Most patients (95%) with fibular hemimelia have an absent anterior cruciate ligament (ACL). The purpose of this study was to assess the long-term outcome of such patients with respect to pain and knee function. We performed a retrospective review of patients with fibular hemimelia and associated ACL deficiency previously treated at our institution. Of a possible 66 patients, 23 were sent the Musculoskeletal Outcomes Data Evaluation and Management System (MODEMS) questionnaire and Lysholm knee score to complete. In all, 11 patients completed the MODEMS and nine completed the Lysholm score questionnaire. Their mean age was 37 years (27 to 57) at review. Five patients had undergone an ipsilateral Symes amputation. There was no significant difference in any subsections of the Short-Form 36 scores of our patients compared with age-matched controls. The mean Lysholm knee score was 90.2 (82 to 100). A slight limp was reported in six patients. No patients had episodes of locking of the knee or required a supportive device for walking. Four had occasional instability with sporting activities. . These results suggest that patients with fibular hemimelia and ACL deficiency can live active lives with a similar health status to age-matched controls

To review the results of limb lengthening and deformity correction in fibular hemimelia, fifty-five patients with fibular hemimelia underwent limb reconstruction at Sheffield Children’s Hospital. According to Achter-man and Kalamchi classification, twenty-six were classified as Type IA, six as Type IB and twenty-three as Type II fibular hemimelia. All patients had at least some shortening of ipsilateral femur but forty-nine had sig-nificant femoral deficiency. Lengthening of tibia and in significant cases femur was done using De Bastiani or Vilarrubias or Ilizarov methods. Ankle valgus and heel valgus were corrected through osteotomies either in the supramalleolar region or heel. Equinus was corrected by lengthening of tendoachelis with posterior soft tissue release and in severe cases using Ilizarov technique. The average length gained was 4.2 cm (range 1 to 8) and the mean percentage of length increase was 15.82 (range 4.2 to 32.4). Mean bone healing index was 54.23 days/cm. Significant complications included knee subluxation, transient common paroneal nerve palsy, and recurrence of equinus and valgus deformity of foot. Overall alignment and ambulation improved in all patients. Knee stiffness due to cruciate deficient subluxations needed prolonged rehabilitation. Presence of 3-ray foot gives a better functional result and cosmetic acceptance by patients. The Ilizarov frame has the advantage to cross joints and lengthen at the metaphysis. Limb reconstruction in fibular hemimelia using limb lengthening and deformity correction techniques improve functional status of involved lower limb

Aims: To review the results of limb lengthening and deformity correction in fibular hemimelia. Methods: Fifty-five patients with fibular hemimelia underwent limb reconstruction at Sheffield Children’s Hospital. According to Achterman and Kalamchi classification, twenty-six were classified as Type IA, six as Type IB and twenty-three as Type II fibular hemimelia. All patients had at least some shortening of ipsilateral femur but forty-nine had significant femoral deficiency. Lengthening of tibia and in significant cases femur was done using either De Bastiani, Vilarrubias or Ilizarov methods. Ankle valgus and heel valgus were corrected through osteotomies either in the supramalleolar region or heel. Equinus was corrected by lengthening of tendoachelis with posterior soft tissue release and in severe cases using Ilizarov technique. Results: The average length gained was 4.2 cm (range 1 to 8) and the mean percentage of length increase was 15.82 (range 4.2 to 32.4). Mean bone healing index was 54.23 days/cm. Significant complications included knee subluxation, transient common paroneal nerve palsy, and recurrence of equinus and valgus deformity of foot. Overall alignment and ambulation improved in all patients. Knee stiffness due to cruciate deficient subluxations needed prolonged rehabilitation. Presence of 3-ray foot gives a better functional result and cosmetic acceptance by patients. The Ilizarov frame has the advantage to cross joints and lengthen at the metaphysis. Conclusion: Limb reconstruction in fibular hemimelia using limb lengthening and deformity correction techniques improve functional status of involved lower limb

We reviewed retrospectively 22 patients (23 limb segments) with fibular hemimelia treated by amputation or limb lengthening to evaluate these methods of treatment. There were 12 boys and 10 girls, all with associated anomalies in the lower limbs. Twelve patients (13 limb segments) had early amputation and prosthetic fitting and ten had tibial lengthening using the Ilizarov technique. At the latest follow-up, the twelve patients who had amputation were functioning well and had few complications. The ten patients who had lengthening had suffered numerous complications, and all had needed either further corrective surgery or to wear braces or shoe-raises. Two of the ten lengthened limbs required late amputation for poor function or cosmesis. There were fewer hospital admissions, clinic visits, and periods of absence from school in the amputation group. Our findings suggest that amputation is a more effective method of management than limb-lengthening in severe fibular hemimelia. The Ilizarov method is an attractive alternative for selected patients, but its exact role is not yet established. One problem is that families often have unrealistic expectations of the surgical and prosthetic technology available and may refuse amputation when this has been recommended

Introduction and Aims: Fibular hemimelia presents a problem with leg length discrepancy and equinovalgus foot deformity. Our protocol is to simultaneously treat both problems, with the goals of equalising limb length and achieving a plantigrade painless functional foot. Method: Seventy-eight patients with fibular hemimelia underwent 92 lengthenings and foot deformity correction. Equinovalgus foot deformity was corrected by four different methods in 67 cases: distraction, soft tissue release, release plus supramalleolar and/or subtalar osteotomy, and fibular transport. Results: Goals of lengthening and foot deformity correction were achieved in all cases. Foot deformity recurred in 19 patients and was retreated: 9/16 (56%) distraction cases, 4/18 (22%) soft tissue release cases, 2/28 (7%) release plus osteotomy cases, and 4/5 (80%) fibular transport cases. Genu valgum developed in many cases with no or partial anlage resection. Genu valgum did not develop in any cases with complete anlage resection. Final results based on functional and radiographic evaluation: 46 excellent, 28 good, 18 fair. Final result did not correlate with number of rays in foot. Conclusion: Limb length discrepancy and foot deformity can be successfully treated by simultaneous lengthening and foot deformity correction. Soft tissue release plus osteotomy and complete anlage resection yielded best results. Lengthening reconstruction surgery is an excellent alternative to ablative surgery and prosthetic fitting for patients with all severities of fibular hemimelia

Fibular hemimelia is associated with equinovalgus deformity of the ankle and hind foot and antero-medial bowing of tibia. A wedge shaped distal epiphysis of the tibia and tight posterolateral soft tissues play an important role in the pathogenesis of ankle valgus and lateral subluxation of foot. Tethering effect of fibular anlage may contribute to the deformities in the tibia and ankle. Lengthening procedures are associated with progression of these deformities. The purpose of this study is to determine whether Exner Osteotomy and Excision of Fibular anlage will correct the valgus deformity of the ankle and antero-medial bowing of tibia. A bending osteotomy through the distal tibial physis as described by Exner and excision of Fibular Anlage was performed in six limbs in five children (4 boys, 1 girl) with fibular hemimelia. Histology of Excised Fibular anlage was studied under light microscopy. The mean age at the time of surgery was twenty two months (range: 8 months to 5 years). The mean follow-up was two years and two months (13 months to 4 years and 8 months). Full Correction of ankle valgus and tibial bowing was achieved in three feet where, a cortical strut graft was used in the open wedge osteotomy. In two feet synthetic bone substitute was used. In these, tibial bowing corrected but slight ankle valgus remained. In one foot where synthetic bone substitute was used and the postoperative compliance with AFO was poor, bowing of tibia improved but ankle valgus recurred. Premature fusion of growth plate did not occur in any of the cases. Histology of fibular anlage showed replacement of bone tissue by mature collagen bundles surrounded by fibroconnective tissue. Exner Osteotomy and Excision of Fibular Anlage in Fibular Hemimelia corrects the ankle valgus and antero-medial bowing of tibia

Until recently the accepted treatment of choice for severe type-II fibular hemimelia has been Syme’s or Boyd’s amputation. The alternative of distraction lengthening using the Ilizarov technique is now available. We report three patients (four limbs) with type-II fibular hemimelia who were treated by the Ilizarov technique and followed up for two to six years. Severe progressive procurvatum and valgus deformity of the tibia and valgus deformity and lateral subluxation of the ankle were found in all four limbs. Multiple additional soft-tissue and bony surgery was necessary. In view of these problems we feel that reappraisal of the indications for lengthening in type-II fibular hemimelia is necessary

The purpose of the study was to evaluate the usefulness of the techniques introduced for correction of the deformities associated with fibular hemimelia. Material. 10 children (6 boys and 4 garils) with affected 11 limbs were analyzed. All presented Achterman-Kalamchi type II fibular hemimelia (absence of the fibula, anterior tibial bowing and hypoplastic foot). Limb length discrepancy ranged from 2 to 9 cm. Only 2 feet had 5 rays, 4 – 4 rays and 5 three rays. In 10 feet talo-calcaneal synostosis was diagnosed intra-operatively. Age at operation ranged from 7 to 23 months (mean 13.2). Follow-up was 4.7 years (1 – 8.5). Technique. Two groups of patients were analyzed. The 1st group consisted of 3 children (3 affected limbs) operated on by partial or complete release of the ankle. Correction of the equinus and valgus deformity was possible by rotation of the talus in the ankle joint in coronal and sagittal plain (the oval shape of talar dome allowed its rotation in the ankle joint). In 2 patients the tibial osteotomy were made as a separate procedure. The 2nd group consisted of 7 children (8 affected limbs) operated on by one-stage technique consisting of (1) trapezoid resection of the tibia for correction of anterior bowing and internal torsion (2) posterior and lateral release of the foot with lengthening of tendo Achilles and peroneals tendons (3) •opening wedge osteotomy through talo-calcaneal synostosis with bone graft taken from the tibia for correction of valgus and equinus deformity (4) skin plasty with subcuteneous flap for wound covering. In this group relationships between talus and tibia were not changed by operation (flat top talus). Results. Both techniques resulted in stabile and properly aligned tibia and hindfoot. Five children were treated later by Ilizarov method at age of 57 months (53 – 80). Other five patients walked independently in orthopaedic or normal shoes. Two of them wait for limb lengthening. The method used in the 2nd group was especially useful for patients with bilateral deformity. The relapse of hindfoot valgus deformity was observed after limb lengthening. Conclusion. One-staged correction of the complex deformity in fibular hemimelia is safe and cost effective. The treated limb was properly prepared for lengthening, wear-bearing in shoes (bilateral cases), orthosis or pros-thesis

Introduction: Fibular hemimelia (FH) is a congenital limb reduction deficiency characterised by partial or complete absence of the fibula and a spectrum of associated anomalies. For children with a major anticipated limb length discrepancy and severe foot deformity, management (amputation or limb reconstruction) is controversial. Materials and Methods: 8 children who are now adults (average age 28 years) underwent limb reconstruction as children in one of two UK centres for severe fibular hemimelia. All 8 participants were recalled to our institution for instrumented gait analysis. The SF-36 and lower limb domains of the Toronto Extremity Salvage Score (TESS) questionnaires were also administered. Results: Partcipants scored well for general health but had functional limitations reflected in lower TESS scores. Kinematic analysis revealed decreased sagittal knee motion and valgus knee alignment. Also ubiquitous were anterior pelvic tilt and obliquity with incomplete hip extension and reduced range of hip abduction. Kinetic analysis showed reduced peak plantar flexion moment with reduced push-off power and an internal hip adduction moment in late stance. These parameters are compared to control data for below knee amputees. Discussion and conclusions: Although the number of participants is small, this is the first study to use instrumented gait analysis for severe fibular hemimelia managed with limb reconstruction. The results add objective data to the debate over limb reconstruction or amputation in this group of children

We looked at the outcome of management of 16 patients (19 limb segments) with congenital fibular hemimelia treated in our unit over a 24-year period from 1978 to 2001. Eight boys and eight girls, all with associated musculoskeletal abnormalities in the lower limbs, were presented for management at or before the age of six months. On four patients no surgery was performed. In the other 12, orthopaedic management was completed during the skeletal growth period. Primary amputations (one below-knee, one Syme and one Boyd) were performed on three patients and prostheses fitted in early childhood. Three patients with bilateral fibular hemimelia were treated initially with a Gruca ankle reconstruction procedure. Using the Ilizarov technique, we performed tibial lengthening procedures on nine patients. At the latest follow-up, the three patients who had amputations were functioning well and had no complications. The nine patients in whom tibial lengthening was the main reconstructive procedure suffered numerous complications and all needed further corrective surgery or footwear alterations. None required or requested late amputation because of poor function or cosmesis. Analysing results by parameters such as restriction of activity, pain, complication rate, treatment costs, hospital and clinic visits, periods of absence from school, and patient satisfaction, we found notably better results among patients who underwent early primary amputation than among those who underwent tibial lengthening. This needs to be kept in mind when advising parents of the most appropriate course of management of their child’s disorder

Introduction & Aim: The usual clinical presentation in Fibular hemimelia involves equinovalgus deformity of the foot and ankle instability with absence of the lateral rays of the foot. The aim of this study is to evaluate the results of ankle joint reconstruction, using remnants of the fibula, fibular analge or contra lateral fibular graft, in conjunction with the Ilizarov Technique. Methods: We reviewed 13 limb segments in 12 patients with fibular hemimelia, with an average age of 4.7 years. According to Catagni’s classification 2 limbs were type I, 1 limb was type II and 10 limbs were type III. The ankle joint was reconstructed using remnants of the fibula if present in type I, fibular analge or a contra lateral fibular graft. The Ilizarov technique was then used to correct limb length discrepancy and any concomitant deformities. Results: The results were assessed by the satisfaction of patients and families, the functional outcome in terms of daily activities and clinical examination of the patients. A satisfactory stability of the ankle foot complex was achieved in all patients. The average lengthening achieved using the frame was 5.6 cm and all limbs were equalized to within 2 cm. Conclusion: Reconstruction of the ankle joint bring the foot in good position, preserves the ankle joint motion, facilitate fitting shoes and stabilize the joint in a more normal way compared to distal tibial osteotomies. The ilizarov technique corrects the concomitant deformity and achieves an equal limb length. The combination of both techniques provides a better outcome compared to other treatment modalities

Introduction: The usual clinical presentation of fibular hemimelia is of leg discrepancy, an equinovalgus deformity of the foot, ankle instability and the absence of the lateral rays of the foot. The aim of this study is to evaluate the results of ankle joint reconstruction, using remnants of the fibula, fibular analge or contra lateral fibular graft, in conjunction with the Ilizarov Technique. Methods: Thirteen limb segments in 12 patients with fibular hemimelia were reviewed, with an average age of 4.7 years. According to Catagni’s classification 2 limbs were type I, 1 limb was type II and 10 limbs were type III. The ankle joint was reconstructed using remnants of the fibula if present in type I, fibular analge or a contra lateral fibular graft. The Ilizarov technique was the used to correct limb length discrepancy and any concomitant deformities. Results: The functional outcome was assessed by the ability to undertake daily activities. All cases were clinically examined and the satisfaction of the patients and family were assessed. A satisfactory stability of the ankle foot complex was achieved in all patients. The average lengthening achieved using the frame was 5.6 cm and all limbs were equalized to within 2 cm of the contralateral side. Discussion: Reconstruction of the ankle joint brings the foot into a good position, preserves the ankle joint motion, facilitates fitting shoes and stabilizes the joint in a more normal position compared to distal tibial osteotomies. The Ilizarov technique corrects the concomitant deformity and achieves a near normal limb length. The combination of both techniques provides a better outcome compared to other treatment modalities

Purpose of the study. We describe a new technique of talar dome osteotomy in the treatment of fixed equinovalgus deformity of the foot in patients with Fibular Hemimelia and successfully applied it in two patients. Background. Fibular Hemimelia is a congenital absence or hypoplasia of fibula with associated fixed equinovalgus deformity of the foot. Treatment for this deformity ranges from corrective osteotomy of the tibia, calcaneum to Syme's amputation. Methods. The procedure of talar dome osteotomy is best applied to children before they start to walk. Through a Cincinnati approach, fibular anlage was excised and a talar dome osteotomy performed in the axial plane to correct the valgus deformity of the ankle. Additional procedures if required include corrective osteotomy of the distal tibia to correct remaining foot deformity after the initial correction, and tendo achillis lengthening. The corrected position is then maintained with a K-wire inserted through the calcaneum, osteotamised talus up into the distal tibia. K-wire was removed at 6 weeks and foot position thereafter maintained in an AFO orthrosis with the foot slightly inverted for next 2 years. Two patients diagnosed with fibular hemimelia (Coventry and Johnson type II) underwent correction of their fixed equino-valgus deformity with the above mentioned technique at the ages of 6 and 10 months respectively. AFO orthosis was used for two years and at 5 years of follow-up the deformity has remained corrected in both the ankles. Both these patients are due to undergo limb-lengthening procedures

Introduction. Congenital deficiency of the fibula frequently presents as spectrum of musculoskeletal anomalies involving the ipsilateral hip, femur, knee, tibia/fibula, ankle and foot. Until recently the treatment of choice for sever type-II fibular hemimelia has been Syme’s or Boyd’s amputation. The present technique of limb lengthening with distraction osteogenesis have proved to be a valid alternative. The study shows that simultaneous treatment of tibial and foot deformities allows the patient to obtain a plantigrade foot and to avoid the prosthetic choice of treatment. Materials and methods. 12 patients with 15 involved extremities underwent tibial lengthening and correction of the foot deformities for congenital tipe II fibular hemimelia with Ilizarov apparatus. There were 10 boys and 2 girls, range 7 years 3 month to 16 years 2 month (mean 10 years 7 month). The mean follow up time was 28 months ( range 15–63 month). Most of the patients had hypoplasia of the lateral femoral condyle and femoral shortening and simultaneous lengthening of femur in 9 cases was performed. Valgus-procurvatum deformity of tibia was present in all cases, absent lateral rays were present in 8 feet, foot coalition in 5 feet. Results. Lengthening of the tibia was performed at one level in 4 cases In the other 11 it was performed simultaneously with a proximal osteotomy of tibia to correct thevalgus and with a supramalleolar osteotomy to obtain axial realignment of ankle. Correction of the foot deformities was performed by closed method in 5 cases with overcorrecton in varus-adduction and plaster cast. Subtalar osteotomy in the presence of coalition was performed in 5 cases, osteotomy of calcaneus for equinus in3, and in 2 cases osteotomy through rigid subtalar joint. Osteotomy of midfoot for abducted and equines forefoot was performed in 3 cases. In two difficult rigid cases ankle arthrodesis was needed to stabilise the foot. Prophylactic anlage excision with soft tissue release and Achilles-tendon lengthening in 13 cases. Good results were achieved in 12 cases. 2 were successful and one poor because the patient refused continuing treatment with external fixator. There were 5 major and 16 minor complications. Complications involving delayed consolidation, bending or deformation of regenerated bone, early consolidation were observed in 4 cases. Complications involving soft tissue were observed in 9 cases, There were no permanent neurological and vascular injures. Conclusions. The Ilizarov technique provides a means of achieving simultaneous lengthening of the femur and tibia, angular and rotational deformities correction in children with congenital type II fibular hemimelia. This method should be combined with simultaneous ankle and foot reconstruction for correction of eqininovalgus deformity associated instability and subluxation of ankle. This method should be combined with simultaneous ankle and foot reconstruction for correction of eqininovalgus deformity associated instability and subluxation of ankle. Various types of osteotomy of the hind and mid food give the possibility to achieve the stable result of correction

Fibular Hemimelia is not just a fibular anomaly but there is entire limb involvement with varied expression in each segment. Factors which we have considered in treatment are the amount of fibula present, percentage of shortening, tibial and leg deformity and foot deformity. Residual or recurrent foot deformity is the prime reason for unsatisfactory results, so we have used Paley's classification which takes into consideration foot deformity. Our series is of 29 cases, Paley type I-7, Type II-6, Type III-16 and none of type IV. Tibial lengthening (+/−) bow correction was performed in 28 cases. Supramalleolar osteotomy was done in 4 cases. In foot, soft tissue release only was done in 6 cases and soft tissue release with osteotomy (subtalar or calcaneal) was done in 14 cases. Amputation was done in 2 cases. Age ranged from 11 months to 16 years. Mean follow up was 4.2 years. Mean lengthening was 3.5 cm. Desires lengthening was achieved in 21/29 cases and plantigrade foot was achieved in 16/29 cases. Complications faced were recurrence of foot deformity, knee valgus, knee fixed flexion deformity, knee subluxation and pin tract problems. Less than 3 rays and more than 25 cm of limb length discrepancy were poor prognostic factors. We had 7 excellent, 16 good and 6 poor results. To conclude, it is difficult to achieve the aim of plantigrade foot and limb length equality in all cases but radical surgery with foot correction and tibial lengthening can give good results

Amputation vs. limb salvage in FH has been based on fibular presence or absence and a ‘good’ or ‘bad’ foot. None of the current FH classification systems address ankle joint, hindfoot and forefoot morphology. We present a new, comprehensive FH classification which delineates leg, ankle and foot morphology. Three major groups are proposed; I-mild fibular shortening; II-small or miniature fibula; III-absent fibula. Ankle mortise morphology is defined as H=horizontal; S=spherical; V=valgus. A small “c” denotes a tarsal coalition. Numerals 1–5 reflect the number of forefoot rays present. For example, a patient with a miniature fibula, valgus ankle, tarsal coalition and 4 rays would be classified as II Vc4.

Thirty-two limbs in 31 FH patients were assessed by teleoroentgenograms, weight-bearing ankle and foot radiographs and examination. All had shortened femora, the amount of which did not correlate with fibular type. Type III fibulae were highly associated with valgus ankles (56%), decreased number of rays (46–100%), and tarsal coalition (69%). Coalition was found in all ray categories but diminished number of rays (42–100%) with associated valgus ankles (68%) correlated strongly with a coalition. In patients with type III fibulae, one-third had horizontal ankles, 53% had 4 or 5 rayed feet and 30% had no coalition. Fibular absence did not correlate with percent tibial shortening or ankle valgus.

We present a reproducible classification which reflects the spectrum of ankle and foot involvement seen in review of 32 FH cases. Early amputation is recommended for limbs with fewer than 3 rays. Twenty-seven patients underwent limb reconstruction and 4 had ankle disarticulation and required adjunctive bony and soft tissue procedures. Extension of the fixation to the foot should be done during tibial lengthening in FH.

Introduction

The purpose of our study was to retrospectively analyze our patients who were treated for FH and PFFD by means of guided growth temporary Hemiepiphysiodesis.

We sought to determine the effectiveness of the procedure, as well as its success rates, complications, and rebound phenomena.

Amputation vs. limb salvage in FH has been based on fibular presence or absence and a ‘good’ or ‘bad’ foot. None of the current FH classification systems address ankle joint, hindfoot and forefoot morphology. We present a new, comprehensive FH classification which delineates leg, ankle and foot morphology. Three major groups are proposed: I-mild fibular shortening; II small or miniature fibula; III-absent fibula. Ankle mortise morphology is defined as H=horizontal, S=spherical, V=valgus. A small ‘c’ denotes a tarsal coalition. Numerals 1–5 reflect the number of forefoot rays present. For example, a patient with a miniature fibula, valgus ankle, tarsal coalition and 4 rays would be classified as II Vc4.

We present a reproducible classification which reflects the spectrum of ankle and foot involvement seen in review of 31 FH cases. Early amputation is recommended for limbs with fewer than 3 rays. Twenty-seven patients underwent limb reconstruction and 4 had ankle disarticulation and required adjunctive bony and soft tissue procedures. Extension of the circular fixation to the foot should be done during tibial lengthening in FH.

Thirty-two limbs in 31 FH patients were assessed by teleoroentgenograms and weightbearing ankle and foot radiographs. All had shortened femurs, the amount of which did not correlate with fibular type. Type III fibulae were highly associated with valgus ankles (56%), decreased number of rays (46–100%), and tarsal coalition (69%). Coalition was found in all ray categories but diminished number of rays (42–100%) with associated valgus ankles (68%) correlated strongly with a coalition. In patients with type III fibulae, one third had horizontal ankles, 53% had 4 or 5 rayed feet and 30% had no coalition. Fibular absence did not correlate with percent tibial shortening or ankle valgus.