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Background: Congenital longitudinal deficiency of the fibula is the most common lower extremity congenital deficiency, with a broad spectrum of severity and subsequent reconstructive treatment. Published classification schemes do not accurately predict reconstructive treatment currently in practice. Methods: We reviewed all medical records of patients with a dominant deformity of congenital fibular deficiency who were managed at our institution between 1971 and 2005. We assessed the impact of limb-length inequality, foot deformity, bilateral extremity involvement, and extent of fibular preservation on the treatment of the limb deficiency. Results: One hundred and four patients (including twenty-two with bilateral congenital fibular deficiency) with 126 affected extremities had adequate radiographs to be included in the study. Femoral shortening was noted in seventy (85.4%) of eighty-two patients with unilateral limb involvement. Limb-length discrepancy prior to any treatment remained proportional in forty-seven (82.5%) of fifty-seven patients during an average duration of follow-up of ten years and ten months (range, two years to fifteen years and six months). Limb salvage with foot preservation was deemed feasible in thirty-eight (97.4%) of thirty-nine five-rayed feet, thirty (81.1%) of thirty-seven four-rayed feet, twenty (48.8%) of forty-one three-rayed feet, and one of nine feet having fewer than three rays. Twenty-two (41.5%) of fifty-three limbs with an absent or vestigial fibula were not treated with amputation. Of the twenty-two patients with bilateral fibular deficiency, twelve (54.5%) had preservation of both feet, three (13.6%) had unilateral amputation, and seven (31.8%) had bilateral amputation. Conclusions: We propose a simplified classification for congenital fibular deficiency based on the clinical status of the foot and the magnitude of limb shortening as a percentage of the contralateral limb on radiographs. This classification may be effectively applied in infancy to allow the physician and family to anticipate the extent of deformity at maturity and to estimate the amount of treatment required to reconstruct this limb deformity. This system more accurately predicted the management of patients with fibular deficiency who were managed at our institution over the past three decades. [ABSTRACT FROM AUTHOR]

The article presents a study on compression injuries as the most common cause of peroneal neuropathy. It states that leg-crossing and prolonged immobility can cause compression injuries which damage the head of fibula. It mentions the application of ankle-foot orthotsis (AFO) devices can serve as a primary remedy for peroneal neuropathy or foot drop and that treatments for the condition are not fully based on concrete science. Moreover, the study suggests that foot drop can be prevented.

Purpose: Fibula hemimelia is the most common congenital deficiency of long bones. Primary treatment options include amputation with prosthetic fitting or limb reconstruction. The aim of our study was to conduct a systematic review comparing amputation with limb reconstruction for fibula hemimelia., Methods: MEDLINE, EMBASE, Web of Science, Elsevier Scopus, and the Cochrane Registry of Clinical Trials were searched from 1951 to 2019 for studies that evaluated amputation versus limb reconstruction for fibula hemimelia. Random effect models were utilized for the meta-analytic comparisons of amputation versus limb reconstruction for patient satisfaction and surgical complications. Descriptive, quantitative, and qualitative data were extracted., Results: Seven retrospective cohort studies were eligible for the meta-analysis, with a total of 169 fibula hemimelia cases. Amputation resulted in an odds ratio of 6.8 (95% confidence interval: 2.4, 19.2) when compared with limb reconstruction in terms of patient satisfaction. Furthermore, limb reconstruction was found to have an odds ratio of 28 (95% confidence interval: 7.8, 100.3) for complications. The total surgical complication rates in the amputation and limb reconstruction groups were 0.2 and 1.2 complications per limb. The rate of surgical procedures per patient was 1.5 and 4.2 for amputation and limb reconstruction, respectively., Conclusions: The cumulative evidence at present indicates better patient satisfaction with less surgical complications and less number of procedures with amputation for fibula hemimelia when compared with limb reconstruction. Absence of uniform protocols make it difficult to compare results accurately., Level of Evidence: Level III-therapeutic.

Background: Patients with congenital fibular deficiency often develop genu valgum secondary to lateral femoral condylar hypoplasia. Guided growth strategies are often performed to correct limb alignment when adequate skeletal growth remains., Methods: A retrospective review of patients with postaxial hypoplasia of the lower extremity managed with an amputation strategy and who had a guided growth procedure for coronal plane limb malalignment during their course of treatment was performed. Clinical and radiographic data, including measures of coronal plane deformity and alignment, type of amputation, subsequent operative procedures, and complications were recorded., Results: Seventeen patients (20 extremities) met study inclusion criteria (mean follow-up 8.8 y). Foot ablation and hemiepiphysiodesis for valgus deformity of the knee was performed in all extremities. The average age at the time of initial hemiepiphysiodesis was 11.2 years at an average of 8.8 years from the initial amputation procedure. The mean preoperative mechanical axis deviation was 26.5 mm, which was corrected to a mean mechanical axis deviation of 7.0 mm. Fifteen (75%) of the extremities had correction of the deformity to neutral alignment after the initial procedure. Lack of correction occurred in 3 extremities, and overcorrection occurred in 2 extremities. Additional procedures were required in 5 extremities for rebound valgus deformity after hardware removal., Conclusions: In patients with postaxial hypoplasia, regular monitoring of the residual limb for growth-related changes must occur to ensure optimal function and prosthetic fit. Timing of the guided growth procedure is critical, as younger patients may be more likely to experience rebound deformity. Families and patients should be made aware that growth might be unpredictable in this population with risks of both overcorrection and undercorrection., Level of Evidence: Level IV-case series.

Background: Congenital pseudarthrosis of the fibula (CPF) is a rare disorder characterized by a deficiency in the continuity of the fibula and can lead to progressive ankle valgus malalignment. An existing classification system for CPF is imperfect and may contribute to heterogeneity in reporting and discrepancy of outcomes in the literature., Methods: Fifteen patients with CPF treated at our institution between 1995 and 2017 were retrospectively identified. Only patients with dysplasia leading to spontaneous fracture or pseudarthrosis were included in this series. The median age at presentation was 2.5 years (range: 3 mo to 13.4 y). The median duration of follow-up from the initial presentation was 11.8 years (range: 2.0 to 24 y). Chart review and serial radiographs were analyzed to assess natural history and outcomes following surgery., Results: The coexistence of tibial dysplasia in CPF is very common. Patients were classified into 3 groups based on the degree of tibial involvement-group 1: no evidence of tibial dysplasia, group 2: mild tibial dysplasia, and group 3: significant tibial dysplasia. Age at presentation and age at which fibular fracture occurred were progressively younger with a greater degree of tibial involvement (P<0.05). In the absence of surgical intervention, group 1 patients did not undergo progressive ankle valgus (defined as the valgus change in tibiotalar angle by ≥4 degrees), whereas all patients in groups 2 and 3 did (P<0.001). Fibular osteosynthesis was performed in 6 patients, with union seen only in group 1 patients. Ten patients underwent distal tibiofibular fusion, with no cases of nonunion seen. Distal tibiofibular fusion with or without medial distal tibial hemiepiphysiodesis halted the progression of ankle valgus in 8 of the 10 patients. Further progression of ankle valgus occurred only in patients who did not undergo concurrent medial distal tibial hemiepiphysiodesis and with considerable wedging of the distal tibial epiphysis at the time of fusion., Conclusions: Tibial dysplasia and CPF are intimately related. Grouping patients on this basis may help guide natural history and treatment and may explain discrepancies in findings in the literature. Fibular osteosynthesis, distal tibiofibular fusion, and medial distal tibial hemiepiphysiodesis may all have an important role in the treatment of CPF., Level of Evidence: Level IV-case series.

Defects occurring in the femoral-fibular-ulnar developmental field are believed to cause the cluster of anomalies seen with femoral, fibular and ulnar limb deficiencies. Upper limb function must be considered in the management of lower limb deficiencies. The purpose of this study is to determine the frequency and type of upper extremity anomalies found in children with femoral and/or fibular deficiency. A retrospective review of 327 consecutive patients with the diagnosis of femoral and/or fibular deficiency was performed using existing records and radiographs. Characteristics of those with and without upper extremity anomalies were compared. Upper extremity anomalies were identified in 56 patients. They were more common among those with bilateral, compared with unilateral, lower extremity deficiencies (P < 0.0001). Seventy-five upper limbs were involved with 50 ulnar deficiencies, nine congenital transhumeral deficiencies, four congenital shoulder disarticulations, seven cleft hands, two radial head dislocations and one each - radial deficiency, syndactyly and capitate-lunate coalition. Two patients with bilateral upper extremity anomalies had ulnar deficiency on one side and a transverse deficiency on the other. Upper extremity anomalies are found in 17% of children with femoral and/or fibular deficiency, especially with bilateral lower extremity involvement. Ulnar deficiency is the most common type but one-third had other anomalies. The frequent finding of congenital transverse upper extremity deficiencies suggests there may be common embryology.

Background: Deformity of the tibia, including shortening and angulation, may accompany severe forms of postaxial hypoplasia (fibular deficiency). The current literature reflects varying opinions on the appropriate management for tibial deformity in the setting of fibular deficiency., Methods: We performed a retrospective review to determine outcomes of tibial deformity correction in patients with a primary diagnosis of fibular deficiency. Clinical and radiographic outcomes of patients treated with foot ablation were reviewed to establish indications for tibial deformity correction, identify occurrence of additional surgical procedures related to limb alignment or deformity, and characterize difficulties with prosthetic wear potentially related to residual or recurrent tibial deformity., Results: From 1989 to 2016, 51 patients (57 extremities) with fibular deficiency were managed with a foot ablation procedure. Twenty-five (44%) had simultaneous correction of the tibial deformity. The initial tibial deformity measured 42.5 degrees, was corrected to 5.6 degrees intraoperatively, and measured 18.6 degrees at follow-up, suggesting recurrent deformity. In follow-up, approximately half of the patients complained of redness and one third complained of a continued prominence along the anterior tibia. Thirty-two extremities had an isolated foot ablation procedure without tibial osteotomy. Radiographic review demonstrated mild tibial bowing at the time of amputation with a mean angular deformity of 15.4 degrees and remained unchanged during the follow-up period (mean, 12.7 degrees). Similar to the osteotomy group, approximately half of the patients complained of redness and erythema over the anterior bow, with one fourth noting prominence, and only 2 reporting significant pain., Conclusions: Tibial osteotomies in patients with more significant degrees of angular deformity can be safely performed at the same setting as foot ablative procedures for fibular deficiency. Recurrent deformity with growth may occur. Patients and their caregivers should be aware that rebound deformity may occur, but typically can be managed with prosthetic adjustment and without significant disruption to the child's daily activities., Level of Evidence: Level IV (case series).

Background: Limb lengthening for congenital femoral deficiency (CFD) with or without fibular hemimelia can be performed with both external and internal devices. The purpose of this study is to compare clinical outcomes of femoral lengthening utilizing monolateral external fixation versus a magnetically motorized intramedullary nail in patients with CFD with or without fibular hemimelia., Methods: This retrospective review included 62 patients with femoral lengthening, 32 patients had monolateral external fixation (group A), 30 patients had internal lengthening nail (group B). Mean age in years was 9.4±3.8 and 15.4±4.9 for groups A and B, respectively. Mean follow-up in years was 4.47±2.7 and 1.86±0.7 years for groups A and B, respectively., Results: Mean lengthening achieved was 5.6±1.7 and 4.8±1.4 cm for group A and group B, respectively (P=0.052). Mean distraction index was 0.7±0.2 mm/d for group A and 0.7±0.2 mm/d for the group B (P=0.99). Mean consolidation index for group A was 29.3±12.7 and 34.8±11.2 d/cm for group B (P=0.08). Mean arc of motion before surgery and at final follow-up were similar between groups (P=0.35). Group A had significantly less range of motion at the end of distraction (P=0.0007) and at consolidation (P<0.0001). Both groups had similar rates of obstacles and complications. A significant difference between groups was found in the total problems (P<0.001) specifically with pin site/superficial infection (P<0.0001)., Conclusions: The intramedullary nail had superior range of motion during the lengthening phase and at consolidation and an overall lower problem complication rate, while maintaining similar distraction and healing indices to monolateral external fixation. Internal lengthening nails represent a significant advance in technology for CFD lengthening., Level of Evidence: Level IV-therapeutic.

Background: Dysplasia of the cruciate ligaments has been found in many patients with congenital fibular deficiency. A recent classification system has shown that radiographic tibial spine changes can predict the hypoplasia and aplasia of the cruciate ligaments. We used this radiographic classification to determine the frequency of these abnormalities and how they correlate with the severity of fibular deficiency and lateral femoral condylar hypoplasia., Methods: Using a hospital database search for fibular deficiency, 99 patients ≥6 years with unilateral fibular deficiency were identified. Existing radiographs of both knees were available for 75 patients and reviewed for the tibial spine changes and Achterman and Kalamchi classification of the fibular deficiency. Measurements of femoral condyle heights in 74 of 75 patients were recorded before any surgery to the distal femoral physis to assess lateral femoral condylar hypoplasia., Results: Twenty-two patients had hypoplasia of the lateral tibial spine+normal medial spine, 29 had absence of the lateral tibial spine+hypoplastic medial spine, and 11 had absence of both tibial spines. Five tibial spines were normal and 8 were unclassifiable. The severity of the tibial spine dysplasia, particularly absence of the lateral tibial spine, correlated with the severity of the fibular deficiency. (P<0.0001) The mean lateral femoral condylar hypoplasia, measured by involved: uninvolved lateral condyle heights, was 0.85±0.11. Those with some preservation of the lateral tibial spine had less lateral femoral condylar hypoplasia (P=0.0009). This lateral femoral condylar hypoplasia was positively associated with the severity of the fibular absence (P=0.039) and foot ray deficiency (P=0.036)., Conclusions: The severity of cruciate ligament dysplasia in fibular deficiency is directly correlated with the severity of fibular absence, lateral femoral condylar hypoplasia, and the absence of foot rays. This suggests that the embryological factors involved have a complex interplay for all of these clinical findings., Level of Evidence: Level III.

To assess the characteristics of ulnar deficiency (UD) and their relationship to lower extremity deficiencies, we retrospectively classified 82 limbs with UD in 62 patients, 55% of whom had femoral, fibular, or combined deficiencies. In general, UD severity classification at one level (elbow, ulna, fingers, thumb/first web space) statistically correlated with similar severity at another. Ours is the first study to show that presence of a lower limb deficiency is associated with less severe UD on the basis of elbow, ulnar, and thumb/first web parameters. This is consistent with the embryological timing of proximal upper extremities developing before the lower extremities.

Introduction: Achondroplasia is the most common skeletal dysplasia with a rate of nearly 1/10,000. The development of lower extremity deformity is well documented, and various modes of correction have been reported. There are no reports on the use of growth modulation to correct angular deformity in achondroplasia., Methods: Medical Records from 1985 to 2015 were reviewed for the diagnosis of achondroplasia and growth modulation procedures. Patients who had been treated for angular deformity of the legs by growth modulation were identified. A detailed analysis of their medical record and preoperative and final lower extremity radiographs was completed., Results: Four patients underwent growth modulation procedures, all to correct existing varus deformity of the legs. Three of the 4 patients underwent bilateral distal femoral and proximal tibial growth modulation. The remaining patient underwent tibial correction only. Two of the 4 patients had a combined proximal fibular epiphysiodesis. All limbs had some improvement of alignment; however, 1 patient went on to bilateral osteotomies. Only 1 limb corrected to a neutral axis with growth modulation alone at last follow-up, initial implantation was done before 5 years of age., Conclusions: Growth modulation is an effective means for deformity correction in the setting of achondroplasia. However implantation may need to be done earlier than would be typical for patients without achondroplasia. Osteotomy may still be required after growth modulation for incomplete correction.

Background: Absent lateral osseous structures in congenital fibular deficiency, including the distal femur and fibula, have led some authors to refer to the nature of foot ray deficiency as "lateral" as well. Others have suggested that the ray deficiency is in the central portion of the midfoot and forefoot.We sought to determine whether cuboid preservation and/or cuneiform deficiency in the feet of patients with congenital fibular deficiency implied that the ray deficiency is central rather than lateral in patients with congenital fibular deficiency., Methods: We identified all patients with a clinical morphologic diagnosis of congenital fibular deficiency at our institution over a 15-year period. We reviewed the records and radiographs of patients who had radiographs of the feet to allow determination of the number of metatarsals, the presence or absence of a cuboid or calcaneocuboid fusion, the number of cuneiforms present (if possible), and any other osseous abnormalities of the foot. We excluded patients with 5-rayed feet, those who had not had radiographs of the feet, or whose radiographs were not adequate to allow accurate assessment of these radiographic features. We defined the characteristic "lateral (fifth) ray present" if there was a well-developed cuboid or calcaneocuboid coalition with which the lateral-most preserved metatarsal articulated., Results: Twenty-six patients with 28 affected feet met radiographic criteria for inclusion in the study. All affected feet had a well-developed cuboid or calcaneocuboid coalition. The lateral-most ray of 25 patients with 26 affected feet articulated with the cuboid or calcaneocuboid coalition. One patient with bilateral fibular deficiency had bilateral partially deficient cuboids, and the lateral-most metatarsal articulated with the medial remnant of the deformed cuboids. Twenty-one of 28 feet with visible cuneiforms had 2 or 1 cuneiform., Conclusions: Although the embryology and pathogenesis of congenital fibular deficiency remain unknown, based on the radiographic features of the feet in this study, congenital fibular deficiency should not be viewed as a global "lateral lower-limb deficiency" nor the foot ray deficiency as "lateral.", Level of Evidence: Level IV-prognostic study.

Few papers have described patients treated surgically with single osteotomy for congenital posteromedial bowing of the tibia and fibula. Only one paper has described two-level osteotomy for deformity correction: the first for deformity correction and the second for bone lengthening. There are no publications describing the surgical correction of deformation only by the method of multilevel tibial and fibular osteotomy. Research material included four children aged between 3.1 and 5.1 years (average age: 3.7 years) who were operated upon for bowing of the tibia and fibula exceeding 35° in the coronal plane. In all cases, tibial osteotomy was carried out at three or two levels accompanied by fibular osteotomy, and with intramedullary stabilization using K-wires (three patients) or Rush pin (one patient). Follow-up ranged from 3 to 7.7 years. In all cases, axis correction and bone healing were achieved. In large congenital posteromedial bowing of the tibia and fibula, a multilevel tibial and fibular osteotomy may be a better solution than an orthosis or a long time waiting for a spontaneous correction of the deformation. Large circumferential periosteal release that accompanied the surgery influenced the stimulation of bone growth. It may induce the process of lower limbs' equalization not to require the application of intensive surgical procedures.

Fibular hemimelia is the most common lower extremity congenital longitudinal deficiency leading to cosmetic deformity, pain, and gait disturbance. Eight-plate hemiepiphysiodesis has proven to be successful in correcting angular deformity of the knee; it is an easy surgical technique and yields a more rapid rate of correction. The aim of this study was to determine the effectiveness of eight-plate hemiepiphysiodesis in bilateral genu valgum resulting from fibular hemimelia and overall height improvement in two girls and four boys with a mean age of 22 months who were followed up for a period of 18 months. The variables studied were arm span, the total height ratio, intermalleolar distance, tibiofemoral angle, and activity. All the parameters improved significantly. The mean improvement in the valgus was 7.41°. The mean intermalleolar distance reduced by 19.5 cm, and all patients achieved a significant improvement in lower limb function. Although there was significant improvement in total height, it still lagged behind arm span at the time of final follow-up. The results of eight-plate hemiepiphysiodesis were found to be satisfactory. We conclude that it is a reasonable treatment option for young patients with an adequate growth potential.

Recent studies on ankle syndesmosis injuries have shown that a significant amount of rotational malediction of the distal fibula are missed and may lead to poor functional outcome. A new set of radiographic criteria were developed to help detect distal fibula internal and external rotation using conventional fluoroscopy. The criteria were tested using a cadaveric model for Weber C ankle fractures fixed with the fibula in various degrees of internal and external rotation. Using the criteria orthopaedic trauma surgeons were able to improve their accuracy and agreement on assessment of degree and direction of fibula rotation.

The objective of this study is to present treatment of fibular hemimelia along with the complications, results, and an algorithm for treatment indications on the basis of authors' experience. A group of 31 patients was studied. In patients fulfilling the criteria for amputation, Syme's amputation should be performed. Elongation should be performed in case of type IA or IB fibular hemimelia, with a functional foot with more than three rays, leg shortening less than 5 cm at birth, and less than 10 cm at 9 years of life. The combination of epiphysiodesis with elongation produces the best outcome and is best accepted by the patients.

Congenital diastasis of the inferior tibiofibular joint is an extremely rare variant of dysplastic tibial anomaly, which is usually associated with significant shortening of the lower leg and ipsilateral foot deformity due to talus incarceration in the distal tibiofibular mortise. The purpose of this study was to present the long-term results of reconstructive treatment and the functional outcome after a follow-up of 11-16 years. The principles of extremity preservation and reconstruction with the Ilizarov frame have shown a stable ankle joint, a plantigrade foot, and fully independent outdoor ambulation in both patients at the final follow-up. We concluded that amputation should not be performed under this condition.

Tibiofibular torsion was measured by computed tomography in three series of patients affected by congenital clubfoot who were treated with different protocols. The normal leg of unilateral deformities served as the control. For the bilateral cases, only the right side was included in the study. The angle between the bicondylar axis of the tibia and the bimalleolar axis was the index of tibiofibular torsion. There were 34 clubfeet in the first series, treated with a posteromedial release, and 40 clubfeet in the second series, treated with a modified Ponseti method, whereas the third series included 16 clubfeet, treated with the original Ponseti method. All 90 clubfeet were graded at birth as group 3 according to the Manes classification. No patient had previous treatment. The patients of the first and the second series were followed up to maturity, whereas the patients of the third series were followed up to a maximum of 11 years of age. In the congenital clubfoot, the tibia and the fibula were externally rotated, in comparison with the normal leg; in fact, the average value of the angle of tibiofibular torsion was 32.2° in the first series, 23.9° in the second series, and 21.1° in the third series. In the normal tibiae, the average value of the angle of tibiofibular torsion was 21.4°. The difference between the first series and the normal controls was statistically significant, as was the difference between the first one and the other two series. The value of the tibiofibular torsion angle seems to be related to the manipulation technique used to treat clubfoot: when the manipulation does not allow a progressive eversion of the talus underneath the calcaneus, the external tibial torsion increases. At follow-up, an intoeing gait was present in seven treated clubfeet of the first series. In all of them except one, the highest value of the external tibial torsion angle was observed, with a low value of the Kite's angle and/or residual forefoot adduction. In the treated congenital clubfoot, persistent intoeing is not related to the angle of tibial torsion but rather to the amount of correction of calcaneal inversion and residual forefoot adduction.

We report the results of surgical treatment of congenital postero-medial bowing of the tibia and fibula. Twenty patients with congenital postero-medial bowing were seen with nine patients treated surgically (corrective osteotomy or lengthening and deformity correction with Ilizarov fixator) and 11 patients managed conservatively. The angles of medial and posterior angulation and limb length discrepancy were recorded serially and compared. Surgical complications were recorded. The mean follow-up was 9.5 and 6.1 years after surgery. Although there was a reduction in angulation and correction of limb length discrepancy, we encountered complications in the surgically treated patients. There was no statistically significant difference between the surgically treated and conservatively managed groups with respect to mean angulation, though there was a significant difference in the mean limb length discrepancy. In conclusion, we advocate a one-stage lengthening and correction of the residual deformity closer to skeletal maturity.

The aim of this study was to determine the prevalence of ipsilateral congenital musculoskeletal anomalies associated with fibular hemimelia. We also attempted to determine the corelation between the eventual limb length discrepancy at maturity and these associated anomalies, as well as the Achterman-Kalamchi class of these patients. The records and roentgenograms of 45 patients with fibular hemimelia were reviewed retrospectively. All patients were classified into three groups (types I-A, I-B, and II) according to the Achterman-Kalamchi system. The prevalence of congenital limb anomalies, lower extremity discrepancy percentage, and ultimate limb length discrepancy at skeletal maturity were evaluated. There was no significant statistical association between the number of congenital limb anomalies and severity of the limb length discrepancy, but patients included in the mildly affected group (type I-A) had a higher average prevalence of congenital limb anomalies than those classified as types I-B and II. There was a substantial correlation between congenital short femur as well as the Achterman-Kalamchi classification system and the predicted limb length discrepancy. A similar percentage of limb length discrepancy in types I-B and II was seen. In conclusion, the numbers of associated congenital limb anomalies are not predictive of the eventual limb length discrepancy. Presence of a congenital short femur and ball and socket ankle are predictive of a higher limb length discrepancy. Achterman-Kalamchi types I-B and II show a similar percentage of limb length discrepancy and this is significantly higher than that seen in type I-A.

The aim of this study is to evaluate the long-term results of management of fibular hemimelia (Achterman-Kalamchi type-II) using the Ilizarov method. We reviewed 157 consecutive patients (180 limb segments) with a mean follow-up period of 10.7 years (1.2-21 years). The results were favorable. Although, this type of management is technically demanding and entails a lengthy procedure with many complications anticipated, the Ilizarov lengthening after limb reconstruction is still an option for management of this type of limb deficiency.

We studied case records and radiographs of 20 children with congenital posteromedial bowing of the tibia (CPMBT) retrospectively to determine the pattern of correction of the bowing, the associated growth abnormalities of the tibia and fibula, and the role of surgical intervention in CPMBT. The magnitude of diaphyseal bowing in two planes and the physeal inclination were measured. Abnormalities of ossification of the distal tibial epiphysis and inclination of the distal articular surface if present were noted and shortening of the tibia was recorded. The rate of resolution of deformity was noted from sequential radiographs and expressed as percentage reduction per month of follow-up. At initial presentation the magnitude of deformity varied; the most severe posterior diaphyseal bow was 70 degrees whereas the most severe medial diaphyseal bow was 64 degrees. Two distinct mechanisms seem to be responsible for resolution of the deformity in CPMBT; one involves physeal realignment and the other involves diaphyseal remodeling. In the first year of life, rapid resolution of angulation was noted; the rate of resolution reduced significantly thereafter. In a proportion of children with CPMBT residual deformity may persist till over 4 years of age. Physeal realignment occurred at a faster rate than diaphyseal remodeling. The degree of shortening was related to the severity of bowing and shortening as great as 40% was noted in a patient. Wedging of the distal tibial epiphysis and fibular hypoplasia with valgus inclination of the distal tibial articular surface occur in some children with CPMBT. Eccentric ossification of the distal tibial epiphysis in early childhood may be a predictor of wedging of the distal tibial epiphysis later on. We recommend all the children with CPMBT to be followed up periodically till skeletal maturity, to identify cases with residual bowing, ankle deformity, muscle weakness, and limb length inequality as active surgical intervention may be needed to correct these problems.

Background: Progressive varus deformity of the tibia in pediatric patients after transtibial and Syme amputations has not been reported in a series. A distal tibia to fibula synostosis, created surgically to minimize the risk of terminal overgrowth or occurring spontaneously, was noted in most patients. The goals of this study are to address the causes of the deformity, the implications for prosthetic wear, and potential treatment options., Methods: Twelve patients identified from the juvenile amputee database at 2 centers developed progressive varus deformity of the residual limb. One patient had bilateral involvement. A distal tibia-fibula synostosis was noted in 12 (92%) of the residual limbs, and in one, a fibrous union was suspected. The level of amputation was trans-tibial in 10 patients, and Syme amputation in 3 patients. Two patients had acquired trans-tibial level of amputation from congenital constriction band syndrome. Nine of the patients (75%) were between the ages of 3 and 5 years at the time of injury., Results: The mean proximal medial tibial angle was 80.5 degrees (range, 75-85 degrees). Ten of the patients underwent procedures to correct the mechanical axis and resolve or prevent problems with prosthetic fitting. Four patients has proximal tibial osteotomies (HTO), 2 oblique closing wedge osteotomies, 1 shaft osteotomy, and 4 lateral proximal tibial hemi-epiphyseodesis. In 2 patients, no correction was recommended., Conclusions: The presence of a distal tibia-fibula synostosis in pediatric amputee patients may contribute to the risk of developing a progressive varus deformity and should be monitored during a child's growth. Distal tibiofibular synostosis may disrupt normal differential longitudinal growth and may contribute to this progressive angular deformity. Severe deformity may require prosthetic modifications or operative correction to provide for a normal mechanical axis. Lateral hemiepiphyseodesis of the proximal tibia can be effective if the deformity is detected early. We do not recommend creation of a synostosis in the young child for terminal growth., Level of Evidence: Level 4.

Treatment of cases of congenital pseudarthrosis of the fibula presenting with severe or progressive valgus deformity in early childhood is challenging. The purpose of the study presented was to analyse the deforming mechanisms to get therapeutic recommendations for this condition. A 2.5-year-old child was treated by resection of pseudarthrosis and distal tibiofibular fusion (Langenskiold operation) with autogenous bone grafting, associated with subtalar arthroereisis: progressive correction of the deformity was achieved. A 21-month-old child was treated with resection of pseudarthrosis, homologous bone grafting and fibular osteosynthesis: after failure, Langenskiold operation with autogenous bone grafting was performed and stabilization of the deformity was achieved. In young children with congenital pseudarthrosis of the fibula and valgus deformity, early surgical treatment is advisable: fibular ostheosynthesis has limited indications; distal tibiofibular fusion remains the treatment of choice, providing correction that may exceed the simple stabilization of deformity expected. Radiographic findings lead the authors to suggest that part of the effectiveness of the procedure is due to abnormal growth patterns.

In this case report, we present a patient with right teratologic high hip dislocation, femoral hypoplasia and below-knee hemimelia associated with left fibular hemimelia. Combined open reduction, proximal femoral osteotomy and a Dega acetabuloplasty were performed in the right hip. Closed tibial wedge osteotomy and centralization of the foot with lateral release and Achilles tendon lengthening were performed for the left side. The patient was able to walk with her prosthesis successfully within the first six postoperative months.

The purposes of this study were to determine the clinical course of isolated congenital pseudarthrosis of the fibula (ICPF) and to establish optimal treatment guidelines for this disease. Thirteen fibulae in 12 patients with ICPF were followed for an average of 6.2 years. Twelve of the 13 fibulae were regarded to originally have had a pre-pseudarthrotic condition. Five fibulae remained intact during follow-up, but 8 developed pseudarthrosis. Two cases with established pseudarthrosis were observed for ankle alignment change without intervention, and in 4 cases successful fibular osteosynthesis maintained the ankle alignment. The remaining 2 adolescent cases presenting with ankle valgus were treated using the Ilizarov technique and distal tibiofibular synostosis. ICPF follows a unique clinical course, which should be taken into consideration along with fibular status, ankle alignment, and patient age when planning treatment.

Congenital diastasis of the inferior tibiofibular joint presents clinical problems of talipes equinovarus, ankle diastasis, and leg-length inequality. In the past, foot disarticulation and prosthetic fitting has often been chosen as a solution because of the difficulty involved in functional reconstruction. The authors report three additional cases of congenital diastasis of the inferior tibiofibular joint that were satisfactorily treated by lengthening of the tibia and fibula and foot repositioning by the Ilizarov technique. After treatment, all patients had a plantigrade, functional foot with normal sensation at skeletal maturity and were satisfied with the cosmetic and functional outcomes. The Ilizarov technique is effective in accomplishing the goals of leg-length equalization and foot repositioning.

We have noted a number of patients with features of fibular hemimelia with radiographically normal fibulae. This study was undertaken to further define this group. A review of hospital records and radiographs over a 72-year period identified 149 limbs in 123 patients with features of fibular hemimelia syndrome. Sixteen limbs in fourteen patients had findings of fibular hemimelia with radiographically normal fibulae. Thirteen of 16 had absent lateral rays with either ball and socket ankle joint, tarsal coalition or both. Six of the 13 had limb shortening. Three limbs in three patients did not have absent lateral rays, but had at least two other features of fibular hemimelia syndrome. All of these three limbs had ball and socket ankles and tarsal coalitions and two had shortening. These patients represent a mild subset of fibular hemimelia syndrome and we propose that they be classified as type 0 fibular hemimelia.

The Melnick-Needles syndrome (MNS) is a rare bone dysplasia with characteristic clinical and radiologic diagnostic findings. The diagnosis of MNS was made for the patient reported in this paper clinically and radiologic confirmation was done afterwards. Her left fibula was shorter than the right one. She had lateral insufficiency in her left ankle and could not bear weight on her left foot. A fibular length discrepancy of 2 cm was assessed. Her ankle-hindfoot score was 49 according to the American Orthopedic Foot and Ankle Society (AOFAS) clinical rating system for ankle and hindfoot. Simple fibular lengthening was performed with a unilateral external fixator. Two centimetres of fibular lengthening was obtained in 14 weeks. Her AOFAS ankle-hindfoot score was 82 after 27 months of follow-up. Ankle stability and plantigrade foot were achieved and the result was satisfactory.

We describe two unrelated boys with bilateral fibula aplasia, poly- and oligodactyly, and bowed tibiae in two non-consanguineous Brazilian families. These cases are similar to those reported by Fuhrman et al. [(1980). Skeletal Dysplasias. New York: Alan R. Liss Inc. pp. 519-524].

Fibular hemimelia is associated with a deformity of the distal tibial epiphysis resulting in a convexity with lateral and posterior slope of the distal joint surface. The deformity results into an equinovalgus position of the foot and ankle and frequently consecutive dislocation of the foot. A new procedure is presented, in with a metaphyseal ostotomy is performed towards the physis in order to bend the postero-lateral third of the distal tibial epiphysis forming a concave distal tibial joint surface. This procedure was done in four feet in three patients at ages between 7 and 20 months combined with soft tissue releases and rebalancing the tendons. At a follow-up between 6 and 42 months the position of the feet is well retained.

A new classification system for fibular hemimelia is proposed based on the authors' experience with 32 patients with 33 involved limbs representing a spectrum of involvement. The data demonstrate the broad and unpredictable relationships among the fibula, ankle, and foot in this disorder. The classification system, based on fibular and ankle morphology, hindfoot coalition, and foot ray deficits, is designed to improve communication about the condition. Because of the variability and unpredictability of the multiple relationships, limb salvage criteria should also include the nature of the foot and ankle and not merely depend on the length discrepancy or the presence or absence of the fibula.

This study was designed to determine the incidence of clubfoot in our fibular hemimelia population. A retrospective review of fibular hemimelia patients treated at our institution over the past seventy years was conducted. We identified twenty-three cases of clubfoot in 121 patients with 147 involved limbs. Nineteen of twenty-three limbs retained the foot, four were converted to Syme's amputation because a plantigrade functional foot could not be obtained. Hindfoot coalitions were present in eighteen limbs and nineteen had one or more missing lateral rays. In summary, the association of clubfoot deformity with fibular hemimelia is more common than previously reported. It was not always evident to the surgeon treating these patients that fibular hemimelia syndrome was present in addition to clubfoot deformity. The presence of a coalition is a relatively constant finding in this condition and should be anticipated by the treating clubfoot surgeon.

The instability of the upper and the lower ankle joint represents a serious problem in patients with fibular aplasia. None of the previous techniques demonstrated at least sufficient results. In this report we present a new technique of malleolus externus plasty applied in a 5 1/2-year-old boy with fibular aplasia (type II according to Coventry and Johnson or type Ib according to Achtermann and Kalamchi) with a 6 cm length deficiency of the lower leg and 1 cm of the upper leg. Furthermore, he showed a tibial antecurvation deformity of 20 degrees, a fourth ray foot with adduction deformity of the hindfoot, cutane syndactyly D 2-4, and a hallux varus. This new technique works on the following principle. A triangular iliac crest transplant is implanted with an apophysis and the annexing fascia glutealis, including the osseous part of the transplant, in the lateral distal tibia in a way that the growing apophyseal part lays distally covering the lateral talus. Gluteal fascia annexed to the apophysis was used for the reconstruction of a lateral tendon. Using a ring fixator the transplant is fixed and coincidentally the lower leg lengthened and the axis corrected. The axis deformity and the leg-length deficiency of 7 cm were equalized. The fixator could be removed after 6 months. Radiologically, an entire integration of the iliac crest transplant was found. Magnetic resonance imaging showed it had sufficient circulation. The reexamination 2.5 years later demonstrated a simultaneous growth of the malleolus externus and the distal tibia and stable ankle joints with sufficient mobility and full weight-bearing capability. For support of the foot the boy uses ready-made shoes with curved arch supports. In conclusion, using this new technique an individually adapted lateral malleolus with growth-potential can be constructed that stabilizes the foot and the ankle joints. Thus, reluxations of the foot specifically in relation to lengthening of lower legs can be avoided with concurrent preservation of the range of movement of the ankle joint.

Roberts-SC phocomelia syndrome (RS) is an autosomal recessive disorder of symmetric limb defects, craniofacial abnormalities, pre- and postnatal growth retardation, and mental retardation. Patients with RS have been reported to have premature separation of heterochromatin of many chromosomes and abnormalities in the cell-division cycle. No case has been reported who had normal intelligence and normal cell division with typical clinical features of the RS. We report a case of a six-year-old male of clinical and radiologic findings of typical RS with normal cell division and normal intelligence. Although he showed growth retardation, his intelligence was normal. Van Den Berg and Francke later reported that 79 out of 100 cases of Roberts syndrome had premature cell separation (PCS). We think that this case may demonstrate severe expression of the Roberts syndrome even though PCS is not exhibited. The limb involvement of this case was symmetrical, and he showed phocomelia of upper limbs, equinus valgus deformity of ankle, aplasia of fibula, and shortness of fifth toes while his hands and feet were normal with 5 rays each. Craniofacial abnormalities of this case were typical; he showed scaphocephaly, mild hypertelorism, mandibular hypoplasia, dysplastic helix of ear, narrowing of external auditory canal, and cleft palate with wide gap. This report supports the theory that normal intelligence can make social-personal adjustment possible even if all of the stigmata of Roberts syndrome is present.

Fibular dimelia accompanied by complete tibial agenesis is a rare developmental anomaly that has been reported in the anatomic and clinical literature as both a dimelia and a diplopodia. Previous reports have offered variable descriptions of structural aberration and possible modes of altered embryologic development. We present a clinical and radiographic history of the progressive development of this anomaly, and a detailed postsurgical dissection and histopathologic examination. The specific mirroring of the separate components in this case has not been previously reported, and suggests that a reestablishment of limb polarity may have occurred during embryogenesis. Our detailed morphologic investigation strongly suggests a central role for the mesenchymal precursor from which chondro-osseous morphology subsequently emerges, and the relevance to establishing muscular attachments.

Fibular hemimelia is associated with an equinovalgus deformity of the foot and ankle and different degrees of wedging of the distal tibial epiphysis. This deformity is often a major problem during lengthening of the shortened tibia. To determine the significance of the wedge-shaped distal tibial epiphysis in the pathogenesis of the equinovalgus deformity of the foot and ankle during and after lengthening, we reviewed 20 patients who had undergone tibial lengthening by either the Wagner or the Ilizarov technique. The mean duration of follow-up after removal of the fixator was 5.2 years (range, 2.3-9.7 years). Three types of wedge-shaped distal tibial epiphyses were identified. A mildly wedged (type I) epiphysis was found in seven patients, a moderately wedged (type II) epiphysis was found in seven patients, and a severely wedged (type III) epiphysis, in six patients. Premature fusion of the lateral part of the distal tibial physis and growth retardation of the tibia were common after lengthening in patients with the type II or type III epiphysis. After lengthening, all patients with a type II or type III epiphysis had a recurrence or aggravation of foot deformities that existed before lengthening. This usually necessitated various secondary operative procedures to obtain a plantigrade foot. We believe that after lengthening, one should anticipate varying degrees of mild growth retardation and minimal foot deformity in patients with type I epiphysis, worsened asymmetric growth retardation and progressive foot deformity in patients with type II epiphysis, and severe growth retardation and severe foot deformity in patients with type III epiphysis.

Congenital deficiency of the fibula may present a variety of patterns. In a series of 20 patients with this diagnosis, we have observed limb-length inequality and a spectrum of musculoskeletal anomalies involving the ipsilateral hip, femur, knee, tibia/fibula, ankle, and foot. Considering the frequently associated abnormalities of the lower extremity, the term postaxial hypoplasia may be more descriptive than the traditional terms fibular hemimelia or fibular a/hypoplasia. By raising the awareness of associated deformities, the clinician is better prepared to advise patients and to intercede accordingly. Based on our experience, we advocate a modular treatment approach combining limb lengthening with hemiepiphysiodesis of the distal femur and/or ankle to correct valgus alignment and establish a neutral mechanical axis. Contralateral epiphysiodesis as an adjunct may be preferable to double or repeated lengthening. The goal is to achieve symmetry and stable joints at skeletal maturity with a minimal number of well-timed surgical interventions. By using this strategy and with minimal morbidity, 10 of our patients who have reached skeletal maturity have achieved a satisfactory outcome.

Eight pediatric patients who underwent nine simultaneous ipsilateral femoral and tibial lengthenings with the Ilizarov external fixator were reviewed. The patient's demographics, diagnoses, corticotomy levels, mechanical axes, healing indices, amounts of lengthening, and complications were recorded. The patients' average age was 8 years 10 months (5 years 4 months-15 years 10 months) with an average follow-up of 49 months (30-88 months). The percentage of femoral lengthening averaged 16.7% (8-23%) with an average healing index of 28 days/cm (20-38 days/cm). The percentage of tibial lengthening averaged 18% (9.6-23.6%) with an average healing index of 29 days/cm (1940 days/cm). Four complications in three patients occurred as a direct result of the lengthening process. Three of the complications involved soft-tissue contractures, which were each successfully treated with one additional surgical procedure, whereas the fourth complication involved poor bone regeneration and required bone grafting and additional immobilization.

We describe a case of a surgically proven intraosseous venous anomaly of the fibula in a patient who presented with ankle pain and swelling with deep venous thrombosis. MRI, including gadolinium-enhanced MR angiography, provides a less invasive means than conventional venography for diagnosing these lesions and assessing venous patency while also allowing for evaluation of extravascular structures.