One of the primary methods of casting used for the treatment of arthrogryposis is the Ponseti Method, which is mainly used to treat a deformity known as clubfoot. The Ponseti Method gradually corrects the deformity by using serial casting and bracing to maintain the correction (Boehm, Limpaphayom, Alaee, Sinclair, & Dobbs, 2008). This is typically performed along with a percutaneous tenotomy, or tendon lengthening, of the Achilles tendon to correct ankle equines, a plantar-flexed position of the foot. Tenotomy is performed before the patient receives their last cast if they have less than 10° of dorsi-flexion (Boehm et al., 2008). After casting, braces are then prescribed to be worn full time, followed by a period of part-time use to maintain the correction. If initiated within the first few years of life, extensive surgical procedures may be avoided (Boehm et al., 2008). In a past study on treatment for distal arthrogryposis, the Ponseti Method was successful for 11 of the 12 patients involved. At the conclusion of their treatments, they measured with an average ankle dorsi-flexion of 15° bilaterally and no evidence of hind foot varus (Boehm et al., 2008). One remaining patient did require an extensive soft tissue release operation after a second relapse and at the time of the latest follow up presented with plantigrade feet, a neutral heel position, and 0° of ankle dorsi-flexion. This patient’s family reported continued noncompliance with brace wear that is used to maintain correction of club foot deformity. This specific patient was a prime example that noncompliance with brace wear is a critical factor leading to a possible clubfoot relapse following initial correction with the Ponseti Method (Boehm et al., 2008).

Knee flexion contractures, common joint contractures associated with arthrogryposis, can drastically diminish one’s ability to ambulate even short distances without assistance or an assistive device. Casting has shown improvement in knee flexion in young children, but it runs a risk of creating a posterior dislocation of the tibia on the femur. “The Quengel hinge can be used to avoid this problem” (Bernstein, 2002, p. 421). The Quengel cast involves applying a below-the-knee cast with a cast cuff around the thigh and a Quengel hinge incorporated in the cast. Thick padding is applied under the cast over bony pressure points. A ring nut on the hinge is turned one to four times a day to gradually extend the knee, usually to 20 to 30 degrees of flexion (Tepeneu et al., 2010). The rotational component of the Quengel hinge is placed to correspond to the rotational components of the knee, which are the femoral condyles (Bernstein, 2002). This method, as well as similar non-invasive methods of treatment, has shown to be generally successful in the treatment of mild contractures, and are often used as adjuncts after radical soft-tissue release (Tepeneu et al., 2010).

In addition to casting, which is used for correcting joint contractures, night splinting and bracing are methods used for maintaining these corrections. The main difference between casting and these alternatives are the removability of splints and braces. “Night splinting is the most effective method of preventing recurrent deformity” (Staheli, Hall, Jaffe, & Paholke , 1998, p. 34). Night splinting is very useful with children, as it does not interfere with daytime activities such as playing and socializing (Staheli et al., 1998). The most important aspect of night splinting is comfort. Uncomfortable splints will prevent the child from sleeping and will cause irritability of the skin. Ideal splints are usually made from fiberglass and lined with foam, although plaster and plastics are also deemed adequate. Splinting is utilized throughout early childhood, often lasting until at least the age of five, and should continue to be used as long as there is a significant chance of recurrence of the deformity (Staheli et al., 1998).

Bracing is also used for maintaining corrections. Braces are worn during the day in cases where they enhance effective mobility. This process begins with the use of long leg braces, which cover the entire leg to maintain proper joint positioning. Later, short leg braces are utilized, as less coverage is needed to maintain the correction. Eventually, the patient graduates out of braces entirely once the correction is determined to be permanent (Staheli et al., 1998).

When corrections cannot be achieved and maintained through casting and bracing, surgical options may become inevitable to achieve ambulation for even the most common activities of daily living such as transferring to the bathroom or standing to brush one’s teeth. Surgical options for the treatment of arthrogryposis are always a last resort, as these methods carry much greater risk than non-surgical options.

Clubfoot has been presented as one of the most common deformities associated with arthrogryposis. Clubfoot can also be one of the most disabling conditions if left untreated, as it can lead to severe deformities later in the maturation process. Mackenze (1958) found the following:

In general, the more rigid the deformity is at the start of treatment, the greater the tendency to relapse regardless of the treatment selected. This deformity, however, tends to progress with growth, and incorrect weight-bearing and contractures are a common occurrence. (p. 17)

According to multiple sources, treatment should be commenced as early as possible, with surgery being the last option due to the traumatic effects it can have on bone growth (Mackenze, 1958; Shapiro & Specht, 1993).

Talectomy method

Primary soft tissue release of the ankle

Although clubfoot deformity may be the most common deformity associated with arthrogryposis, contractures of the knees can present the most difficult problems for these patients. Knee contractures are classified as either flexion contractures or extension contractures, with flexion contractures being both the more common and the more disabling of the two. According to a study by Bernstein (2002), only 50% of patients with flexion contractures were able to become community walkers, meaning they were able to ambulate outside the home in minimally crowded public areas. However, every patient in this study suffering from extension contractures eventually became a community walker. Due to the problematic nature of this condition, and the high rate of recurrence, surgical options are practically a necessity in these cases.

As mentioned earlier, surgical options are a last resort when treating arthrogryposis. When all other options have been exhausted, soft-tissue release is the most common surgical procedure used for knee contractures in infants. Some of the methods of soft-tissue release used in order to obtain sufficient extension for ambulation are lengthening of the hamstrings, release of the gastrocnemius, release of the posterior cruciate ligament, and division of the posterior capsule (Bernstein, 2002; Shapiro, & Specht, 1993). Which procedure (or multiple procedures in some cases) is used is determined by the severity of the flexion contracture. Full correction is avoided during this procedure because it could result in a stretch injury, as the popliteal artery and nerve is usually tight (Bernstein, 2002). Hypertension is another concern with this procedure, making careful blood pressure monitoring highly recommended afterwards. If elevated blood pressure occurs, decreased extension of the knee will usually correct the problem. Once the blood pressure has normalized, gradual extension can be reinitiated. Following the procedure, casting and physical therapy rehabilitation are necessary to maintain the correction. Although soft-tissue release and gradual extension facilitated by casting may result in improved knee extension, permanent improvement of the motion arc is rare (Bernstein, 2002).

Soft-tissue release procedures performed for the correction of extension contractures have shown results that are more favorable. In these cases, anterior lengthening of the quadriceps was used followed by immediate and prolonged use of continuous passive motion. These procedures have shown an increase of flexion from 10 to 70 degrees, with maintenance of correction two years later (Shapiro & Specht, 1993). This procedure does come with the possibility of the patient needing the assistance of orthotic support, but the cases presented in one particular study reviewed by Shapiro and Specht (1993) were able to continue ambulation without braces.

Although soft-tissue release is the most common recommended surgical procedure for infants with knee contractures, extension osteotomy would be the preferred method of choice for skeletally mature individuals (Bernstein, 2002). If a knee flexion contracture of more than 15 to 20 degrees remains after alternative treatment options, an extension osteotomy may be indicated to obtain functional extension for ambulation. An extension osteotomy procedure involves removal of 1-1.5 cm of the distal femur as well as an anteriorly based wedge to allow relaxation of the posterior neurovascular structures. It is important to know that this procedure does not change the actual joint range motion, but rather realigns the axis of the joint to allow for more effective weight-bearing and braceable positioning (Shapiro & Specht, 1993). Unfortunately, this procedure carries a risk of neurovascular damage, which is a common risk factor associated with operative correction of knee flexion contractures (Shapiro & Specht, 1993). As is the case in any surgical correction for arthrogryposis, this procedure must be followed up with casting and physical therapy to maintain the correction.