Family and everyone else involved with the patient’s treatment should participate in a general management plan, with this plan involving intensive and aggressive physiotherapy, bracing, and surgical interventions. ^[39]  There are, however, no guidelines for the care of affected individuals.

Affected babies should be delivered in tertiary care centers.

No completely successful approach to treat arthrogryposis has been found. Goals include lower-limb alignment and establishment of stability for ambulation and upper-limb function for self-care. A literature review by Gagnon et al found that rehabilitation was the most frequently reported form of conservative treatment for arthrogryposis. ^[40]

Early, gentle manipulation soon after birth improves passive and active range of motion. This is especially true in the case of the inherited distal arthrogryposes, in which prolonged immobilization associated with casting may be undesirable. Late manipulation is of little value.

Early, vigorous physical therapy to stretch contractures is very important in improving joint motion and avoiding muscle atrophy. Patients with amyoplasia or distal arthrogryposis respond well to physical therapy, with excellent functional outcome. However, physical therapy may actually be harmful in patients with conditions of bony fusion such as diastrophic dysplasia, because it may lead to joint ankylosis. Recurrence of deformities following stretching is common, and surgery is often indicated.

Splinting combined with physical therapy appears preferable to continuous casting. Night splinting after surgical procedures is indicated to maintain increased range of motion.

Feeding assistance and intubation is needed in patients with severe trismus.

Using the Pirani scoring system to measure treatment progress, a retrospective study by Henstenburg et al indicated that the Ponseti serial casting method can improve clubfoot in individuals with arthrogryposis but that the amount of improvement decreases with each casting series. In the study's patients, the pretreatment versus the post-treatment Pirani scores differed in the first series by 3.12, while in the second and third series they differed by 1.50 and 1.05, respectively. The average patient age at first casting was 2.31 years. Final follow-up occurred an average of 27 months after the end of the third series, with the total mean study length being 71.0 months. ^[41]

Despite the promising results in the above report, a retrospective study by Schaibley et al of children up to age 18 years found a more than 80% recurrence rate in patients with arthrogrypotic clubfoot who underwent Ponseti serial casting. Analyzing patients with either idiopathic or arthrogrypotic clubfoot, the investigators reported that 93.4% and 72.2% of individuals, respectively, experienced successful correction through Ponseti casting and Achilles tenotomy. However, at average follow-up of 3.4 years for the idiopathic patients and 4.2 years for those with arthrogrypotic clubfoot, the recurrence rates were 44.6% and 83.3%, respectively. Patients in the study with arthrogryposis had either distal arthrogryposis or amyoplasia. ^[42]

The aforementioned literature review by Gagnon et al found that osteotomy was the most common type of surgery reported for arthrogryposis. ^[40]

The perioperative management of the arthrogrypotic patient can be difficult and is managed by an experienced anesthesia and medical team. ^{[43, 44, 9]} The following issues are noted:

• Difficulty in venous access

• Prone to develop intraoperative hyperthermia

• Certain drugs influenced by decreased muscle mass

• Postoperatively, increased difficulty with atelectasis and stridor with an increased risk of aspiration

• Vertebral instability secondary to decreased muscle mass and high cervical hypoplasia

If surgery is contemplated, early (age 3-12 mo) one-stage (bone and tendon transfer) surgery should be performed. Fine-tuning procedures, such as opponensplasty, may improve function at a later stage.

Specific joint problems should be addressed with regard to treatment of other joints and the goals for the patient. Soft-tissue surgery should be performed early, with osteotomies carried out when growth is completed. In soft-tissue release procedures, tenotomies should be accompanied by capsulotomies. Long-term bracing and assistive devices are usually needed.

An international questionnaire study by Sawatzky et al of adults with arthrogryposis found that participants had an average of 9.8 surgeries, at least half of which were carried out during childhood. The rate of foot, knee, and hip surgeries was 78%, 45%, and 31%, respectively. A beta regression model indicated that knee and/or shoulder operations correlated negatively with adult quality of life, as measured using the Short Form 36 Health Survey Questionnaire physical capacity score (PCS), while elbow surgery correlated positively with the PCS. ^[45]

Feet

The most common deformity is a rigid talipes equinovarus deformity. The goal of treatment is a plantigrade, braceable foot.

Casting in the first 3 months and attempting to stretch the skin often fail to correct the deformity. The patient eventually needs an extensive medial and lateral release, followed by prolonged casting and bracing.

Recurrence is common as the child grows, and the patient may need eventual treatment with bony procedures, such as lateral column shortenings (Lichtblau procedures) or talectomy.

In a skeletally mature deformed foot, triple arthrodesis often gives a satisfactory plantigrade foot. In an older child with no previous treatment, a combination of soft-tissue releases and bony procedures may be indicated.

Knees

The goal of treatment is an extended knee for ambulation. This is more easily accomplished in an extension or hyperextension deformity than in a flexion deformity.

Flexion knee deformities are more common than fixed knee deformities and are more resistant to treatment.

A mild contracture (< 20°) does not interfere with functional ambulation and can be treated with passive stretching and splinting.

Moderate contractures (20-60°) need soft-tissue releases, including posterior capsulotomy of the knee joint, followed by long-term bracing.

Severe contractures (>60°) may need femoral shortening, in addition to soft-tissue releases, to decrease tension on neurovascular structures behind the knee. In an older child with severe flexion deformity, a knee disarticulation may be indicated.

Extension deformities often present as recurvatum or even anterior dislocation, but they respond better to physical therapy than flexion deformities. Initial treatment for recurvatum is passive stretching and splinting. If unsuccessful, quadricepsplasty should be performed when the patient is younger than 6 months. The deformity should be corrected before treating a dislocated hip to facilitate hip reduction.

Hips

Hip surgery should follow foot and knee surgery, especially in the presence of knee extension deformities. Hip surgery should be performed when the patient is younger than 1 year to facilitate ambulation. In some patients with bilateral hip dislocations and extremely mobile hips, open reduction may be attempted.

Hip flexion contractures are more difficult to treat than dislocations. Mild hip flexion contracture is acceptable for ambulation. A flexion contracture greater than 35° requires soft-tissue releases.

A bilateral hip dislocation greater than 35° with flexion contracture should be treated with stretching and soft-tissue releases but not with reduction.

Unilateral hip dislocation requires reduction to avoid pelvic obliquity and scoliosis.

Upper extremities

Treatment involves development of self-help skills (eg, feeding and toileting) and mobility skills (eg, pushing out of chair and using crutches). In evaluating the upper extremities, overall function of the entire extremity should be considered rather than function of the individual joints.

Upper-extremity surgery should not be considered until the patient is older than 5-6 years. Shoulder function is usually satisfactory without a rotational osteotomy unless the shoulder is severely internally rotated.

Elbows

The goal of treatment is an elbow with passive or active flexion capability (feeding arm) and extension capability (toilet arm). Extension is corrected with either capsulotomy (ie, to allow passive flexion) or capsulotomy plus provision of a motor power, provided by a Steindler flexorplasty, a triceps transfer, or a pectoralis major transfer.

Because the elbow is crucial to hand function, the elbow must be mobile before a wrist deformity is corrected.

Wrists

The major wrist deformity is flexion with ulnar deviation. Treatment should begin with stretching and splinting.

A severe deformity requires proximal row carpectomy with or without fusion.

A trapezoid wedge excision improves dorsiflexion.

Fingers

Minimal to moderate flexion deformities require passive stretching and splinting.

More severe deformities require soft-tissue releases and often require proximal interphalangeal joint fusions.

Thumb-in-palm deformities need to be corrected to provide opposition-improved grasp.

Spine

The spine is affected in about one third of patients. Scoliosis begins early and progresses to become a long, severe, rigid, C-shaped curve. This curve responds poorly to orthoses because it is progressive.

Curves greater than 35° should be treated with spinal fusion and instrumentation.

See the list below:

• Anesthetist

• Clinical geneticist

• Orthopedic surgeon

• Plastic surgeon

• Radiologist

• Neurologist

• Developmental pediatrician

• Pathologist

• Psychologist

• Physical and occupational therapists

• Social worker

• Educator

• Orthotist

• Rehabilitation engineer

See the list below:

• No special diet is required.

See the list below:

• Physical activity may be limited because of existing orthopedic problems. As a group, patients appear to cope well socially, participating in social activities that correspond to their needs.

• Walking is more restricted in patients with flexion contractures of the lower extremities than in those with extension contractures. Flexion contractures of the hips severely impair walking ability.

• Contracture of the elbow can cause a significant degree of disability in hand function.

• The use of crutches can be impossible for patients with upper extremity involvement associated with severe spinal deformity.

• Patients with more severe joint involvement depend on more help from other people than those with less severe joint involvement.