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Orthopaedic Proceedings
Vol. 90-B, Issue SUPP_I | Pages 29 - 29
1 Mar 2008
Saldanha K Fernandes J Bell M Saleh M
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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


Orthopaedic Proceedings
Vol. 86-B, Issue SUPP_III | Pages 236 - 236
1 Mar 2004
Saldanha K Bell M Fernandes J Saleh M
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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


Orthopaedic Proceedings
Vol. 87-B, Issue SUPP_III | Pages 317 - 317
1 Sep 2005
Paley D Saghieh S Song B Young M Herzenberg J
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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


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_I | Pages 8 - 8
1 Jan 2011
Saldanha KAN Nayagam S
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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


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 278 - 278
1 Mar 2003
Napiontek M Shadi M
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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


Orthopaedic Proceedings
Vol. 92-B, Issue SUPP_III | Pages 372 - 373
1 Jul 2010
Thomas S McCahill J Stebbins J Bradish C McNally M Theologis T
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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


Orthopaedic Proceedings
Vol. 84-B, Issue SUPP_I | Pages - 80
1 Mar 2002
Erken E
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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


Orthopaedic Proceedings
Vol. 88-B, Issue SUPP_I | Pages 125 - 125
1 Mar 2006
Shalaby H Hefny H Thakeb M El-kawy S Elmoatasem E
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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


Orthopaedic Proceedings
Vol. 87-B, Issue SUPP_III | Pages 396 - 396
1 Sep 2005
Hefny PH Thakeb M El-kawy S Shalaby H Elmoatasem E
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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


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_VI | Pages 12 - 12
1 Mar 2012
Akula M Madhu T Scott B Templeton P
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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


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_III | Pages 268 - 269
1 Mar 2003
Kirienko A Portinaro N
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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


Orthopaedic Proceedings
Vol. 95-B, Issue SUPP_1 | Pages 148 - 148
1 Jan 2013
Singh N Kulkarni R Kulkarni G
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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


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_II | Pages 162 - 162
1 Feb 2003
Stanitski D
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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.


Orthopaedic Proceedings
Vol. 103-B, Issue SUPP_6 | Pages 49 - 49
1 May 2021
Gigi R Kurien B Giles S Fernandes J
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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.

Materials and Methods

We retrospectively reviewed the medical records and all routine preoperative and post operative long standing radiograph of all the FH and PFFD patients that were operated in our institute using guided growth hemiepiphysiodesis technique of distal femur or proximal tibia between 2007 to 2017.


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_II | Pages 110 - 110
1 Feb 2003
Stanitski D
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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.


Orthopaedic Proceedings
Vol. 105-B, Issue SUPP_18 | Pages 2 - 2
1 Dec 2023
Basheer S Kwaees T Tang C Ali F Haslam P Nicolaou N
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Objectives. Congenital cruciate ligament deficiency is a rare condition that may occur in isolation or in association with longitudinal limb deficiencies such as fibular hemimelia or proximal femoral focal deficiency. Often anomalies of the menisci and their attachments can be very abnormal and impact on surgical management by standard techniques. Arthroscopic surgical knee reconstruction is undertaken to improve symptomatic instability and/or to stabilise and protect the knee for future planned limb lengthening surgery. The aim of this study is to evaluate the arthroscopic findings of patients undergoing surgery for congenital cruciate ligament deficiency, and specifically to determine the frequency and types of meniscal anatomical variations seen in these cases. Methods. Patients undergoing surgery for congenital cruciate ligament deficiency were identified from a prospectively collated database. Diagnosis was confirmed through review of the clinical notes and imaging. Operative notes and 4K saved arthroscopic images and video recordings for these cases were reviewed. Results. Over a six-year period (July 2017 – September 2023), 42 patients underwent surgery for congenital ligament deficiency and tibiofemoral instability (45 surgical episodes). Median age of patients at time of surgery was 10 years (range 4 – 17 years). The most frequent diagnosis was congenital longitudinal limb deficiency syndromes in 27 cases, with the most frequent being fibular hemimelia. Isolated congenital ligament deficiency without any other associated extra-articular manifestations occurred in 11 cases. Absence of meniscal root attachments or hypertrophy of meniscofemoral ligaments acting as ‘pseudo-cruciates’ were seen in over 25% of patients. In isolated ACL deficiency these were injured causing onset of instability symptoms and pain following trauma. Often these abnormal structures required addressing to allow surgical reconstruction. Conclusions. Our findings demonstrate that there are often meniscal variations seen in association with congenital absence or hypoplasia of the cruciate ligaments. In these patients hypertrophied meniscofemoral ligaments may act as cruciate-like structures and play a role in providing a degree of sagittal plane stability to the knee. However, when the knee becomes unstable to the point that cruciate ligament reconstruction is indicated, these meniscal variants may often require stabilisation using complex meniscal root repair techniques or variations to standard cruciate ligament reconstruction techniques to accommodate the variant anatomy


Orthopaedic Proceedings
Vol. 84-B, Issue SUPP_III | Pages 210 - 210
1 Nov 2002
Lincoln T Mack P Birch J
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Introduction: Current classification schemes of fibular hemimelia concentrating on the radiographic appearance of the fibula do not adequately aid management of these patients. We reviewed our fibular hemimelia patient experience to devise a classification scheme which could serve as a better guideline for management decisions for this disorder. Method: We reviewed the records/radiographs of all patients with the diagnosis of fibular hemimelia treated at our institution between 1957 & 1996. We excluded patients with PFFD, inadequate radiographs, or whose treatment was initiated elsewhere. We proposed a clinical management-oriented classification based on the presence/absence of a functional foot and overall limb shortening relative to the contralateral side (irrespective of the relative contributions of femoral & tibial shortening, or bilateral disease). The classification (with treatment guidelines) is:. Type I. Functional foot. Shortening 5% or less (none or epiphysiodesis). Shortening 6–10% (epiphysiodesis or lengthening). Shortening 11–30% (1–2 lengthenings). Shortening > 30% (multiple lengthening or amputation). Type II. Non-functional foot. Upper extremities functional (amputation). Foot needed for prehension (no treatment). A functional foot was defined as one which was or could be made plantigrade and stable with at least three rays. Results: We identified 146 extremities in 122 patients with fibular hemimelia. 117 extremities were Type I (53 IA, 32 IB, 29 IC, and 1 ID), and 29 were Type II (28 IIA and 1 IIB). Limb length inequality remained proportional throughout growth, so that the distribution between groups did not change during growth. Twenty-four patients had bilateral involvement. Twenty of 48 feet in these patients were Type II (nonfunctional). Nine patients with bilateral involvement had bilateral functional feet; these patients were short-statured, but were Type IA functionally. The number of rays correlated directly with function: 100% of 5-rayed, 90% of 4-rayed, and 64% of 3-rayed feet were salvaged in this series. No feet less than 3-rayed were salvaged in this group; all such feet were associated with a completely absent fibula. Thirty-six of 63 limbs with completely absent fibula were classified as Type I. Eighty-two extremities in 65 patients have completed definitive management by virtue of skeletal maturity or amputation. A total of 39 extremities underwent Syme amputation, including 1 of 30 type IA, 1 of 11 type IB, 9 of 12 type IC, the single patient type ID, and all 27 type IIA deformities. Conclusions: This classification system correlated well with the treatment required in our patients. While the amount of fibula present correlated with limb length inequality, 56% of patients in this series with absent fibula have been managed with limb salvage. The definition of a functional foot, and the boundary between multi-staged lengthening and amputation will remain an individual decision, but our classification scheme accurately predicted the amount of deformity present and the treatment required


Orthopaedic Proceedings
Vol. 106-B, Issue SUPP_5 | Pages 25 - 25
23 Apr 2024
Aithie J Oag E Butcher R Messner J
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Introduction. Genu valgum is a common presentation in paediatric patients with congenital limb deformities. The aim of this study is to assess the outcome of guided growth surgery in paediatric patients referred via our physiotherapy pathway with isolated genu valgum and associated patellar instability. Materials & Methods. Patients were identified from our prospective patellar instability database. Inclusion criteria was acquired or congenital genu valgum associated with patellar instability in skeletally immature patients. The mechanical lateral-distal femoral angle was assessed on long leg alignment radiographs (mLDFA <85 degrees). Surgical treatment was the placement of a guided growth plate (PediPlate, OrthoPediatrics, USA) on the medial distal femoral physis (hemi-epiphysiodesis). KOOS-child scores were collected pre-operatively and post-operatively (minimum at 6 months). Results. Eleven patients (seven female) with mean age of 12(range 5–15) were identified. Five patients had congenital talipes equinovarus(CTEV), one fibular hemimelia, one di-George syndrome, one septic growth arrest and three had idiopathic genu valgum. Pre- and post-operative KOOS-child scores showed overall improvement: 58(range 36–68) to 88(65–99) and knee symptoms subscores: 64(43–71) to 96(68–100) p<0.01, t-test. Mean follow-up was 10 months (range 3–23). No subsequent dislocations/subluxations occurred during follow-up. Conclusions. Guided growth surgery is an effective way of treating symptomatic patellar instability in skeletally immature patients with genu valgum in the absence of other structural pathology. It was most common in our cohort in patients with unilateral CTEV. We would recommend to screen syndromic and congenital limb deformity patients for patellar instability symptoms in the presence of genu valgum


Orthopaedic Proceedings
Vol. 104-B, Issue SUPP_5 | Pages 18 - 18
1 Apr 2022
Varasteh A Gangadharan S James L
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Introduction. Amputation or disarticulation is a reliable option for management of severe foot deformities and limb-length discrepancies, the surgical restoration of which are unpredictable or unfavourable. Of the various surgeries involving foot ablation, Syme's amputation is preferred for congenital deformities as it provides a growing, weight bearing stump with proprioception and cushioning. Materials and Methods. We reviewed data of all children who underwent Syme's amputation over the past 13 years at our institution. Surgical technique followed the same principles for Syme's but varied with surgeons. Results. Ten boys and ten girls, with an average age of 18 months and average follow up of 70 months were included in the study. The most common indication was fibular hemimelia. Wound complications were reported in three children, phantom pain in one, heel pad migration in two. None had wound dehiscence, flap necrosis, stump overgrowth, or calcaneal regrowth. None of this required surgical intervention. One child required an amputation at a higher-level secondary to a congenital malformation of nervous tissue in the affected leg. Prosthetic compatibility was 94.7 % and none used mobility aids. Six children participated in sports. Conclusions. Syme amputation is a safe and potentially advantageous procedure in children, with a low incidence of complications to offer patients with non-salvageable foot conditions. It offers good prosthetic use with minimal risk of complications and can offer patients a functional solution with only one surgical intervention throughout their childhood


Orthopaedic Proceedings
Vol. 96-B, Issue SUPP_18 | Pages 9 - 9
1 Dec 2014
Aranganathan S Carpenter C Thomas D Hemmadi S O'Doherty D
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Complex congenital foot deformities pose a challenge to the surgeon due to poor results after extensive surgery. We report the clinical outcomes of children with complex congenital foot deformities treated with UMEX® (Universal mini-external fixator System) frames. This is a prospective review of our experience in patients treated in this way, from 2004 to 2011. The indications for treatment included resistant/recurrent Congenital Talipes Equino Varus (CTEV), cavo-varus deformity secondary to Charcot-Marie-Tooth disease, arthrogryposis, fibular hemimelia and other congenital abnormalities. A total of 32 children (35 feet) have been treated, out of which 22 were male and 10 were female patients. Age at surgery ranged from 3 to 15 years (median age – 7 years). Three patients underwent bilateral procedures; the reminder (29 patients) underwent unilateral foot operations. Twenty-eight patients had undergone previous surgery including soft-tissue and/or bony corrective procedures. The frames were removed at an average of 69 days after application, and the patients spent a further 6 weeks in a walking cast. Good functional outcomes were noted in 26 patients in the first postoperative year and in 19 patients in the fifth postoperative year. Further operations were needed in 10 patients. Complications occurred in 10 patients, predominantly pin-site infections and 1 case of bony overgrowth at pin-site and 1 of proximal tibio-fibular diastasis. This is a simple fixator to use with a short learning curve. In groups of patients with complex congenital abnormalities, we achieved good functional outcome with low-complication rates