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Factors Associated With Functional Mobility in 256 Children With Arthrogryposis Multiplex Congenita: A Multicentric
Ahlam Zidan1, Laurie Snider2, Emmanouil Rampakakis3
1School of Physical and Occupational Therapy, Faculty of Medicine and Health Sciences, McGill University, Montreal, Quebec, Canada; Department of clinical research, Shriners Hospitals for Children, Montreal, Quebec, Canada.
Insights
Functional mobility in children with Arthrogryposis Multiplex Congenita (AMC) is significantly impacted by joint involvement and socioeconomic factors. Targeted rehabilitation strategies are crucial for improving outcomes in this population.
Area of Science:
- Orthopedics and Rehabilitation Medicine
- Pediatric Musculoskeletal Disorders
- Functional Mobility Assessment
Background:
- Arthrogryposis Multiplex Congenita (AMC) is a rare condition characterized by multiple joint contractures, potentially affecting functional mobility.
- Limited research exists on the comprehensive functional mobility status and associated factors in children, adolescents, and young adults with AMC.
Purpose of the Study:
- To characterize functional mobility in a large cohort of individuals aged 5-21 years diagnosed with AMC.
- To identify clinical (e.g., joint involvement, AMC subtype) and environmental (e.g., socioeconomic) factors associated with functional mobility outcomes.
Main Methods:
- A multisite, cross-sectional study involving 256 participants with AMC across eight pediatric orthopedic hospitals.
- Functional mobility was assessed using the Functional Independence Measure for Children (WeeFIM) and the Gillette Functional Assessment Questionnaire (FAQ).
- Multivariable models analyzed associations between mobility outcomes and perinatal, clinical, and environmental factors.
Main Results:
- CNS/Syndromic and Amyoplasia AMC subtypes showed significantly lower WeeFIM scores and FAQ levels compared to Distal Arthrogryposis (DA).
- Increased joint involvement, particularly of the knees, and perinatal joint contractures were strongly associated with poorer mobility.
- Parental unemployment and higher surgical burden negatively impacted functional mobility outcomes.
Conclusions:
- This study represents the largest cohort to date investigating functional mobility in AMC.
- Identified clinical and socioeconomic factors provide valuable insights for developing personalized rehabilitation strategies.
- Tailored interventions can potentially enhance functional mobility and improve overall outcomes for individuals with AMC.
Objective:
To describe functional mobility among children, adolescents, and young adults with arthrogryposis multiplex congenita (AMC) and identify factors associated with mobility outcomes.
Design:
Multisite cross-sectional study.
Setting:
Eight orthopedic hospitals for children.
Participants:
A total of 256 individuals (N=256) aged 5-21 years with a confirmed clinical diagnosis of AMC were recruited between October 2019 and December 2022. AMC subtypes included amyoplasia (n=122), distal arthrogryposis (n=62), and Central Nervous System (CNS)/syndromic AMC (n=39).
Interventions:
Not applicable.
Main Outcome Measures:
Functional mobility was assessed using the mobility domain of the Functional Independence Measure for Children (WeeFIM) and the Gillette Functional Assessment Questionnaire (FAQ). Multivariable models were used to examine associations with perinatal and current clinical and environmental factors.
Results:
Children with CNS/syndromic and amyoplasia subtypes demonstrated significantly lower WeeFIM scores (coefficients [Exp(B)]=0.74 and 0.85, respectively; both P<.05) and reduced odds of higher FAQ levels (adjusted odds ratio [AOR]=0.16 and 0.18, respectively; both P<.001) compared with those with distal arthrogryposis. Greater joint involvement, particularly at the knees, was a strong negative factor. Each additional perinatal joint contracture was associated with a 3.1% reduction in WeeFIM scores (Exp(B)=0.97, P<.001) and an 8.2% decrease in the odds of higher FAQ levels (AOR=0.92, P<.05). Current knee involvement was associated with a 27.7% reduction in WeeFIM scores (Exp(B)=0.72, P<.001) and nearly 90% lower odds of higher FAQ levels (AOR=0.10, P<.001). Parental unemployment (AOR=0.44, P<.05) and higher musculoskeletal surgical burden (AOR=0.24, P<.001) were significantly associated with poorer mobility.
Conclusions:
This is the largest cohort to date examining functional mobility in AMC. Clinical and socioeconomic factors identified may guide tailored rehabilitation strategies to promote positive outcomes in children with AMC.
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