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Longitudinal fibular deficiency (LFD) is the most common congenital long bone deficiency. This study aimed to objectively assess the physical performance of children and adolescents with LFD compared with unaffected peers, and to examine trends over age for subgroups of the LFD population. Differences between children with LFD and unaffected peers were examined with hand-held dynamometry for lower-limb muscle strength, Six-Minute Walk Test, Timed up and down stairs test, Star Excursion Balance Test, and Standing long jump. Thirty-nine children with LFD and 284 unaffected peers participated. Children with LFD performed at a lower level than their unaffected peers, on all measures of physical performance (mean 2.1 z-scores lower, all p < 0.01), except in long jump (p = 0.27). When comparing the performance of children with LFD to their unaffected peers across four age groups, there was a significant between-groups difference on all strength measures, and on the Six-Minute Walk distance, between children with and without LFD. These differences were smallest in young children (3–6 years) and largest in the older children (15–18 years) (all p < 0.01). Children with no lengthening surgery performed better on the Six-Minute Walk Test, covering a greater distance during the test, than those who had surgery (mean difference 83 metres, p < 0.01). There were no significant differences between children who had or had not undergone an amputation. Children with LFD performed at a significantly lower level than unaffected peers on all measures of physical performance other than jumping. The largest differences were in older children. This paper provides baseline functional data for future interventions in LFD. Cross-sectional study. This paper provides the first baseline functional data using validated objective measures on a consecutive cohort of children and adolescents with longitudinal fibular deficiency. Children with LFD performed significantly worse than their unaffected peers on all measures of physical performance other than jumping, with children falling further behind their peers as they age. Children who undergo an amputation typically have the most severe anatomical presentation and yet perform at an equivalent functional level. This paper identifies multiple modifiable impairments that represent potential opportunities for rehabilitation professionals to target with conservative treatment options to improve functional performance. [ABSTRACT FROM AUTHOR]

Background: Fibular hemimelia is the most common congenital long-bone deficiency. It is usually unilateral and results in a limb-length discrepancy. The literature generally subscribes to the concept of constant inhibition, a process by which limb-length ratios between the shorter and longer extremity remain constant throughout growth, but scientific data supporting this concept are sparse. Additionally, recent literature suggests that these children have abnormal skeletal maturation.Objective: To elucidate the lower-extremity long-bone growth patterns and skeletal maturation of children with unilateral fibular hemimelia.Materials and Methods: We reviewed medical records of children with unilateral fibular hemimelia seen at a large pediatric hospital over a 17-year period. Inclusion criteria were: at least two scanograms prior to any shortening/lengthening procedure, and no other congenital or acquired disorders. We collected the study cohort's femoral and tibial lengths (scanogram reports), plotted them against patient chronological ages and compared them to published growth standards. When these children's bone ages (Greulich and Pyle) were available, we plotted them against the children's chronological ages.Results: Twenty-three children were included (total=115 scanograms). At least 1 bone-age assessment was performed in 19 children (total=84 bone ages). All bone growth curves were within normal growth standards for the femur and tibia. Length ratios between shorter and longer limbs remained constant. Skeletal maturation was within two standard deviations of normal in 90% of bone ages.Conclusion: Lower-extremity long bones of children with unilateral fibular hemimelia have relatively normal growth curves, supporting and confirming the concept of constant inhibition. Most children show normal skeletal maturation. [ABSTRACT FROM AUTHOR]