Foot and Ankle Deformities in Fibular Hemimelia: Narrative Review.

Objective: To review indications for limb amputation or reconstruction using the SUPERankle procedure in patients diagnosed with Paley type 3 or 4 fibular hemimelia; To evaluate the correction of deformities needed to achieve a stable plantigrade foot, review variations of the original technique and their applicability, and describe challenges encountered in their execution. Methods: Qualitative, retrospective, descriptive study of 4 patients who underwent the SUPERankle procedure between 2019 and 2020 for treatment of foot and ankle deformities in fibular hemimelia. Pre- and postoperative clinical and radiographic evaluations were performed to identify objective evidence of the correction of foot and ankle deformities. The operative techniques employed are described. Results: The included patients met anatomical and psychosocial criteria for reconstruction, as established in the literature. Clinical correction of foot and ankle deformities was achieved, but on radiographic evaluation, there was no statistical difference; this was likely due to the small number of patients, given the rarity of fibular hemimelia. Conclusion: The procedure is reproducible, but requires a knowledgeable, well-trained surgical team. Correction of foot and ankle deformities can be achieved. The choice between amputation or reconstruction should be the result of an informed decision by Family members, supported by a multidisciplinary team. Level of Evidence IV; Qualitative Study; Case Series.

Purpose: The aim of this study is to evaluate foot deformities and anomalies present in congenital limb deficiencies (CLDs). We sought to define the relationship between the type of limb deficiency and foot posture, patterns of ray deficiencies, and association between tarsal coalition (TC) and ball-and-socket ankle. Materials and Methods: This is a single institution, retrospective radiograph, and medical record review of patients with CLD, comprising congenital femoral deficiency (CFD), tibial hemimelia (TH), and fibular hemimelia (FH) from January 2000 to January 2019. Data extracted included patient demographics, predicted leg length discrepancy, associated deformities and anomalies, and specifics of the foot deformity. Surgical procedures were recorded. Data were reported using descriptive statistics. Fisher's exact test analyses of contingency tables were used for the exploratory components of this study. Results: Eighty-one patients with 97 limb deficiencies were identified (16 – CFD, 18 – TH, and 63 – FH). Isolated CFD was not associated with foot and ankle deformity and TH was associated with an equinocavovarus foot in all limbs. In FH, an equinocavovarus deformity was present in 15/63 (24%) feet, most requiring foot surgery. Of 48 patients with FH and absent rays, two lacked lateral rays, whereas the remainder missed one or more intermediate rays. TC was unrelated to the presence of ball-and-socket ankle. Conclusions: This study demonstrates heterogeneity in the spectrum of foot and ankle deformity in CLD, particularly in FH. Recognition of this phenotypic variation is critical for surgeons to formulate a comprehensive treatment plan and ensure optimal functional outcomes. Level of Evidence: IV.

Purpose To assess the midterm results of acute centralization of the foot in cases of tibial and fibular hemimelia associated with severe foot deformity and ankle dislocation.Patients and methods We treated 11 limbs in 10 patients: eight patients with tibial hemimelia and two patients with fibular hemimelia. All cases were associated with severe foot deformity (equinovarus in tibial hemimelia and equinovalgus in fibular hemimelia), dislocated ankle, and tight tendoachilis. There were seven boys and three girls, with a mean age of 2.35 years. In tibial hemimelia, two limbs were type Ia, four limbs were type II, and three limbs were type IV according to Jones classification. In fibular hemimelia, all cases were type II according to Achterman and Kalamachi classification. The mean preoperative limb-length discrepancy was 5.4 cm (range, 4.5–7 cm). We had acute centralization of the foot by means of calcaneofibular or tibiocalcaneal arthrodesis in cases of tibial or fibular hemimelia, respectively, and talectomy, fibular shortening, and tibiofibular synostosis were done. Transcalcaneal fibular Kirschner wire was inserted to maintain the foot position. First-stage lengthening by Ilizarov fixator was done, with 5-cm gain of length in seven cases, and second-stage lengthening was done in two cases.Results The mean follow-up period was 50 months. We achieved foot correction and centralization of the foot, which was stable in all cases, except one with failed tibiofibular synostosis in Jones type IV. No neurovascular complications occurred. Wound dehiscence occurred in five limbs, unstable knee was seen in two cases, recurrent equinus of more than 20° was seen in three cases, and two cases had poor regenerate potential. All patients and families were satisfied with the foot procedure and were not satisfied with knee centralization procedure owing to knee instability.Conclusion Acute centralization of the severely deformed foot in cases of tibial and fibular hemimelia by calcaneofibular or tibiocalcaneal arthrodesis, respectively, can correct severe foot deformity, achieve plantigrade foot, and preserve the patient but was associated with many secondary procedures.Level of evidence Level IV.

Fibular hemimelia is a congenital disorder that is characterized by the absence of the fibula that could be either partial or complete. Successful management aims to restore normal weight bearing and normal limb length. The introduction of the Ilizarov method of limb lengthening has provided an attractive alternative to amputation. During lengthening, the tight posterolateral soft-tissue structures, the thick fibrous fibular band, and the shortened Achilles tendon become tighter and transfer a valgus force to the talus and calcaneus, further aggravating the deformity. We have developed a strategy to address this in patients with Paley type III fibular hemimelia via ankle reconstruction that provides posterolateral stability and buttressing of the ankle and hind foot by reconstructing the lateral buttress. This is achieved through excision of the fibrous fibular anlage, centralization of the ankle, restoring talocalcaneal coronal alignment, and reconstruction of the lateral malleolus by transplanting the cartilaginous remnant of the lateral malleolus or by crafting a bone block autograft taken from the iliac crest or tibia. A prospective non-randomized clinical trial included ten ankles in eight patients with fibular hemimelia Paley type III (two patients had bilateral deformity). The patients' ages ranged from 7 to 36months. After a follow-up ranging from 48 to 96months, a stable plantigrade foot was achieved in nine ankles; one ankle had residual equinus, five ankles had residual valgus heel, and eight ankles had complete range of motion of the ankle, whereas one patient lost 5° of dorsiflexion. One ankle had equinus deformity. To achieve satisfactory results, a stable plantigrade foot and ankle is necessary in patients with fibular hemimelia before attempting to equalize limb length discrepancy. It is important to reconstruct the ankle through an extra-articular soft tissue release, anlage resection, osteotomies, and restoring the abnormal talocalcaneal relationship before any attempt to equalize LLD.

Management of ankle joint deformity and instability are challenging issues in congenital fibular hemimelia (FH). This study aims to assess how much the SUPERankle procedure improves ankle alignment and provides durable ankle stability in patients with severe FH. Seventeen children aged 53.4±44.1 months with severe form of FH, equinovalgus foot deformation, ankle instability, and tibial curvature (Paley type IIIC), affecting 19 limbs, underwent the SUPERankle procedure. Foot and ankle position was evaluated clinically and radiologically before surgery, immediately after, and at follow-up of 63.0±19.7 months. Mechanical lateral distal tibial angle (mLDTA), tibiocalcaneal angle (mTCA), and tibiocalcaneal distance (mTCD) were measured on the AP radiograms, while the anterior distal tibial angle (mADTA) and lateral tibiocalcaneal angle (mLTCA) were measured on the lateral radiograms. Recurrences, additional procedures, and complications were documented based on medical records. Quality of life was evaluated with Limb Deformity-SRS questionnaire. On clinical examination, the normal tibia and ankle alignment, along with a plantigrade foot were achieved in all limbs after the first surgery. In 11 limbs (58%) this result was maintained at follow-up. Due to recurrence, additional procedures were necessary to provide durable ankle alignment in 7 limbs (37%), while in 1 limb (5%) the ankle joint remained in equinus at the last follow-up. Significant improvement of radiologic alignment was found in all parameters (preoperative vs. postoperative vs. FU) as follows-mLDTA: 71.4±11.2 versus 88.7±5.6 versus 88.1±2.7 degrees, P =0.0001; mTCA: 41.4±14.9 versus 8.7±8.4 versus 11.6±8.9 degrees, P =0.0001; mTCD: 22.3±7.9 versus 4.0±3.6 versus 7.7±6.5mm, P =0.0001; mADTA: 99.5±19.4 versus 82.3±4.2 versus 81.5±5.9 degrees, P =0.0002; mLTCA: 116.7±23.9 versus 95.8±11.7 versus 93.5±15.1 degrees, P =0.0002. The mean follow-up LD-SRS score was 4.03. In children with severe fibular hemimelia, the SUPERankle procedure provided clinically and radiologically fully corrected ankle joint and plantigrade foot, suitable for further lengthening procedure. The 40% rate of deformity recurrence was managed with additional surgical intervention to achieve a good clinical, radiologic, and functional outcome in 95% of children at 5-year follow-up. Level IV.

Fibular hemimelia (FH) represents the most common deficiency of the long bones and is associated with multiple deformities. Reconstructive treatment with external fixators in FH restores normal lower extremity alignment and length with plantigrade feet for a balanced and effective gait. The aim of this study is to evaluate the outcomes of lower limb lengthening and simultaneous tri-plane deformity correction with a computer-assisted hexagonal external fixator in children with FH. A retrospective review was performed for FH cases treated with a computer-assisted hexagonal external fixator in a tertiary referral center. Leg length discrepancy (LLD), interphyseal angles, tibiocalcaneal distances, healing index (HI), and callus shapes were analyzed for radiologic evaluation, and the Pediatric Quality of Life Inventory (PedsQL) was used for functional assessment. Limbs with HI <50 days/cm, PedsQL >75, and without regenerate fractures were considered successful lengthenings. Twenty-four limbs of 23 patients were included. The limbs were lengthened for a mean of 7.24cm (range, 4.7 to 15.6). The initial LLD of 5.6cm (range, 0.5 to 19cm) increased to 1.7cm (range, 0.1 to 6cm), and the mean interphyseal angle was 12.7 degree (range, 1.5 to 54.2 degree), tibiocalcaneal distance was 0.85cm (range, 0.1 to 1.7) at final follow-up. The most common regenerated bone morphology was cylindrical, as seen in 11 limbs (45.8%). The average PedsQL score was 83.5 (range, 69.5 to 96.7). Sixteen limbs (66.7%) had successful lengthening at their first, and 4 limbs (80%) had successful lengthening at their second surgeries. Seven limbs had complications requiring surgical intervention (29.1%), with 3 (12.5%) regenerate fractures after external fixators removal. Limb reconstruction with computer-assisted hexapod fixators is a successful and reliable option for the treatment of LLD in FH, and patients demonstrate good functional outcomes. Surgeons should be aware of potential complications and should utilize prophylactic measures when necessary. Level III, retrospective comparative study.

Fibular hemimelia is a rare but the most common congenital long bone deficiency, encompassing a broad range of anomalies from isolated fibular hypoplasia up to substantial femoral and tibial shortening with ankle deformity and foot deficiency. Most cases of fibular hemimelia manifest clinically significant leg length discrepancy (LLD) with time that requires adequate correction by bone lengthening for stable walking. Bone lengthening procedures, especially those for pathological bones, are sometimes associated with severe complications, such as delayed consolidation, fractures, and deformities of the lengthened bones, leading to prolonged healing time and residual LLD at skeletal maturity. The purpose of this study was to review our clinical results of lower limb lengthening for fibular hemimelia.This study included 8 Japanese patients who diagnosed with fibular hemimelia from physical and radiological findings characteristic of fibular hemimelia and underwent single or staged femoral and/or tibial lengthening during growth or after skeletal maturity. LLD, state of the lengthened callus, and bone alignment were evaluated with full-length radiographs of the lower limb. Previous interventions, associated congenital anomalies, regenerate fractures were recorded with reference to medical charts and confirmed on appropriate radiographs. Successful lengthening was defined as the healing index <50 days/cm without regenerate fractures.A significant difference was observed in age at surgery between successful and unsuccessful lengthening. The incidence of regenerate fractures was significantly correlated with callus maturity before frame removal. LLD was corrected within 11 mm, whereas mechanical axis deviated laterally.Particular attention should be paid to the status of callus maturation and the mechanical axis deviation during the treatment period in fibular hemimelia.

Children with fibular hemimelia (FH) present with a short lower leg of varying degrees of severity, often accompanied by a wide range of associated anomalies of the ipsilateral foot, ankle, and knee. Mild cases need only appropriately timed contralateral epiphysiodesis. Moderate cases require tibial lengthening, with realignment of the ankle in selected cases. For severe cases, the choice comes down to amputation versus limb salvage. One of the biggest challenges in limb salvage is to obtain a plantigrade foot. For the stiff equinovalgus ankle (the most common severe manifestation of FH), special reconstruction techniques are recommended to achieve a plantigrade foot. The knee may require anterior or posterior cruciate ligament reconstruction. Knee valgus is often treated by growth modulation. Associated congenital femoral deficiency should be addressed, either with lengthening or with contralateral epiphysiodesis. In summary, treatment of FH must be individualized according to the needs and expectations of each family/child and the reconstructive options available regionally.

805 Congenital disorder of glycosylation with fibular hemimelia

To explore the effectiveness of QIN Sihe's surgical strategy combined with Ilizarov technique in treating foot and ankle deformities on the verge of amputation. A retrospective analysis was conducted on the clinical data of 56 patients (62 feet) with foot and ankle deformities on the verge of amputation treated with QIN Sihe's surgical strategy and Ilizarov technique between May 2010 and December 2020. Among them, there were 39 males and 17 females. The age ranged from 8 to 62 years (median, 27.5 years). QIN Sihe's surgical strategy: subcutaneous release or open lengthening of contracted Achilles tendons, limited correction of bony deformities through multiple osteotomies during surgery, tendon transfer to balance the power of the foot and ankle, simultaneous percutaneous osteotomy and correction of tibial torsion deformity to restore the weight-bearing line of the lower extremity, and installation of Ilizarov foot and ankle distraction devices for slow distraction and correction of residual foot and ankle deformities. After removal of external fixation, individualized braces were used for protection during exercise and walking. For patients with bilateral deformities, staged surgeries were performed. The effectiveness was evaluated according to the QIN Sihe's Postoperative Evaluation Criteria for Lower Limb (Foot and Ankle) Deformity Correction and Functional Reconstruction at last follow-up. All patients achieved the preoperative expected orthopedic and functional reconstruction goals. The postoperative wearing time of external fixator was 3-7 months, with an average of 5.5 months. The incidence of pin tract infection during the treatment period was 6.5% (4/62). All patients were followed up 25-132 months (median, 42 months). All 56 patients successfully retained their limbs. At last follow-up, foot and ankle deformities were corrected, the weight-bearing line was basically restored, and plantigrade feet were restored. At last follow-up, according to QIN Sihe's Postoperative Evaluation Criteria for Lower Limb (Foot and Ankle) Deformity Correction and Functional Reconstruction, the effectiveness was rated as excellent in 37 cases (39 feet), good in 18 cases (21 feet), and fair in 1 case (2 feet). The excellent and good rate was 96.8% (60/62). The combination of QIN Sihe's surgical strategy and Ilizarov technique in treating foot and ankle deformities on verge of amputation is minimally invasive, safe, and the therapeutic effect is controllable. This combined approach has unique advantages in preserving limb function and restoring biomechanical balance.

Background Charcot neuroarthropathy of the ankle is an extremely challenging clinical dilemma, and its surgical management can be highly complicated. The goal of Charcot ankle treatment is to to restore a plantigrade and stable foot, and thus to avoid ulceration with subsequent infection. This report aims to introduce a method of correcting ankle deformity using a novel 3D printing technique. Patient and methods One patient with Charcot ankle deformity was operated in this study. The ankle deformity of this patient was quantified in three dimensions through computed tomography. On the basis of the computed tomography scans, a new titanium implant was designed and manufactured using 3D printing. The implant was applied in the surgery of tibio-talo-calcaneal arthrodesis to restore local anatomy of the affected ankle of the patient with Charcot neuroarthropathy. Results Evaluation of the post-operative radiography indicated union in the affected ankle. After surgery, the planar foot in this patient was restored. The patient was satisfied with the post-operative course, and joint fusion was successful as indicated by 2-year post-operative evaluation. The results of post-operative follow-up showed that the lower limb length of the patient with Charcot neuroarthropathy was salvaged, and the patient retained the plantigrade foot. Conclusion Three-dimensional printing technique combined with tibio-talo-calcaneal arthrodesis may help to correct ankle deformity in patients with Charcot neuroarthropathy.

Background:Fibular hemimelia, a congenital disorder characterized by the partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency, is the most common deficiency of long bones. The purpose of the present study of children with congenital fibular hemimelia was to examine the functional and psychosocial outcomes at a minimum of 2 years after treatment either with amputation and a prosthesis or with reconstruction and lengthening.Methods:Twenty children who were managed with primary amputation were compared with 22 children who were managed with staged limb reconstruction. The average age of the patients at the time of evaluation was 9 years (range, 5 to 15 years). Patients and parents completed psychosocial, quality-of-life, and satisfaction surveys. Patients underwent instrumented gait analysis and a timed 25 or 50-yard dash. The number and nature of surgical procedures were recorded from a retrospective chart review.Results:Families of children managed with amputation had lower economic and educational levels and were more ethnically diverse compared with the families of children managed with limb reconstruction. Scores on psychosocial and quality-of-life surveys were comparable with those from healthy patient populations. Parents of males treated with amputation perceived a lower school-related quality of life for their child; socioeconomic and ethnic differences between groups might account for this finding. Statistically but not clinically significant differences were measured during instrumented gait analysis at a self-selected walking speed and during a timed 25 or 50-yard dash. The majority of patients and parents reported satisfaction with the treatment method selected and would select the same treatment method again.Conclusions:At this interim stage of growth, there were no significant functional or psychological differences between groups. Both groups were satisfied with the outcome in mid-childhood, irrespective of the selection of amputation or limb reconstruction.Level of Evidence:Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

Charcot arthropathy related foot and ankle deformities are a serious challenge. Surgical treatment of these deformities is now well established. Conventional surgical treatment includes extensive surgical exposure, excision of bone, acute correction and internal fixation, which is not always appropriate in presence of active ulceration, infection and poor bone quality. A minimally invasive approach to osteotomies and gradual correction of deformities using a circular frame are proving helpful in minimizing the complications. Taylor Spatial Frame (TSF) hexapod with its various modules is well suited for a range of foot and ankle deformities. We have advocated minimally invasive targeted hind and mid foot osteotomies and gradual correction with Taylor Spatial Frame (TSF) in 10 patients with recurrent ulceration and deformity. There are 2 female and 8 male patients in this cohort. Appropriate TSF module was chosen for each patient– a long bone module for ankle and hindfoot deformities (4 patients) and a forefoot 6x6 butt frame (6 patients) for foot deformities. An osteotomy through the midfoot was performed in all chronic stable foot deformity cases. In the ankle and hindfoot deformities, a combination of soft tissue distraction correction of equinus and acute correction of hindfoot deformity through a calcaneal osteotomy, were used. Our outcome measures are complete healing of the ulcers and resolution of infection, clinically plantigrade foot and ability to wear regular or diabetic footwear. Complications included eight episodes of pin infection that responded to oral antibiotics only and two pin breakages. We achieved ulcer and infection free plantigrade feet that fit in to regular or diabetic footwear in 9 out of 10 patients. 9 patients remain ulcer and infection free at a minimum of 7 years and maximum of 14 years follow up. Taylor Spatial Frame treatment provides an alternative to conventional surgery in high-risk complex Charcot neuroarthropathy foot and ankle deformities.How to cite this article: Lahoti O, Abhishetty N, Shetty S. Correction of Foot Deformities from Charcot Arthropathy with the Taylor Spatial Frame: A 7–14-year Follow-up. Strategies Trauma Limb Reconstr 2021;16(2):96–101.

Fibular hemimelia is partial or total aplasia of the fibula; it represents the most frequent congenital defect of the long bones. It usually is associated with other anomalies of the tibia, femur, and foot. We reviewed 32 patients with Type III fibular hemimelia treated by successive lower limb lengthening and deformity correction using the Ilizarov method. We had three aims; first, to analyze complications, including the need for reoperation. The second was to assess knee and ankle function, specifically addressing knee ROM and stability and function of the foot and ankle. The third was assessment of overall patient satisfaction. Thirty-two patients underwent 56 tibia lengthenings and 14 ipsilateral femoral lengthenings. Their mean age and mean functional leg-length discrepancy at initial treatment were 6.7 years and 6.2 cm, respectively. Activity level, pain, patient satisfaction with function, pain, and cosmesis, complications, and residual length discrepancy were assessed at the end of treatment. The mean number of surgeries was six per case. The healing index was 44.9 days/cm. Although complications were observed during 60 lengthenings (82%), the highly versatile system overcame most of them. Nearly equal limb length and a plantigrade foot were achieved by 16 patients. For two patients, a Syme's amputation was performed. The outcome was considered satisfactory in 17 patients (53%) and relatively good in eight patients (25%). The Ilizarov technique has satisfactory results for treatment of Type III congenital fibular hemimelia and can be considered a good alternative to amputation. Level IV, therapeutic study. See the Guidelines for Authors for a complete description of levels of evidence.

Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia. In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared. Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years. Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening. level IV.