Comparison of Outcomes by Reconstructive Strategy in Patients with Prostheses for Proximal Femoral Focal Deficiency.

BACKGROUND: The present study compares prosthetic treatment options for proximal femoral focal deficiency in terms of gait analysis, oxygen consumption, and patient-reported outcomes. METHODS: Twenty-three patients who had been managed with a prosthesis for unilateral proximal femoral focal deficiency underwent gait analysis; this group included 7 patients who had received an equinus prosthesis, 6 who had received a rotationplasty prosthesis, and 10 who had undergone Syme amputation and had received an above-the-knee prosthesis. Cadence parameters, kinematic and kinetic data, and oxygen consumption were measured, and the Gait Deviation Index (GDI) was calculated. Medical records and radiographs were reviewed. The Pediatric Outcomes Data Collection Instrument (PODCI) was completed by the child's parent. RESULTS: Patients underwent gait analysis at a mean age of 11.6 years (range, 4 to 19 years). Proximal femoral focal deficiency classification was not predictive of the chosen treatment. Patients in the rotationplasty group had undergone more procedures than those in the Syme amputation and equinus groups (mean, 3.3, 1.8, and 0.7 procedures, respectively) (p = 0.001). Oxygen cost did not differ between groups; however, all required greater energy expenditure than normal (170%, 144%, and 159%, in the equinus, rotationplasty, and Syme amputation groups, respectively) (p = 0.427). Likewise, hip power, abductor impulse, and GDI did not differ, but all groups had GDI scores >3 standard deviations below normative values. Patients in the equinus group walked faster (97% of normal for age) than those in the rotationplasty (84%) and Syme amputation groups (83%) (p = 0.018), whereas those in the Syme amputation group had superior knee range of motion (55° from the prosthetic knee) than those in the equinus (20°) and rotationplasty groups (15° generated from the ankle) (p = 0.003). There were no differences in terms of the PODCI subscales for pain, sport/physical function, happiness, or global function. Transfer/basic mobility improved with age (r = 0.516, p = 0.017), but no other associations were found between gait variables and PODCI scores. CONCLUSIONS: Rotationplasty provided no patient-reported benefit and no functional benefit in terms of gait parameters or oxygen consumption, despite requiring more surgical procedures compared with other prosthetic options. Patients with an equinus prosthesis walked the fastest, whereas treatment with a Syme amputation and prosthetic knee yielded equivalent gait parameters and oxygen consumption as compared with those for patients using an equinus prosthesis. These findings contradict those of previous reports that rotationplasty provides superior function over other proximal femoral focal deficiency prosthetic treatment options. LEVEL OF EVIDENCE: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Problemas rotacionales de las extremidades inferiores en niños y adolescentes / Rotational problems of lower limbs in children and adolescents

Las deformidades torsionales de las extremidades inferiores corresponden a una de las causas de visita más frecuente al ortopedista infantil. En la mayoría de los casos, estas consultas son innecesarias, pues se trata de condiciones normales del esqueleto en desarrollo, que suelen corregir espontáneamente. El médico no especialista debe estar familiarizado con los cambios fisiológicos que ocurren en las extremidades inferiores del niño, de manera de identificar aquellos casos severos o aquellos que producen alteraciones funcionales.Estas deformidades, pueden ocurrir en cualquier nivel, desde la pelvis a los pies, por lo que un examen físico sistemático permitirá identificar la causa y proponer un tratamiento de acuerdo a los hallazgos. El único tratamiento eficaz, cuando la situación lo requiere, es la cirugía ortopédica. El uso de plantillas, calzado ortopédico y órtesis no cumplen ninguna función en el manejo de estas alteraciones

Torsional deformities of lower limbs are one of the most frequent causes to visit a pediatric orthopedic surgeon. In most cases, these are unnecessary, as they represent a normal stage of the developing skeleton, which usually correct spontaneously. The nonspecialist physician must be familiar with the physiological changes that occur in children's lower limb, in order to identify those cases that are severe, or cause functional disabilities.These deformities can occur at any level, from the pelvis to the feet, so a systematic physical examination will allow the cause to be identified and treated to be proposed according on to the findings. The only effective treatment, when the situation requires it, is orthopedic surgery. The use of insoles, orthopedic footwear, and orthoses have no role in the management of these alterations

Comparative study of the use of Paediatric Quality Of Life Inventory 4.0 generic core scales in paediatric patients with spine and limb pathologies.

AIMS: The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients' HRQoL. METHODS: Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples t-test, and correlation tests of PedsQL and SRS-22r. RESULTS: A total of 566 paediatric patients were recruited: 357 (63.0%) having idiopathic scoliosis, and 209 (37.0%) with limb conditions. Patients with limb pathology had lower functioning scale, summary, and total scores of PedsQL than scoliosis patients (p < 0.05 to p < 0.001). No floor or ceiling effects (< 15%) were detected for PedsQL Psychosocial Health Summary and total scores in both groups. PedsQL was sensitive in differentiating patients with/without problems in their daily lives (p < 0.05 to p < 0.01). PedsQL summary and total scores correlated with SRS-22r total score. CONCLUSION: PedsQL is an effective HRQoL measure for both paediatric orthopaedic groups with minimal ceiling and floor effects, and is capable of detecting worse HRQoL in patients with limb pathology. The multidimensional PedsQL is sensitive in differentiating among those with daily life problems, especially for scoliosis patients. Cite this article: Bone Joint J 2020;102-B(7):890-898.

Deformity progression in congenital posteromedial bowing of the tibia: a report of 44 cases.

BACKGROUND: congenital posteromedial bowing of tibia (CPMBT) is a very rare birth defect, characterized by shortened bowed leg and ankle deformity. We described a single institution experience in the management of CPMBT. METHODS: we identified 44 CPMBT in 44 children. The age at presentation was 5.5 ± 5.6 years and the mean age at the final review was 10.1 ± 4.8 years. Radiographic evaluation included the antero-posterior and lateral inter-physeal angle (AP-IPA and L-IPA), the limb length discrepancy (LLD), the morphology of the distal tibia and the lateral distal tibial angle (LDTA). During the study period, 26 children underwent surgical treatment. RESULTS: the estimated curves showed a progressive spontaneous correction of both AP-IPA and L-IPA during growth, but a progressive increase of the LLD. The L-IPA showed a more predictable behaviour while the AP-IPA showed a scattered correction, with a wider variation of the estimated final angle. The final LDTA was 85.3° ± 4.2° and was correlated with the L-IPA (r = 0.5; p = 0.02). Among the 26 children who underwent surgical treatment, 23 cases had limb lengthening, 1 case had contralateral epiphysiodesis, 1 child underwent tibial osteotomy, 1 patient was treated by hemiepiphysiodesis of the distal tibia to correct ankle valgus deformity. CONCLUSIONS: our study described the largest case series of CPMBT. A combination of surgical treatments, in a staged surgical process, should be tailored to the developmental characteristics of this abnormality. An experience-based algorithm of treatment is also proposed. Further studies are needed to understand which is the best strategy to correct this deformity during childhood. LEVEL OF EVIDENCE: level IV prognostic study.

Predicting Late Follow-up and Understanding Its Consequences in Growth Modulation for Pediatric Lower Limb Deformities.

BACKGROUND: Growth modulation with implants facilitates correction of angular deformities and limb-length discrepancies (LLDs) in children. Close follow-up is necessary when using growth modulation to prevent overcorrection. We examined factors associated with late follow-up and overcorrection rates in patients with late versus timely follow-up. METHODS: This was a retrospective review of growth modulation procedures in children at 1 institution from 2000 through 2014. Procedures were assigned to the following categories on the basis of deformity: ankle valgus, genu valgum, genu varum, knee flexion contractures, and LLDs. Radiographic and clinical parameters were assessed. Late follow-up was defined as delaying a recommended appointment by ≥6 months. Loss to follow-up was defined as failure to return for a recommended postoperative appointment. Associations were evaluated using the following tests: χ, Fisher exact, analysis of variance, Mann-Whitney U, and logistic regression. Statistical significance was set at P<0.05. RESULTS: Of the 112 patients, there were 41 cases of genu valgum, 23 of ankle valgus, 18 each of genu varum and LLD, and 12 of knee flexion contractures. Twenty-two patients had late follow-up. Another 22 patients were lost to follow-up with retained implants. Patients with late follow-up had significantly higher odds of experiencing overcorrection deformities versus patients with timely follow-up (odds ratio, 19.2; 95% confidence interval, 5.2-71.4; P<0.005). The only deformity for which there was a significant difference in final alignment between patients with timely versus late follow-up was genu valgum (P<0.005). Late follow-up was associated with having a primary language other than English (P=0.05) and being obese/overweight (P=0.004). CONCLUSIONS: Late follow-up and loss to follow-up were common, occurring in 39% of patients combined. Late follow-up was associated with overcorrection in guided-growth procedures, as were overweight/obesity and primary language other than English. LEVEL OF EVIDENCE: Level IV-retrospective case series.

The syndrome of proximal femur, fibula, and midline metatarsal long bone deficiencies.

Human lower limb congenital long bone deficiencies cluster primarily at three distinct skeletal locations. Proximal femoral and fibular reductions are known phenomena. In contrast, midline metatarsal deficiencies have been misrepresented as lateral. The popular term, "fibular hemimelia," is inaccurate and its use is discouraged. All three locations correspond to discrete sites of evolving angiogenesis during transition from a single embryonic axial limb artery to the familiar and complex adult arterial pattern. Initiation of bone formation of cartilaginous primordia of the long bones at all three sites occurs in proximity to, and depends upon, successful invasion by mature nutrient vessels, formed during the 6th and 7th weeks of embryonic development. The adult arterial pattern is fully established by 8th embryonic week. Arterial transitions occur later in development, around the time of cessation of the molecular processes of patterning/specification of the embryonic limb. Evidence of flawed embryonic arterial transitions, involving missing, reduced and/or retained primitive vessels in association with congenital skeletal reductions have been demonstrated at all three sites. Current molecular models of limb development do not explain the distribution of this triad of congenital skeletal reductions. These dysmorphologies are most accurately described as post-specification errors of limb development. Recognition of this distinctive model of limb maldevelopment demands further investigation to create a more exact taxonomy, one consistent with both clinical and molecular criteria. The established terminologies originated by Frantz and O'Rahilly should be reconsidered or abandoned. Designation of this clinical triad as a syndrome of proximal femur, fibula, and midline metatarsal dystrophisms initiates that endeavor.

Dynamic Lower Extremity Deformity in Children With Pseudoachondroplasia.

BACKGROUND: Pseudoachondroplasia is a diverse group of skeletal dysplasias with a common pathway of altered cartilage oligomeric matrix protein (COMP) production. This rhizomelic dwarfism is commonly associated with deformities of the lower extremities, accelerated osteoarthritis, and ligamentous laxity. One of the most common alignment problems is coronal knee angulation which combined with tibial torsion, results in a complex deformity. The outcome of surgical correction of these deformities is variable. METHODS: This study used 3-dimensional gait analysis to describe the kinematic deformities in 12 children (aged 3 to 15 y) and compared them to the static deformities measured on standing anteroposterior radiograph. RESULTS: Both gait analysis and radiographs showed large variability in the coronal deformities but strong correlation to each other. Gait analysis showed mean varus alignment of the knee to be 13.5±13.1 degrees; that mean is not statistically different from radiographs, which showed a mean varus of 16.2±17.1 degrees. The correlation coefficient between radiographic and kinematic measurement was 0.70. The kinematic internal tibial torsion measured an average 15±19 degrees, which was moderately correlated to knee varus (r=0.45, P<0.01). CONCLUSIONS: Measurements of varus-valgus alignment correlated well between gait analysis and radiographs. Tibial torsion correlated with varus. In the absence of gait analysis, anteroposterior standing leg length radiographs with the patella facing foreward can be used to assess deformity. As this study does not correlate these measurements to postoperative results, an appropriately powered prospective study and further investigation of biological effects of altered cartilage oligomeric matrix protein production are needed to explain the variable surgical outcomes. LEVEL OF EVIDENCE: Level IV-case series without control group).

Intramedullary magnetically actuated limb lengthening in a patient with congenital humeral limb shortening.

The treatment of leg length discrepancy has become a common procedure in orthopaedic surgery. However, lengthening of humeral deformities is still infrequent. The purpose of this case report was to present humeral lengthening with a new intramedullary lengthening device (PRECICE® P2 for tibia) in a 32 year old female patient with congenital shortening. Hereby the telescopic device presents a promising tool for humeral limb lengthening with excellent outcome at short-term in this case.

Left Ventricular Non-compaction in Holt-Oram Syndrome.

Holt-Oram Syndrome is an autosomal dominant condition with complete penetrance and which involves upper limb skeletal and cardiac abnormalities. The latter can be structural defects or involve the conduction system. This report details the occurrence of left ventricular non-compaction in multiple family members with Holt-Oram Syndrome. It is recommended that patients with the Holt-Oram Syndrome be considered for comprehensive cardiac evaluation to exclude non-compaction cardiomyopathy as this may have significant prognostic implications.

The Tibial Slope in Patients With Achondroplasia: Its Characterization and Possible Role in Genu Recurvatum Development.

BACKGROUND: Genu recurvatum, a posterior resting position of the knee, is a common lower extremity deformity in patients with achondroplasia and has been thought to be secondary to ligamentous laxity. To the best of our knowledge, the role of the tibial slope has not been investigated, and no studies describe the tibial slope in patients with achondroplasia. Our goals were to characterize the tibial slope in children and adults with achondroplasia, explore its possible role in the development of genu recurvatum, and compare the tibial slope in patients with achondroplasia to that in the general population. METHODS: We reviewed 252 lateral knee radiographs of 130 patients with achondroplasia seen at our clinic from November 2007 through September 2013. Patients were excluded if they had previous lower extremity surgery or radiographs with extreme rotation. We analyzed patient demographics and, on all radiographs, the tibial slope. We then compared the mean tibial slope to norms in the literature. Tibial slopes >90 degrees had an anterior tibial slope and received a positive prefix. Statistical analysis included intraclass and interclass reliability, Pearson correlation coefficient, and the Student t tests (significance, P<0.05). RESULTS: The overall mean tibial slope for the 252 knees was +1.32±7 degrees, which was significantly more anterior than the normal slopes reported in the literature for adults (7.2 to 10.7 degrees, P=0.0001) and children (10 to 11 degrees, P=0.0001). The Pearson correlation coefficient for mean tibial slope and age showed negative correlations of -0.4011 and -0.4335 for left and right knees, respectively. This anterior tibial slope produces proximal and posterior vector force components, which may shift the knee posteriorly in weightbearing. CONCLUSIONS: The mean tibial slope is significantly more anterior in patients with achondroplasia than in the general population; however, this difference diminishes as patients' age. An anterior tibial slope may predispose to a more posterior resting knee position, also known as genu recurvatum. LEVEL OF EVIDENCE: Level IV-retrospective case series.

[Blood Flow and Regional Blood Flow Rate in the Middle Cerebral Artery during Surgical Leg Lengthening in Patients with Congenital and Acquired Limb Shortening].

This is a comparative study of changes in blood flow rate in the popliteal artery, the arteries of bone regenerate and cerebral arteries in 45 patients with congenital and acquired diseases of the limbs at different stages of surgical lengthening of 3-15 cm shortened shin by Ilizarov method. We observed an increase in regional blood flow rate in all patients during the periods of distraction and fixation. A 25% increase in blood flow rate in the middle cerebral artery on the contralateral side was found only in patients of the first adult age with acquired limb shortening. Basing on the analysis of the reaction of cerebral arteries during a functional test with additional muscle work, we suggested that the absence of reaction in congenital diseases is caused by relative excess of somatic afferentation which results from morphological and functional immaturity of brain regulatory systems.

¿Tienen los niños futbolistas más varo de rodillas? / Do soccer players have more genu varum in their legs?

Introducción: Las deformidades angulares de las extremidades inferiores durante la infancia son un motivo frecuente de consulta en pediatría. Sin embargo, poco se sabe acerca de la relación entre la práctica deportiva y las adaptaciones estructurales durante la etapa de crecimiento. Objetivo: Describir las diferencias en el alineamiento de las extremidades inferiores (genu varo v/s genu valgo) entre un grupo de niños controles sanos y un grupo de futbolistas en formación. Material y método: Consistente en un estudio tipo observacional transversal. Se examinó un total de 206 niños entre 8 y 15 años de edad, de sexo masculino, 103 corresponden a controles sanos no deportistas de un servicio pediátrico de Santiago de Chile y 103 a jugadores seleccionados de fútbol del Club Deportivo Universidad Católica. Se evaluó la distancia intermaleolar, intercondílea y el ángulo femorotibial. Resultados: El ángulo femorotibial en la población control tuvo una media de 7,8°, mientras que en los futbolistas se constató una media de 3,49°. La distancia intercondílea en el grupo control tuvo una media de 0,07 cm, en cambio, en los futbolistas fue de 1,06 cm. Por último, la distancia intermaleolar en controles fue de 6,01 cm y en los futbolistas 0,77 cm. Todas con un p < 0,005. Estas diferencias son estadísticamente significativas para ángulo femorotibial y las distancias intercondílea e intermaleolar. Discusión: Se observa una tendencia a mayor varo de rodillas en los niños que practican fútbol, comparado con los no deportistas. Las siguientes líneas de investigación pueden estar orientadas a correlacionar estas variaciones anatómicas con riesgo de lesionabilidad, particularmente en la práctica deportiva. De esta manera, se podría planificar medidas preventivas en esta población objetivo (AU)

Introduction: Angular deformities of the lower extremities during childhood are a frequent reason for consultation in pediatrics. Is known that the soccer players have tendency to genu varus legs compared with general population. Is poor known about the relationship between soccer and structural adaptations during the growth stage. Objective: to describe the differences in lower tips alignment between a control group of healthy children and a group of soccer players. Material and method: is a transversal observational study, 206 male children between 8 to 15 years old were examinated. 103 are from the control group ( no athletes children from a pediatric care center) and 103 are from the case group ( athletes children from Universidad Católica Football Club. Intermalleolar distance, intercondylar distance and femorotibial angle were evaluated. Results: Femorotibial angle in control group was 7,8º on average, while in soccer player group was 3,49º on average. Intercondylar distance in control group was 0,07 centimeters on average, while in soccer player group was 1,06 centimeters on average. Finally, intermalleolar distance in control group was 6,01 centimeters on average and soccer player group was 0,77 centimeters on average. All results with a p >0,005. These differences found in both group are statistically significants for all measurements. Discussion: A tendency to genu varum was observed in soccer player group compared with control group of healthy children. The following investigation may be oriented to correlate these anatomical variations with risk of injury, particularly, in the sporting context. In this way, some medical measures could be planned to reduce the risk of skeletal injuries (AU)

Wrestlers with Limb Deficiencies: A Descriptive Study.

OBJECTIVE: The objective of this study was to determine if wrestling is a safe, positive athletic option for limb-deficient individuals. DESIGN: This descriptive study consisted of an opportunity sample of limb-deficient wrestlers, aged 5 yrs and older with at least 1 yr of experience. Participants completed a questionnaire regarding health, satisfaction, and achievements. Descriptive statistics were used for analysis. RESULTS: Sixteen male wrestlers reported nine below-the-knee, five above-the-knee, and three below-the-elbow limb deficiencies. There were nine congenital deficiencies and seven amputations acquired during childhood. Two individuals won National Collegiate Athletic Association championships, and seven competed collegiately. All reported a positive impact on quality-of-life, 87% reported no difficulty finding acceptance with the team, and 50% experienced wrestling-related residual limb complications. Associations between (1) residual limb complications before and during wrestling and (2) skin breakdown before and during wrestling did not demonstrate statistical significance (P = 0.30 and 0.1189, respectively). CONCLUSIONS: This study suggests that wrestling is a safe, positive sport for limb-deficient individuals, that it fosters competitive equality between impaired and nonimpaired participants, and that it has a positive impact on health and quality-of-life. The incidence of residual limb complications warrants monitoring.

Secondary nerve lengthening to obtain full knee extension in popliteal pterygium syndrome.

Microsurgical nerve lengthening was performed in two siblings presenting a popliteal pterigium syndrome with a knee flexion contracture of 80 degrees. After the first attempt for nerve lengthening and knee extension elsewhere, a repeated lengthening was required due to continuing tip-toe walking and recurrent knee contracture at the age of 3 years. An extensive external and internal interfascicular microsurgical neurolysis resulted in a lengthening of the nerves. A full length of leg procedure had to be performed, inclusive of Achilles tendon lengthening to obtain a complete extension of the knee and a 90-degree ankle flexion. Maintaining the leg in a fully extended position was obtained with a dynamic splinting in the first month after the operation. When timing the operation we have to consider the importance of adequate precision of the microsurgical neurolysis, down to the identification of the Fontana bands, and the adequate postoperative splinting.

Results and biomechanical consideration of treatment of congenital lower limb shortening and deformity using the Ilizarov method.

One of the applications of the Ilizarov apparatus is the correction of congenital shortening and deformities. Ilizarov external fixator produces biomechanical structure with surrounding tissue, which is the reason why very important is correct stability of fixator. Large distraction in the case of high value of lengthening, and large deformity corection result in shear stresses that occur additionally in the regenerate, which can potentially lead to damage of the regenerating nutritive microcirculation of bone tissue and bone fragment displacements. Our objective was to assess the results of the Ilizarov method in the treatment of congenital shortening taking into account treatment strategy and the size of the axis of lengthening and correction. Our research problems include presenting the effects of biomechanics of musculoskeletal deformations on treatment results, presenting complications and their treatment. Between 1989 and 2009, 62 patients underwent surgery to correct congenital lower limb deficiencies at our Clinic; 33 patients were followed-up. In total, there were 70 surgeries (2.12/patient). Axial correction was performed in 26 patients (78.79%). Average age at the start of the treatment was 15.58 years. Mean follow-up was 8.58 years. Mean lengthening per surgery was 3.17 cm with the lengthening index of 50.7 day/cm. Results were very good for 23 patients, good for 7 patients, satisfactory for 3 patients. Complications appeared in 24 patients, problems occurred in 74.42% of the cases, obstacles in 4.65% of cases, and true complications in 20.93% of the cases. The best results were achieved in the treatment of patients with two-stage and two-segment lengthening with a total elongation of less than 7 cm, and without correction of the axis. Congenital shortening of the lower limb should be treated comprehensively because the shortening applies to all segments, and disturbs biomechanics of all lower limb. In the case of axial correction and large amount of elongation high soft tissue forces counteract the distraction forces. Hybrid construction may help to shorten treatment time, increase fixator stability and decrease rate of complications. We suggest use of hybrid Ilizarov fixator, especially when large elongation and axis corection are planned.

The biomechanics of guided growth: does screw size, plate size, or screw configuration matter?

Guided growth with the eight-plate is a commonly used technique to correct angular limb deformities in children. However, the optimal combination of plate size, screw size, and screw configuration has not been determined. Using osteotomized femoral sawbones and a rail frame, we developed a growth model to examine the effect of these variables at 6-month, 12-month, and 18-month growth increments. The mean annual coronal plane change was 11.3°. Screw size and plate size were not associated with the rate of angular correction. Screw configuration was important, with parallel screws resulting in optimal correction at all time points compared with divergent screws (P<0.05).

Regeneration of a discoid meniscus after arthroscopic saucerization.

We report the case of a 5-year-old girl who presented to the clinic with recurrent knee pain after meniscal repair and saucerization at age 3. The finding of a regenerated discoid meniscus was confirmed arthroscopically and radiographically. To our knowledge, this is the first report in the literature to confirm regeneration of a discoid meniscus after saucerization. The case has significant implications for management of pediatric discoid meniscus tears and patient counseling, and is encouraging in terms of the potential for repair and regeneration of meniscal tissue, particularly in the very young pediatric population.