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      Combined surgery with 3-in-1 osteosynthesis in congenital pseudarthrosis of the tibia with intact fibula

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          Abstract

          Background

          Re-fracture is the most serious complication in congenital pseudarthrosis of the tibia (CPT). There are reports that children with small cross-sectional areas in the sections of the pseudarthrosis are more prone to re-fracture. Presently, preventing complications is a challenge. Increasing the cross-sectional area in healed segments may reduce the incidence of re-fracture.

          Purpose

          To elucidate the indications, surgical technique, and outcomes of combined surgery and 3-in-1 osteosynthesis in CPT with intact fibula.

          Methods

          We retrospectively assessed 17 patients with Crawford Type IV CPT with intact fibula (Type A) who were treated with combined surgical technique and 3-in-1 osteosynthesis between March 2014 and August 2015. The average age of the patients at the time of surgery was 3 years. Incidence of re-fracture, ankle valgus, proximal tibial valgus, and limb length discrepancy (LLD) were investigated over an average follow-up time of 47 months.

          Results

          Primary union was achieved in all patients. The average time for primary union was 4.9 months. Fifteen (88%) cases showed LLD with an average limb length of 1.6 cm; 6 (35%) cases exhibited tibial valgus with an average tibial valgus deformity of 7.8°; 2 cases had ankle valgus, wherein the ankle valgus deformity was 12° in one and 17° in another; and the cross-sectional area of the bone graft was enlarged to 1.74 times that of the tibia shaft. No case had re-fracture during the follow-up period. Movement of the ankle joint was restored in 16 patients with an average dorsiflexion of 22° and an average plantar flexion of 41°; the function of the ankle joint was normal. One patient had plantar flexion of 20° but did not have dorsiflexion.

          Conclusion

          Combined surgical technique with 3-in-1 osteosynthesis, which is primarily considered for bone union with a large cross-sectional area, results in a high primary union rate. This can provide satisfactory results in short-term follow-up when treating CPT with intact fibula (Type A).

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          Most cited references13

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          Management of congenital pseudarthrosis of the tibia with the Ilizarov method in a paediatric population: influence of aetiological factors.

          Our study compared the rates of union achieved with the Ilizarov method in congenital pseudarthrosis of the tibia (CPT) associated with neurofibromatosis type 1 (NF1) or CPT of idiopathic origin in paediatric patients.
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            "4-in-1 osteosynthesis" for atrophic-type congenital pseudarthrosis of the tibia.

            According to the authors' multi-targeted, fibular status-based algorithmic approach using the Ilizarov technique, ankle stabilization by end-to-end osteosynthesis of the fibula is advocated for mild (type B1), "4-in-1 osteosynthesis" in which all 4 proximal and distal segments of the tibia and fibula are placed in 1 healing mass for moderate (type B2), and distal tibiofibular (TF) fusion for severe (type B3) fibular pseudarthrosis in association with atrophic-type congenital pseudarthrosis of the tibia (CPT). This report describes the indications, operative technique, and outcomes of "4-in-1 osteosynthesis" for atrophic-type CPT associated with type B2 fibular pseudarthrosis. Thirteen patients presented with atrophic-type CPT associated with type B2 fibular pseudarthrosis underwent Ilizarov osteosynthesis between 1989 and 2007 for atrophic-type CPT. To validate the efficacy of "4-in-1 osteosynthesis" in these patients, fracture risk and ankle function were compared between 2 groups of type B2 patients, namely, 8 patients (mean age, 6.3 y) who underwent "4-in-1 osteosynthesis" according to our current protocol (Group I), and 5 patients (mean age, 3.2 y) treated by other techniques (3 distal TF fusion, 2 failed end-to-end osteosynthesis) during the learning period (Group II). No refracture occurred in Group I, whereas refracture occurred in all except 1 in Group II. Ankles were eventually stabilized by distal TF fusion in all patients in Group II. The Kaplan-Meier method revealed a refracture-free cumulative survival rate of 100% in Group I, whereas in Group II, it dropped progressively and reached 60% at 1.8 years and 20% at 2.7 years. No significant difference in ankle function was evident between the 2 groups (american orthopaedic foot and ankle society (AOFAS) score, 89.25 ± 7.25 after 7.4 y of follow-up in Group I, and 84.6 ± 9.53 after 13 y of follow-up in Group II). It is imperative that fibular status be evaluated carefully to enable the planning of the most effective, safe, practical treatment. "4-in-1 osteosynthesis," which is primarily considered for bony union with a large cross-sectional area and ankle stabilization, seems to be a better choice for atrophic-type CPT associated with type B2 fibular pseudarthrosis, in which end-to-end osteosynthesis of the fibula often fails. Level III, Retrospective comparative study.
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              Current treatment of congenital pseudarthrosis of the tibia: a systematic review and meta-analysis

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                Author and article information

                Contributors
                meihaiboprof@outlook.com
                Journal
                Orphanet J Rare Dis
                Orphanet J Rare Dis
                Orphanet Journal of Rare Diseases
                BioMed Central (London )
                1750-1172
                2 March 2020
                2 March 2020
                2020
                : 15
                : 62
                Affiliations
                ISNI 0000 0001 0266 8918, GRID grid.412017.1, Department of pediatric orthopaedics, Hunan Children’s Hospital, , The Pediatric Academy of University of South China, ; 86 ziyuan road, yuhua district, Changsha City, Hunan Province 410007 People’s Republic of China
                Author information
                http://orcid.org/0000-0002-9434-1716
                Article
                1330
                10.1186/s13023-020-1330-z
                7053109
                32122367
                c5b25819-d756-40f7-95ae-40ae7ac3e0f6
                © The Author(s). 2020

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                History
                : 9 October 2019
                : 7 February 2020
                Categories
                Research
                Custom metadata
                © The Author(s) 2020

                Infectious disease & Microbiology
                3-in-1 osteosynthesis,congenital pseudarthrosis of tibia,fibula intact

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