Search

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]

Fibular hemimelia presents with foot deformity and leg length discrepancy. Previous classifications have focused on the degree of fibular deficiency rather than the type of foot deformity. Published methods of surgical reconstruction have often failed due to residual or recurrent foot deformity. The purpose of this report is to introduce new classification and reconstruction methods. The Paley SHORDT procedure is used to stabilize the ankle when there is a hypoplastic distal fibulawith a dynamic valgus deformity. It involves shortening and realignment of the distal tibia relative to the fibula. In contrast, the Paley SUPERankle procedure is used when there is a fixed equinovalgus foot deformity. The SUPERankle uses a supramalleolar shortening-realignment osteotomy and/or subtalar osteotomies with anlage resection. Due to the bony instead of soft tissue correction of deformity, residual or recurrent deformity is prevented. Weakening of gastro-soleus and peroneal muscles is avoided by shortening of the tibia instead of tendon lengthening. The limitation of ankle motion is related to ankle dysplasia rather than surgery or lengthening. A plantigrade-stable foot and ankle leads to an excellent functional result comparable or better than a Syme's amputation with prosthetic fitting. Serial lengthening procedures combined with the SHORDT or SUPERankle reconstruction lead to limb length equalization with a plantigrade, painless, functional foot. [ABSTRACT FROM AUTHOR]

Abstract: We present the first ever reported case of bilateral total knee replacements in a congenital amputee with bilateral fibular deficiency. A 60year old woman with bilateral fibular hemimelia presented with advanced osteoarthritis in both her knees for which bilateral total knee replacements was performed. The left knee replacement was followed up at 12months and the right knee at 7years. Oxford knee scores improved from 14 to 40 for the left knee and were 37 for the right knee. She was able to walk independently to a distance beyond 400 m. Modification in the surgical procedure and postoperative rehabilitation is discussed. Mid-term follow-up of 7years reaffirms total knee replacement as a viable option for below knee amputees with knee osteoarthritis. [Copyright &y& Elsevier]

Key Clinical Message: We describe a case of association of congenital bone abnormalities. It will allow clinicians to look systematically for other skeletal malformations and teach them how to evaluate these cases for the first time. This early screening will enable appropriate treatment to avoid complications and to preserve functional prognosis. [ABSTRACT FROM AUTHOR]

Objective Fibular hemimelia or longitudinal fibular deficiency is the most frequent congenital malformation of lower limbs. It causes sometimes major limbs shortening. Different treatments could be considered depending on the severity of the malformation: contralateral epiphysiodesis, corrective foot and knee osteotomies, leg-lengthening surgery with external fixation, prosthesis. For the most severe cases (15–20 cm bone length discrepancies or unstable knee or foot), leg-lengthening surgery with external fixation is not recommended. In both cases, there are two potential treatments: orthopedic treatment wearing orthoprosthesis or trans-tibial amputation surgery. Choice is complex and there is no clear objective recommendation for one or the other option. Some parents prefer amputation with removal of portions of the legs to prepare them for prosthetic use. This usually takes place during the first few years of their child. Others will let the child decide when they become teenagers or adults. What are the key factors that could help physical medicine and rehabilitation doctors to guide family in taking this tough decision? Observations We followed a 13-year-old boy suffering from longitudinal fibular deficiency before and after his treatment with trans-tibial amputation. We researched objective data on walk analysis based on a representative sample combined with a subjective study on quality of life using the VSP-A questionnaire. We collected data one month before the amputation and one month, four months and one year post-amputation. Discussion/Conclusion We could not clearly find out significant objective walk criterion before or after surgery. In both cases, we observed compensatory and adaptive mechanisms to anatomical asymmetry initially and acquired later on. Nevertheless, subjective questionnaires highlighted an improvement on a few parameters regarding quality of life after surgery and a major satisfaction from an aesthetic standpoint. This study should be carried out on a bigger scale with a more representative sample population. [ABSTRACT FROM AUTHOR]