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.
Article: Arthrogryposis multiplex congenita[Show abstract] [Hide abstract]
ABSTRACT: From 1975 to 2004 a total of 38 children handicapped by congenital multiple arthrogryposis were cared for. The congenital joint contractures demand a major effort in terms of surgical reconstruction. In the case of distal arthrogryposis the chances that patients will be able to walk without help are good, while those with amyoplasia are likely to be dependent on mobility aids throughout their lives. The ultimate goal of treatment for patients is to develop into self-confident adults who can cope with life despite their handicaps. The hip in arthrogryposis shows variable forms of pathology, ranging from the almost normal hip to hip contractures with dislocation. Its treatment has some limited advantages, but hardly improves mobility. The knee contractures are actively treated to allow patients to sit, stand and walk better. The club foot and the rocker-bottom foot need sophisticated conservative and operative treatments. If conservative manipulation of bilateral extension contractures of the elbow fails operative treatment is carried out on the dominant side. For shoulder, hand and finger contractures conservative manipulation brings about little improvement, and surgical approaches help hardly at all.Der Orthopäde 04/2007; 36(3):281-90; quiz 291. DOI:10.1007/s00132-007-1044-0 · 0.36 Impact Factor
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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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