Unique Lower Extremity Orthotic Interventions For Children With Arthrogryposis: A Single Case
Justina S. Shipley CO, MEd, FAAOP
Lampasi, Antonioli and Donzelli (2012) state that arthrogryposis is a heterogeneous disorder that is characterized by the congenital contractures of multiple joints. Subluxation, dislocation and soft-tissue contractures of the knee joint are common in patients with amyoplasia. Of the various forms of knee involvement flexion contractures are more common in addition to being more disabling then the extension contractures. These contractures also show substantial resistance to treatment and have a higher rate of occurrence. Arthrogryposis is part of the diagnosis for a large spectrum of congenital disorders characterized by multiple congenital contractures. [Image ]
In this case study, the patient has been diagnosed with arthrogryposis also referred to as amyoplasia. She presents with symmetrical limb involvement, some truncal sparing, above-average intelligence and a midfacial hemangioma on her forehead. She came to the hospital at the age of 5 untreated for any of her deformities or contractures. She had corrective surgery for her club feet and right knee flexion contracture. She still had residual valgus at the knee with some knee flexion and varus at the ankle on the right. She was initially fit with a traditional double upright knee ankle foot orthosis (KAFO) with a thermoplastic thigh cuff and ankle foot sections. The knee joint needed to be set in approximately 20 degrees of flexion to accommodate fixed deformity at the knee. The Ankle Foot (AFO) section of the device accommodated ankle varus and approximately 20 degrees of ankle plantar flexion. Due to the weight of the orthosis and the severity of the valgus deformity at the knee, alternative interventions needed to be considered.
The new orthosis was fabricated with no moving knee joint. The device was fabricated out of 3/8 copolymer with a single aluminum upright attached to the lateral side at the knee center extending approximately 3 inches proximal and distal to the knee joint. The valgus deformity of the knee was accommodated for through modifications to the plaster model. Extra p-lite padding in addition to a custom molded insert was added at the time of vacuum forming in the medial knee area and at the lateral aspect of the ankle to assist with skin integrity. [Image ]
The device was fit and delivered with four straps and felt pads to keep the orthosis donned. It had two Velcro straps at the thigh, proximal to the knee, a calf strap at distal to the knee and a figure 8 strap at the ankle. The orthosis was then with inside of a sneaker provided by the family. In addition to the modified nontraditional KAFO, the patient also wore an ankle foot orthosis on the contralateral side with a shoe lift of 1.5 cm. After fitting of the new KAFO, she was able to ambulate more efficiently and she also had less incidence of skin breakdown. [Image ]
Arthrogryposis encompasses a vast variety of diseases; they range from being mildly involved to the severely impaired. Recommendations for treatment are based on individual practitioner experience; there is limited research on the outcomes of the treatment for these deformities with orthotic intervention (Amor, Spaeth, Chafey & Gogola, 2011). The purpose of this case study was to validate the use of unique interventions custom designed for an individual patient based on her specific needs. The follow-up analysis performed by a motion analysis shows improvement in the aspects of ambulation of the patient while wearing the orthosis vs. no orthosis. The following tables show percentages that are closer to those listed as normal with the orthosis wear on the right side. [Table , graph ]
The management of musculoskeletal problems associated with arthrogryposis presents many challenges. Pediatric patients are best managed by a comprehensive care team that includes an orthopedic surgeon, physical therapist, orthotist, a pediatric geneticist and a physiatrist. There are a high percentage of these children that will be able to achieve some measure of functional ambulation with a treatment plan of surgical intervention and unique orthotic devices that are custom designed for each individual (Bevan, Hall, Bamshad, Staheli, Jaffe & Song, 2007). [Image ]
The FDA has cleared all pharmaceuticals and/or medical devices for the use described in this presentation.
Justina S. Shipley, CO, MEd, FAAOP: 3C (Comfort Products; Fillauer Companies); 9 (Louisiana Association of Orthotists and Prosthetists; Orthotics and Prosthetics Activities Foundation) All aspects of this device were financed by the Shriners Hospital for Children in Shreveport, Louisiana
Justina Shipley is affiliated with Shriners Hospitals for Children, Shreveport, Louisiana USA
- Amor, C. J., Spaeth, M. C., Chafey, D. H., & Gogola, G. R. (2011). Use of the pediatric outcomes data collection instrument to evaluate functional outcomes in arthrogryposis. Journal Pediatric Orthopedic, 31(3), 293-296.
- Bevan, W. P., Hall, J. G., Bamshad, M., Staheli, L. T., Jaffe, K. M., Song, K. (2007). Arthrogryposis multiplex congenital (amyoplasia): An orthopaedic perspective. Journal Pediatric Orthopedics, 27(5), 594-600.
- Lampasi, M., Antonioli, D. & Donzelli, O. (2012). Management of knee deformities in children with arthrogryposis. Musculoskeletal Surgery, 96(3), 161-169.