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 Table of Contents  
ORIGINAL ARTICLE
Year : 2018  |  Volume : 4  |  Issue : 2  |  Page : 76-82

Severe infantile blount's disease: Hemiplateau elevation and metaphyseal correction with use of the taylor spatial frame


Limb Reconstruction Unit, Royal National Orthopaedic Hospital, Stanmore, UK

Date of Web Publication4-Mar-2019

Correspondence Address:
Peter Calder
Limb Reconstruction Unit, Royal National Orthopaedic Hospital, Stanmore
UK
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jllr.jllr_2_18

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  Abstract 

Aims: Severe infantile Blount's disease can result in a challenging multiplanar deformity of the proximal tibia with intra-articular and metaphyseal components. We describe our results using the Taylor's spatial frame (TSF) for acute tibial hemiplateau elevation combined with gradual metaphyseal correction in patients with severe infantile Blount's disease with an associated physeal bony bar. Patients and Methods: Eight patients (ten knees) underwent tibial hemiplateau elevation and metaphyseal correction from 2012 to 2016. We undertook a retrospective case note and radiographic review of clinical and radiographic outcomes. The mean age at the time of surgery of was 11.7 years and the mean length of follow-up was 18.2 months. Results: Improvement in radiographic parameters was seen in all patients. The mean tibiofemoral angle improved from −28.3° to −5.9° postoperatively. The angle between femoral condyles and the tibial shaft improved from the mean of 56.3° to 90.3°. The joint depression angle was also seen to improve from the mean 47.4° to 9.8°. No significant complications were seen. Conclusion: This technique is effective in correcting the complex deformity encountered in severe infantile Blount's disease. Use of the TSF may provide certain advantages in comparison to previously described approaches.

Keywords: Blount's disease, deformity, genu varum, hexapod, Taylor spatial frame


How to cite this article:
Wright J, Calder P. Severe infantile blount's disease: Hemiplateau elevation and metaphyseal correction with use of the taylor spatial frame. J Limb Lengthen Reconstr 2018;4:76-82

How to cite this URL:
Wright J, Calder P. Severe infantile blount's disease: Hemiplateau elevation and metaphyseal correction with use of the taylor spatial frame. J Limb Lengthen Reconstr [serial online] 2018 [cited 2019 Jul 18];4:76-82. Available from: http://www.jlimblengthrecon.org/text.asp?2018/4/2/76/253390


  Introduction Top


Infantile tibia vara was first reported by Erlacher in 1922;[1] however, it was the subsequent in-depth description by Blount[2] in 1937 that led to the condition obtaining the eponym of Blount's disease. The condition represents a spectrum of severity, in which the age of onset and the stage of disease dictate the treatment required to correct and hopefully prevent the recurrence of deformity. Early infantile Blount's disease, without an associated bony bridge across the physis, can be corrected with various described metaphyseal osteotomies to restore the mechanical axis.[3],[4],[5],[6],[7]

Severe infantile Blount's disease produces a multiplanar growth deformity of epiphysis and metaphysis, which can present a challenge for the treatment. The natural history of the disease leads to the formation of a bony bridge across the medial tibial physis. The tibia vara deformity is often associated with internal tibial torsion, procurvatum, and shortening with depression of the medial tibial plateau.[2],[8],[9] Simple metaphyseal osteotomy as used in the earlier stages of disease is not sufficient to correct intra-articular deformity, and the physeal bony bar leads to inevitable recurrence of the tibia vara. Medial tibial plateau elevating osteotomy has been described to address the intra-articular deformity, either performed alone[8] or simultaneously with the second metaphyseal osteotomy[10],[11] to address the remaining long-bone deformity.

Developments and modifications of the original technique have been described in the literature from several centers. Acute correction and internal fixation of both tibial plateau and metaphyseal osteotomy as described by the original authors have had similar results replicated by other surgeons.[10],[11],[12],[13] The Ilizarov method has been used to gradually elevate both the medial plateau and correct at the metaphysis[14],[15],[16] or only to correct the metaphyseal osteotomy following acute hemiplateau elevation.[17] The Taylor's spatial frame (TSF; Smith and Nephew, Memphis, TN, USA) has been used for multiplanar metaphyseal correction in Blount's disease;[18] however, at present, there have only been limited published data describing its use as a means of correcting the multiplanar metaphyseal deformity with simultaneous acute hemiplateau elevation.[19],[20]

We report our clinical experience and outcomes with a surgical technique of medial tibial plateau elevation with gradual metaphyseal osteotomy correction, using the TSF in severe infantile Blount's disease with associated medial tibial physeal bony bridge.


  Patients and Methods Top


This study was subject to an institutional research and development department review (Research and Ethics Registration Number: SE16.046).

Between 2012 and 2016, eight children (ten knees) with severe infantile Blount's disease underwent deformity correction using the TSF. A retrospective case note and radiographic review was performed; no cases were lost to follow-up. There were four boys and four girls, with the mean age at the time of surgery of 11.7 years (range, 7–17) and the mean length of follow-up of 18.2 months (7.6–45.2). Four of the eight patients were skeletally mature at the latest follow-up. Five patients (seven knees) were obese with weight at the time of surgery >95th centile. The ethnicity of seven patients was Afro-Caribbean and one patient was of mixed Caucasian and Afro-Caribbean heritage.

Two patients required bilateral surgical procedures, in which both were performed in a staged manner. The second procedure was performed 3 months after the frame removal following the first stage. The remaining patients required unilateral treatment only. Four patients had undergone previous metaphyseal osteotomies with acute correction elsewhere, before referral.

Preoperatively, all children were noted to have pain in the knee with significant varus deformity and a varus thrust on walking. Following initial radiographic assessment, computed tomography scans were obtained in all patients to confirm plateau deformity and the presence of a physeal bony bar. All patients had Langenskiold Stage V or VI changes indicating significant joint depression; patients with earlier stages of disease and no significant intra-articular deformity would have been considered for metaphyseal correction alone. Before surgery, patients and family members underwent a counseling session with a clinical nurse specialist in preparation for management of the external fixator both physically and psychologically in the postoperative period.

Radiographic measurements

Standing long-leg radiographs were obtained with the patellae facing anteriorly and any leg-length discrepancy corrected with blocks. Lateral radiographs of the tibia were also performed [Figure 1]a and [Figure 1]b. Radiographic measurements were based on the described parameters used in the previous studies.[8],[11],[12],[15],[16] These included (a) tibiofemoral angle (TFA) between the anatomical axis of tibia and femur (negative value indicating varus); (b) angle of depression of the medial tibial plateau; (c) angle between femoral condyles and anatomic axis of tibia; (d) the anatomic lateral distal femoral angle[21] (aLDFA: normal 81°; range 79–83); and on the lateral view (e) anatomic proximal posterior tibial angle[21] (aPPTA: normal 81°; range 77–84). The torsional profile was assessed clinically.
Figure 1: (a) Long-leg radiograph demonstrating preoperative coronal plane deformity of the left leg (the patient aged 10 years and Langenskiold Stage VI). (b) Long-leg radiograph demonstrating preoperative coronal plane deformity of the right leg (same patient as Figure 1a)

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The measurements were taken from the radiographs at the first presentation before surgery, following completion of the treatment (after frame removal) and at the time of the latest follow-up. All measurements were made on standing films in a standardized manner.

Surgical technique

All operations were performed by the senior author, an experienced limb reconstruction surgeon.[22],[23] Under anesthesia, the patient was prepared supine on a radiolucent table. A single dose of prophylactic antibiotics was given before application of a tourniquet and exsanguination of the limb.

A fibula osteotomy was performed at the mid-diaphysis with excision of a 1–1.5-cm segment for use as bone graft.

A medial “J-shaped” incision was planned through which the plateau elevation can be performed. The periosteum was cut in line with the planned osteotomy with minimal stripping. A Trethowan ring handle retractor was placed subperiosteally behind the proximal tibia to protect the neurovascular bundle.

The beginning and endpoints of the plateau osteotomy were marked under image intensifier control, passing from the metaphyseal–diaphyseal junction medially to just beneath the medial tibial spine. The osteotomy was predrilled before completion with a sharp osteotome, avoiding entry into the joint [Figure 2]. A lateral view was obtained with intraoperative fluoroscopy to control passage of the osteotome through the posterior cortex [Figure 3]. A laminar spreader was used to elevate the plateau to restore the joint line acutely [Figure 4]. An arthrogram was performed at this stage, delineating the true medial joint line from the epiphysis to confirm the level of elevation achieved.[24] Adjustment of the laminar spreader allows correction of the position by assessing dye pooling in both anteroposterior (AP) and lateral planes, using the medial femoral condyle as a guide to which the tibial plateau was molded. The fibula graft was shaped and inserted into the osteotomy longitudinally as a strut [Figure 5]a. The laminar spreader was removed, and the joint was gently stressed under image intensifier guidance to demonstrate stability of the joint and adequate strut position. Actifuse (silicate-substituted calcium phosphate bone graft substitute; Baxter International, Illinois, USA) was then packed surrounding the graft.
Figure 2: Intraoperative fluoroscopy image demonstrating predrilling of the hemiplateau elevating osteotomy

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Figure 3: Lateral view on fluoroscopy to guide passage of the osteotome to the posterior tibia

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Figure 4: Intraoperative fluoroscopy image demonstrating the use of the laminar spreader to elevate the tibial plateau

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Figure 5: (a) Intraoperative fluoroscopy image demonstrating hemiplateau elevation and bone grafting. (b) CORA indicated at intersection between white and blue lines

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The proximal tibial and fibular incisions were closed and the tourniquet was deflated before application of the TSF. Skin markings were made utilizing the image intensifier to demonstrate the joint line, AP and lateral mechanical axes, and the center of rotation of angulation (CORA) of the deformity. As the epiphyseal component of the deformity has been corrected with the plateau elevation, the CORA is now recalculated, demonstrating the new CORA at the proximal tibial metaphysis [Figure 5]b. A “wires first” technique was undertaken: an initial transverse olive wire from lateral to medial, the second olive wire from posteromedial to anterolateral, and the third wire passing through the fibula head from posterolateral to medial. All wires transfixed the medial fragment. A 2/3 TSF ring was then positioned orthogonal to the knee joint line. A final half-pin is then placed into the medial fragment passing into the lateral block if large enough or into the lateral block alone if not. One or two distal rings were placed with a combination of half-pins and wires orthogonal to the tibial diaphysis.

The metaphyseal osteotomy was then performed through a 1-cm incision over the anterolateral border of the tibia at the CORA of deformity. The periosteum is elevated and multiple drill holes were performed before completion of the osteotomy with a sharp osteotome.[25] Compartment fasciotomies were not performed in any of the patients.

The correction was planned using the proprietary software to correct the residual deformity of varus, internal rotation, procurvatum, and shortening. In unilateral cases, the leg-length discrepancy was corrected with a planned overlengthening according to the calculated growth remaining from the proximal tibial physis.[26] The correction program was commenced following a latency period of 6 days. Full weight-bearing was permitted throughout the treatment as comfort allows [images during correction are indicated in [Figure 6]a and [Figure 6]b.
Figure 6: (a) Postoperative anteroposterior radiograph of the right tibia during correction. (b) Postoperative lateral radiograph of the right tibia during correction

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Following correction and consolidation of both the regenerate and hemiplateau osteotomy sites, the frame is removed under general anesthesia. To prevent recurrence of deformity, an epiphysiodesis of the proximal lateral tibial and fibula growth plate was performed using a 4.5-mm drill. This was required in five knees. Postoperative images are demonstrated in [Figure 7]. A Sarmiento cast was applied on frame removal to allow full weight-bearing. This was removed in clinic at 4 weeks.
Figure 7: Long-leg alignment radiograph demonstrating postoperative appearances

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In one patient (two knees), temporary lateral distal femoral hemiepiphysiodesis, using the Pediplate O-plate, (Orthopediatrics, Warsaw, IN, USA) was performed concomitantly with application of the frame for correction of an increased aLDFA.

Statistical analysis was performed using SPSS 23 (IBM NY, USA) for Mac OS. For radiographic parameters, differences were assessed with the Wilcoxon rank-sum test. P < 0.05 was considered statistically significant.


  Results Top


The average duration of the treatment in the TSF was 136 days (range 94–175). One knee was overcorrected into valgus and one knee had residual varus due to femoral deformity, although the tibial deformity was anatomically corrected. The remaining patients all corrected to neutral or slight valgus. At the latest follow-up, all patients reported no knee or ankle pain. All knees were reported to be stable without varus thrust. No evidence of recurrence was seen at this time.

Radiographic measurements

Improvement was seen from the mean preoperative TFA of −28.3° (range −11° to −50°) to mean postoperative value of 5.9° (range, −8° to −17°). The angle between femoral condyles and the tibial shaft (normal approximately 90°) improved from the mean of 56.3° to 90.3° postoperatively. The joint depression angle was also seen to improve from the mean of 47.4° to 9.8° postoperatively. The change in all three of these measurements achieved statistical significance (P < 0.05). [Table 1] demonstrates the patient-level data for radiographic parameters.
Table 1: Radiographic parameters before surgery and at the latest follow-up for all patients (all measurements in degrees)

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The average degree of distal femoral deformity was minimal, with a preoperative mean aLDFA of 84.6 and postoperative of 84.3 (normal range 79–83). This change in aLDFA was neither clinically nor statistically significant (P = 0.809). The posterior proximal tibial angle was not measurable on a lateral film preoperatively due to the degree of intra-articular proximal tibial deformity. Following correction, the postoperative aPPTA measured the mean of 79.4° (normal range, 77–84).

The mean torsional correction required was 11° (range, 0°–25°). At the latest follow-up, the torsional alignment was clinically corrected. Leg-length discrepancies were seen in the unilateral patients with the mean of 2.3 cm (range, 1–4 cm). The discrepancies were corrected through the metaphyseal osteotomy with additional lengthening performed in those patients according to the further growth that was anticipated (range, 0.5–2 cm). At the latest follow-up, the bilateral patients and those at skeletal maturity had <1 cm leg-length discrepancy.

Complications

Two intraoperative complications were seen. One plateau elevation osteotomy extended into the joint, although remained undisplaced and was stable following frame fixation. One metaphyseal osteotomy extended into the proximal osteotomy; similarly, following frame fixation, the proximal block was stable and did not affect correction.

No patients developed a compartment syndrome. One pin site infection required oral antibiotics; there were no deep infections requiring surgical debridement or changes to the frame.

All osteotomies went onto heal with no regenerate deformity or fracture following removal of the frame.


  Discussion Top


The natural history of Blount's disease as described by Langenskiold[8] involves a progressive growth disturbance of the medial proximal tibial physis leading eventually to the formation of a bony physeal bar and consequent deformity both within the epiphysis and metaphysis of the proximal tibia.

There is some controversy in the literature about the indications for the use of hemiplateau elevation in cases where there is a radiographic evidence for plateau depression.[27] Radiographic studies have suggested that where there is an apparent depression of the medial plateau on plain radiographs, the true cartilaginous height of the epiphysis may be greater when viewed on arthrogram[28] or magnetic resonance imaging.[29],[30] The procedure is not without risk,[16] requiring deformation or the cartilage (if not fracture, as in one case), to achieve correction. For this reason, we use an arthrogram in all cases to ensure that there is an assessment of the true cartilaginous anatomy of the epiphysis and guide elevation of the plateau. Once elevated, performing stress views under image intensifier will demonstrate that stability of the joint has been restored.

The method of combined hemiplateau elevation with metaphyseal corrective osteotomy has seen various modifications in the methods of both correction and fixation, since the first descriptions.[10],[12],[13],[15],[16],[19]

Acute correction with internal fixation avoids a prolonged period in a fixator; however, bearing weight must be restricted until healing and lengthening cannot be achieved with this technique. There could potentially be difficulties in skin closure if the acute correction is large; however, this has not been specifically reported as a problem.[10],[12],[13]

Use of the Ilizarov method, while being a more involved treatment for the patient, allows correction of angular and torsional deformity as well as limb lengthening. Full weight-bearing can be permitted throughout the treatment. The TSF further brings the advantage of allowing “fine tuning” of the program by adjusting the virtual hinge during correction, without adjusting the frame construct.[31],[32] The TSF is less bulky than the traditional Ilizarov apparatus, which may improve the patient tolerance of the fixator. As growth is stopped from the proximal tibial physis, the tibia can also be overlengthened in unilateral disease, in anticipation of the remaining growth differential between the two limbs.

Bone graft substitute was used in this series to augment the fibula cortical graft, which mainly acts as a strut to maintain the correction of the hemiplateau elevation. In this series, the hemiplateau was stabilized with a combination of the fibula strut and three transosseous wires, with no difficulties in fixation experienced. Different methods for fixation of the hemiplateau osteotomy could also be considered, including use of half-pins[20] or use of screws to stabilize the hemiplateau.[19]

van Huyssteen et al.[13] described the importance of lateral tibial and fibula epiphysiodesis at the time of primary surgery to prevent recurrence when the lateral physis is still open. McCarthy et al.[17] and Edwards et al.[20] concurred with this finding; their suggestion was that their recurrences were related to an incomplete lateral epiphysiodesis. We perform epiphysiodesis of the lateral proximal tibia and fibula at the time of frame removal, rather than at the initial operation. Drilling of the physis at the first stage may potentially cause bone loss, which may compromise the stability of the fine wire and half-pin fixation. The effect of the epiphysiodesis must be considered when planning the lengthening required. We estimate the expected growth remaining from the contralateral proximal tibial physis to overlengthen, hopefully achieving equalization by maturity. Follow-up should continue to skeletal maturity to ensure that growth remaining has been calculated correctly, in case supplementary procedures are indicated.

Jones et al.[15] commented on the importance of correcting the posterior tibial slope, commenting that varus thrust was seen in those patients where the posterior slope was excessive. The femoral condyle can be used as a guide onto which to elevate the plateau; adjustment of the laminar spreader and placement of the bone graft posteriorly can then be used to correct this element of the deformity. The intraoperative arthrogram can also be useful to demonstrate the cartilaginous extent of both condyle and plateau to ensure that the adequate correction has been achieved and help with identification of the metaphyseal CORA. Using this method, the posterior tibial slope as measured by the aPPTA was corrected within the normal range in all but one of the patients.

One overcorrection into valgus was seen in our series. When metaphyseal osteotomy alone is performed in the younger child without a bony physeal bar, overcorrection to 10° valgus has been suggested to reduce the risk of recurrence by protecting the “sick” medial physis.[3],[33] In the severe and recurrent cases with a bony bar where hemiplateau elevation is also required, halting further growth with a proximal tibial epiphysiodesis limits the remodeling as well as the risk of recurrence. In view of this, we believe that correction to a neutral or minimally valgus alignment should avoid the persistence of excessive valgus, which can prove to be problematic particularly in the overweight patient with a large thigh diameter.[17]

There are limitations with this study that must be considered. Care must be taken in drawing conclusions from a relatively small series of cases. However, as it is not common for the severity of disease to be significant enough to warrant this procedure, the lessons from the literature have been so far drawn from a limited number of patients. The data collection was retrospective, which brings the risk of incomplete data capture, if not all cases have been identified, although as a single surgeon series, we believe this to be complete. Only half of the patients have reached skeletal maturity, so further monitoring will be required to watch for recurrence or residual leg-length discrepancy at maturity.

This series represents, at present, the largest series of patients undergoing hemiplateau elevation with metaphyseal correction using the TSF. We have shown that while this is a technically challenging technique, it can provide a correction of the complex deformity encountered in severe and recurrent Blount's disease.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

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Gregosiewicz A, Wośko I, Kandzierski G, Drabik Z. Double-elevating osteotomy of tibiae in the treatment of severe cases of Blount's disease. J Pediatr Orthop 1989;9:178-81.  Back to cited text no. 10
    
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Schoenecker PL, Johnston R, Rich MM, Capelli AM. Elevation of the medical plateau of the tibia in the treatment of Blount disease. J Bone Joint Surg Am 1992;74:351-8.  Back to cited text no. 11
    
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Gkiokas A, Brilakis E. Management of neglected Blount disease using double corrective tibia osteotomy and medial plateau elevation. J Child Orthop 2012;6:411-8.  Back to cited text no. 12
    
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van Huyssteen AL, Hastings CJ, Olesak M, Hoffman EB. Double-elevating osteotomy for late-presenting infantile Blount's disease: The importance of concomitant lateral epiphysiodesis. J Bone Joint Surg Br 2005;87:710-5.  Back to cited text no. 13
    
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Janoyer M, Jabbari H, Rouvillain JL, Sommier J, Py G, Catonné Y, et al. Infantile Blount's disease treated by hemiplateau elevation and epiphyseal distraction using a specific external fixator: Preliminary report. J Pediatr Orthop B 2007;16:273-80.  Back to cited text no. 14
    
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Jones S, Hosalkar HS, Hill RA, Hartley J. Relapsed infantile Blount's disease treated by hemiplateau elevation using the ilizarov frame. J Bone Joint Surg Br 2003;85:565-71.  Back to cited text no. 15
    
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Hefny H, Shalaby H. A safer technique for the double elevation osteotomy in severe infantile tibia vara. Strategies Trauma Limb Reconstr 2010;5:79-85.  Back to cited text no. 16
    
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Feldman DS, Madan SS, Koval KJ, van Bosse HJ, Bazzi J, Lehman WB, et al. Correction of tibia vara with six-axis deformity analysis and the Taylor spatial frame. J Pediatr Orthop 2003;23:387-91.  Back to cited text no. 18
    
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Bar-On E, Weigl DM, Becker T, Katz K. Treatment of severe early onset Blount's disease by an intra-articular and a metaphyseal osteotomy using the Taylor spatial frame. J Child Orthop 2008;2:457-61.  Back to cited text no. 19
    
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Edwards TA, Hughes R, Monsell F. The challenges of a comprehensive surgical approach to Blount's disease. J Child Orthop 2017;11:479-87.  Back to cited text no. 20
    
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Calder P. Adolescent Blounts treated with acute plateau elevation and metaphyseal correction with TSF. In: Rozbruch R, Hamdy R, editors. Limb Lengthening and Reconstruction Surgery Case Atlas. Cham, Switzerland: Springer; 2015. p. 483-7.  Back to cited text no. 22
    
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Calder P. Tibial varus deformity and Blount's disease. In: Bentley G, editor. European Surgical Orthopaedics and Traumatology. Springer-Verlag Berlin Heidelberg: EFORT; 2014. p. 4371-84.  Back to cited text no. 23
    
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Haddad FS, Harper GD, Hill RA. Intraoperative arthrography and the ilizarov technique. Role in the correction of paediatric deformity and leg lengthening. J Bone Joint Surg Br 1997;79:731-3.  Back to cited text no. 24
    
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Rozbruch SR, Fragomen AT, Ilizarov S. Correction of tibial deformity with use of the Ilizarov-Taylor spatial frame. J Bone Joint Surg Am 2006;88 Suppl 4:156-74.  Back to cited text no. 31
    
32.
Rozbruch SR, Segal K, Ilizarov S, Fragomen AT, Ilizarov G. Does the Taylor spatial frame accurately correct tibial deformities? Clin Orthop Relat Res 2010;468:1352-61.  Back to cited text no. 32
    
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