Quadricepsplasty in arthrogryposis (amyoplasia): Long-term follow-up
Orthopaedic Department, Santa Casa Medical School and Hospitals, Pavilhão 'Fernandinho Simonsen', São Paulo, Brazil.Journal of Pediatric Orthopaedics B (Impact Factor: 0.59). 06/2005; 14(3):219-24. DOI: 10.1097/01202412-200505000-00015
Eight patients with arthrogryposis multiplex congenita (amyoplasia type) (11 knees) with knee hyperextension deformity underwent quadricepsplasty and were analyzed during an average follow-up period of 11 years and 2 months. The results were clinically analyzed based on gait pattern, range of movement, and orthotic requirements. Joint congruency was evaluated by radiography according to the Leveuf Pais classification. A satisfactory result was the correction of the deformity, articular congruency, sufficient range of movement, adequate gait pattern and no need for orthosis. A satisfactory outcome occurred in five of the eight patients (eight knees). We considered an unsatisfactory result when any of these conditions occurred. Our experience demonstrated that the quadricepsplasty corrected the hyperextension deformity of the knee joint, improved function, gait pattern, and maintained the muscle power of the quadriceps.
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ABSTRACT: The management of the musculoskeletal problems associated with arthrogryposis can be challenging. In our opinion, these children are best managed by multidisciplinary teams that includes an experienced geneticist, an orthopaedic surgeon, a physical therapist, and a pediatric physiatrist. It should be recognized that a high percentage of these children might be able to achieve some measure of functional ambulatory potential, but that many will lose some of these skills as they get older. The need for surgery is high in these children, and often, several procedures will be necessary. A careful discussion of realistic goals with the family is important, as is in ongoing focus on skills necessary for maximising independence in adulthood.Journal of Pediatric Orthopaedics 07/2007; 27(5):594-600. DOI:10.1097/BPO.0b013e318070cc76 · 1.47 Impact Factor
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ABSTRACT: Knee contractures are difficult deformities to manage in arthrogryposis. There is little information regarding the long-term functional outcomes. Patients with a diagnosis of arthrogryposis who had knee releases performed at a single institution with at least 2 years of follow-up were identified retrospectively. Patients were called back prospectively for a clinical examination and administration of the Pediatric Outcomes Data Collection Instrument (PODCI), Pediatric Evaluation of Disability Inventory (PEDI), and the WeeFIM instruments. Functional mobility was quantified using the Functional Mobility Scale (FMS). Thirty-two patients were identified with a total of 50 knees. There were 45 flexion contractures and 5 extension contractures. Average length of follow-up was 11.9 years (range, 2.2-23.6 years). Amount of extension on final follow-up correlated with all final FMS scores (P < 0.02). The FMS demonstrated decreases in mobility as distance increased. Twenty-two of 32 patients completed functional outcomes measures. Pediatric Evaluation of Disability Inventory Mobility scores, Functional Independence Measure for Children (WeeFIM) Mobility, and WeeFIM Self-Care scores were decreased compared with norms, and Normative PODCI scores at final follow-up showed significant impairment in Upper Extremity Function, Transfers/Mobility, Sports/Physical Function, and Global Function Domains. When patients were subdivided by length of follow-up, patients showed decline in scores for all FMS distances; PEDI Mobility Domains; WeeFIM Self-Care and Mobility Domains; and Transfer/Mobility, Sports/Physical Function, and Global Function Domains, as length of follow-up increased. Whereas knee releases may improve function in the short term, function and outcomes decline as patients age. Patients with arthrogryposis demonstrated significant impairment in normative scores for Upper Extremity/Physical Function, Transfers/Mobility, Sports/Physical Function, and Global Function Domains. In addition, function as measured by the PODCI, WeeFIM, and PEDI showed decreased scores as length of follow-up increased. We strongly advise that when counseling parents on this surgical intervention, parents are made aware that ambulatory ability may improve short term but may decline as patients age and contractures recur. Therapeutic Level 4.Journal of Pediatric Orthopaedics 04/2008; 28(3):307-13. DOI:10.1097/BPO.0b013e3181653bde · 1.47 Impact Factor
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ABSTRACT: Amyoplasia (type 1), characterized by quadrimelic limb involvement, is one of the most common types of arthrogryposis. In children with hyperextension or extension contracture of the knee, subluxation or dislocation of the knee joint with an associated subluxation or dislocation of the patella is frequently seen. Patellar malpositioning may be secondary to congenital hypoplasia of the patellar groove of the femur and/or dysplasia of the patellofemoral joint. A contracted quadriceps tendon and illiotibial band, as seen in extension contractures of the knee in arthrogryposis, may also contribute to patella alta and lateral subluxation of the patella. The aim of our study is to determine the position of the patella in children with quadrimelic arthrogryposis and knee extension contracture. The inclusion criteria for this study were as follows: patients diagnosed with arthrogryposis multiplex congenita group 1, a knee extension contracture present from birth, follow-up from birth or early childhood, at least yearly physical examinations, and a knee ultrasonography or magnetic resonance imaging study performed. Patellar position was determined, and the cartilaginous femoral sulcus angle, as well as the osseous femoral sulcus angle, was measured. Clinically, none of patella in any of the patients could be palpated before surgery. The patella was displaced superiorly and laterally in 16/16 knees as seen on ultrasonography. Magnetic resonance imaging also showed the patella to be displaced superior and laterally. All patients in our series with extension contracture of the knee and type 1 arthrogryposis had a patella that was superior and lateral to the patellar groove. We found that quadricepsplasty and relocation of the patella improved knee flexion. All patients regained active knee extension in 6 months postsurgery. Level III-diagnostic.Journal of Pediatric Orthopaedics 06/2008; 28(4):466-70. DOI:10.1097/BPO.0b013e31816c4dd8 · 1.47 Impact Factor
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