. This has led to a major shift in treatment philosophy over the past 2 decades. Modern management emphasizes starting treatment as early as possible to prevent adaptive contractures and bone deformities [47]. The current approach prioritizes conservative methods like casting, often followed by limited surgical interventions to achieve lasting correction [48].

For rigid clubfoot, the most common foot deformity in arthrogryposis, the Ponseti method – involving serial casting, manipulation, and percutaneous Achilles tenotomy – is now the recommended first-line treatment [49]. While this approach shows promising results, even in severe cases, it typically requires more casts and achieves less dorsiflexion compared to the treatment of idiopathic clubfoot [49]. A high initial correction rate (74%) has been reported with the Ponseti method for arthrogryposis-associated clubfoot [48]. However, outcomes differ significantly between subtypes, with patients with amyoplasia showing worse clinical and radiological results and requiring more major surgeries than those with distal arthrogryposis. Despite the initial success, a high recurrence rate remains a significant challenge. A long-term study with a mean follow-up of 8.9 years found that 74% of feet required at least one major surgery [48]. The primary surgical procedure is a comprehensive circumferential soft-tissue release. However, recurrence rates are high (up to 73%), often necessitating secondary procedures [50]. Tendon resection is generally preferred over lengthening. After correction, bracing and physical therapy are essential to maintain alignment and prevent recurrence, although the brace design may need to be adapted due to coexisting contractures [50]. Recent long-term studies, such as one involving 177 children with a mean age of 9.4 years, reinforce this complex picture. While the Ponseti method can improve foot alignment and reduce the need for surgery, the reoperation rate remains high (44%) in the arthrogrypotic group [51]. These patients continue to exhibit more functional limitations in motor function and global mobility compared to controls, highlighting the need for ongoing management and attention to this condition.

For recurrent or residual foot deformities in arthrogryposis, a conservative approach of serial casting and minimal intervention is preferred [52]. When soft-tissue procedures are necessary for moderate equinus or equinovarus, a posterior or posteromedial release can be performed. Due to muscle weakness, tendon transfers are rarely done, although a tibialis anterior transfer may be considered for persistent forefoot supination. In cases of more severe or complex deformity, osteotomy has been shown to significantly improve outcomes [50]. A recent study of ambulatory children with rigid clubfoot demonstrated that osteotomy led to significant improvements in ankle dorsiflexion, forefoot abduction, dynamic foot pressure, and overall mobility. Although a small number of patients required reoperation, the findings confirm the functional and biomechanical benefits of osteotomy in this challenging patient population [53].

For more severe or rigid foot deformities in arthrogryposis, osseous procedures are often necessary. One option is tarsal decancellation (Verebelyi-Ogston procedure), which corrects resistant clubfeet by collapsing bone cortices, but this can lead to long-term arthritis [54]. Alternatively, various osteotomies, such as a metatarsal or lateral closing wedge, or a navicular excision with cuboid wedge resection, can be used to correct complex deformities like cavus, adductus, or hindfoot varus in older children. Talectomy (surgical removal of the talus bone) is generally reserved as a salvage procedure for severe, neglected, or recurrent deformities. While this procedure can yield satisfactory outcomes, 82% of revision cases have recurrence and long-term arthritic changes [55–58]. A study of 20 patients who underwent talectomy showed that while the procedure improved patient satisfaction when a plantigrade foot was achieved, their overall quality of life scores remained significantly lower than those of controls, with footwear adaptation being a major challenge [59].

In patients with complex rigid foot deformities, surgical intervention is often necessary. A retrospective study of supramalleolar osteotomies in 18 children found that the procedure successfully achieved a plantigrade foot initially in all cases. Despite a 33% recurrence rate, requiring further surgery at an average of 5.7 years, this technique was considered a valuable salvage option with manageable complications [60]. For complex, severe, and recurrent deformities, particularly in older children, external fixation using devices like the Ilizarov or Taylor Spatial Frame allows for gradual, three-dimensional correction. This method can be used as a stand-alone procedure for soft-tissue distraction or in conjunction with soft-tissue releases or osteotomies [61]. For children aged 10 to 12 years with severe deformities, triple arthrodesis may also be a viable definitive option [62]. A comparative study of V and Y osteotomies using the Ilizarov method for complex foot deformities found that while both techniques achieved similar final foot lengths, the choice of technique depends on the specific deformity [63]. Overall, these advanced surgical methods provide effective solutions for challenging cases, even with the risk of recurrence.

Arthrogrypotic congenital vertical talus (CVT), which accounts for 3% to 10% of foot deformities, is far less common than arthrogrypotic clubfoot. CVT is typically associated with distal arthrogryposis or other syndromic forms of the condition and is rarely seen in amyoplasia. Treatment for CVT has evolved significantly, particularly with the advent of the Dobbs method. This approach involves a series of “reverse Ponseti” castings to stretch the foot into adduction, plantarflexion, and supination. Following casting, a limited open reduction of the talonavicular joint with percutaneous pinning is performed [64]. This minimally invasive technique, which is based on the Ponseti principles, is emerging as an effective alternative to more extensive soft-tissue releases [65]. While this minimally invasive method achieves significant initial correction, studies show a high recurrence rate, particularly in patients with arthrogryposis. One study found that recurrence occurred in 45% of arthrogryposis patients compared to 14.3% in other syndromic cases, underscoring the need for enhanced follow-up in this high-risk population [66].

In summary, foot deformities in AMC – primarily rigid clubfoot and CVT – require early, systematic, and individualized management aimed at achieving a plantigrade, functional, and pain-free foot [67]. Current evidence suggests that the Ponseti method is recommended as first-line treatment for clubfoot, but reported success rates indicate it is less effective in amyoplasia than in distal arthrogryposis, often requiring more casts and prolonged bracing to maintain correction [67,68]. Studies on talectomy demonstrated satisfactory short-term clinical outcomes when a plantigrade position is achieved, but patient-reported functional scores often remain below normative values over long-term follow-up [57]. External fixation and triple arthrodesis are reserved for severe cases or older children [63]. Because functional outcomes may remain suboptimal despite achieving anatomical correction, multidisciplinary management and family counseling are essential throughout treatment [32].

Multidisciplinary Rehabilitation Management

The management of AMC necessitates a comprehensive and lifelong multidisciplinary approach that extends well beyond surgical correction [69]. Although surgical interventions play a vital role in addressing structural deformities and improving joint alignment, they constitute only one element within an integrated treatment paradigm [12]. Continuous rehabilitation is the cornerstone of AMC management, aimed at maximizing functional independence throughout the patient’s life. The principal objectives include achieving and maintaining optimal active and passive range of motion and enhancing muscular strength [70]. By focusing on these fundamental physical attributes, rehabilitation facilitates the performance of activities of daily living and promotes overall quality of life [15]. The efficacy of this process is greatly augmented by early and individualized interventions, which may incorporate kinesitherapy, therapeutic massage, stretching regimens, and physical agents. Ultimately, this holistic framework is designed not merely to address isolated impairments, but to empower patients toward meaningful participation in societal and personal contexts [71]. Thus, the rehabilitation strategy emphasizes functional optimization across 3 interrelated levels: ameliorating limitations in body structures, enhancing activity performance, and fostering societal participation [12].

In the infant stage (0–18 months), rehabilitation efforts focus on early intervention to take advantage of high tissue plasticity [70]. The primary goals are to enhance both passive and active range of motion (ROM), correct fixed deformities using gentle manipulation, casting, and low-temperature orthotics, and promote anti-gravity movement patterns. Current clinical guidelines suggest that through consistent stretching and positioning strategies, combined with caregiver education, a structured home exercise program and orthotic use are implemented to optimize the window of opportunity for improving joint ROM and reducing contractures [71].

During the toddler stage (18 months to 4 years), the therapeutic emphasis shifts toward translating gains in range of motion and strength into functional independence and exploratory behavior [70,72]. Rehabilitation strategies include developmentally stimulating activities, strategic positioning, and strengthening exercises for the trunk and limbs – all tailored to the child’s individual capabilities. Evidence indicates that the use of assistive devices and compensatory strategies is essential for promoting autonomy at this stage [73]. This phase may also involve ongoing strengthening, possible surgical interventions for contractures, and the continued application of supportive orthotics and adaptive equipment such as gait trainers [73]. A central milestone during this period is the development of upright mobility, achieved through either assisted walking or adaptive floor locomotion, alongside the refinement of fine motor skills via manipulative play, which lays the foundation for future performance in activities of daily living.

For children aged 5–12 years, rehabilitation adopts a more episodic and goal-oriented approach, emphasizing the promotion of autonomy in both school and community environments [73]. Priorities in this age group include maintaining range of motion (ROM), implementing post-surgical protocols, and strengthening to support functional task performance [73]. Strengthening available muscle groups is encouraged to enhance active ROM, strength, mobility, stability, and overall health [74]. Key objectives consist of achieving independence in self-care, enabling full participation in educational activities through assistive technology and environmental adaptations, and developing safe and efficient mobility – whether through ambulation with orthoses or mobility aids, or through proficient use of a wheelchair for navigating varied terrains [44,72].

In the teenage and adult years (ages 13 and above), the focus of rehabilitation shifts toward maintaining functional abilities and integrating therapeutic exercise into a healthy lifestyle to support vocational, educational, and social engagement [75]. Therapeutic exercise remains essential for preserving range of motion, muscle strength, and weight management. Rehabilitation also expands to address instrumental activities of daily living necessary for independent living, utilizing problem-solving strategies, technical aids, and community-based resources [16]. The overarching goal is to maximize long-term participation and quality of life while minimizing reliance on formal therapeutic interventions [76].

The rehabilitation of individuals with AMC is a continuous and evolving process that adapts to the developmental needs and functional goals of each life stage [77]. Adhering to the consensus recommendations by Dahan-Oliel et al [12], the management of arthrogryposis multiplex congenita (AMC) centers on a personalized, multidisciplinary, and goal-oriented approach. This framework spans from early interventions leveraging neonatal plasticity to lifelong strategies for autonomy, establishing standardized, child- and family-centered practices that cover the spectrum from initial development to perioperative care. Available reports suggest rehabilitation aims not only to address physical impairments but also to empower individuals to achieve maximal functional independence and engagement in societal roles [78]. Future efforts should focus on establishing standardized outcome measures and expanding evidence-based practices to further optimize long-term quality of life for those living with AMC.

Conclusions

Arthrogryposis multiplex congenita encompasses a heterogeneous group of disorders characterized by congenital joint contractures, requiring lifelong, multidisciplinary management. Treatment strategies are highly individualized, focusing on functional improvement through early intervention, structured rehabilitation, and staged surgical procedures (Table 1). Key principles include the optimization of joint mobility and muscle strength, correction of deformities using conservative and surgical methods, and the promotion of independence across all life stages. Hip, knee, and foot deformities are addressed through a combination of soft-tissue releases, osteotomies, and occasionally salvage procedures, although recurrence remains common (Figure 1). Long-term outcomes emphasize maintained mobility and participation rather than anatomical perfection. Future efforts should prioritize standardized outcome measures and evidence-based protocols to enhance quality of life for individuals with AMC.

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