Comparative study of different extendable intramedullary rods combined with surgery in the treatment of congenital pseudarthrosis of the tibia.

BACKGROUND: When using traditional extensible intramedullary rods to treat congenital pseudarthrosis of the tibia (CPT), there were cases of re-fracture and internal fixation fracture. Therefore, the authors propose a research hypothesis that a thicker distal extensible intramedullary rod can better protect the tibia and reduce the incidence of refracture PURPOSE: To investigate the clinical efficacy of new and traditional extensible intramedullary rods in the treatment of CPT in children METHODS: From January 2017 to December 2021, the clinical data of 49 children with CPT who were treated with traditional extensible intramedullary rod combined surgery (group A) and new extensible intramedullary rod combined surgery (group B) in our hospital were collected. Inclusive criteria: ① Crawford type IV CPT children; ② The operation was performed by the same team. EXCLUSION CRITERIA: patients with multiple tibial angulation. During follow-up, the initial healing, proximal tibial valgus, tibial length, ankle valgus, refracture and intramedullary rod displacement of CPT children in the two groups were evaluated RESULTS: It was a retrospective investigation. In group A, 26 cases met the inclusion criteria, 24 cases achieved primary healing, with an primary healing rate of 92%, including 1 case of nonunion due to osteomyelitis complications after surgery, and 1 case of delayed healing, with an average healing time of 4.7 ± 0.8 months. 17 cases (68%) had unequal tibia length, with an average difference of 1.6 ± 0.8 cm. Ankle valgus occurred in 10 cases (40%) with an average of 14.4°±4.8°; Proximal tibial valgus occurred in 6 cases (24%) with an average of 7 °± 1.8 °. 20 cases (80%) had tip of the rod migration.10 cases (40%) had re-fracture; The average follow-up time was 2.4 ± 0.4 years. In group B, 22 patients achieved primary healing, and the primary healing rate was 95%, including 1 case with delayed healing. The average healing time was 4.7 ± 1.7months. 14 cases (61%) had unequal tibia length, with an average difference of 1 ± 0.5 cm. Ankle valgus occurred in 4 cases (17%) with an average of 12.3 °±4.9°; The proximal tibia valgus occurred in 9 cases (39%), with an average of 7.7 °±2.5 °. 14 cases (61%) had new type of intramedullary rod displacement. 3 cases (13%) had re-fracture; The average follow-up time was 2.3 ± 0.6years CONCLUSION: Compared with the traditional extended intramedullary rod combined operation, the new type of extended intramedullary rod combined operation has a lower incidence of re-fracture after CPT, but it still needs to be verified by large sample and multi-center research.

Is Using a Ring External Fixator in the Treatment of Congenital Pseudarthrosis of the Tibia Associated With Better Results or Using a Locking Plate?

BACKGROUND: Congenital pseudarthrosis of the tibia (CPT) is a rare disease. CPT is often unilateral and occurs between the middle and distal third of the tibia. Concurrent involvement of the fibula is present in more than half of cases. histologic studies indicate the presence of fibrous hamartoma tissue and a sick periosteum, which leads to recalcitrant bone fracture and, eventually, pseudoarthrosis. Although there are various surgical techniques, we intend to compare the 2 methods of external fixation versus internal plating. METHODS: Demographic data were collected from 26 patients with frank pseudoarthrosis. After exclusion criteria, patients were compared in groups A (12 patients) and B (11 patients). Resection of hamartoma and sclerotic bone, intramedullary rodding and autologous bone, and periosteal grafting were performed for all patients. In group A, we used a ring external fixator for compression and rotational stability, but in group B, a locking plate was used for these purposes. RESULTS: Plating takes less time to use during surgery. In group A, the primary bony union was obtained in 67% of patients, while in group B, 82% of patients had a primary union. Meanwhile, the average time till the final union in group A was 6 months, while in group B, this time was 3.5 months. Positive union mass was obtained in 58% of the patients in group A and 82% of group B. In addition, plating prevented ankle valgus deformity in group B. CONCLUSIONS: Permanent intramedullary rodding is a surgical requirement for correction of deformity and refracture prevention, but additional stability can be achieved with the use of a ring external fixator or internal plate. Cross union and positive union mass are 2 important factors in the treatment of pseudoarthrosis; these results are achieved to a greater extent and in a shorter period of time using the plate. LEVEL OF EVIDENCE: level IV - case series.

Osteogenic organoid for bone regeneration: Healing of bone defect in congenital pseudoarthrosis of the tibia.

BACKGROUND: Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease associated with failure to achieve bone union and recurrent fractures. There is evidence showing that CPT is associated with decreased osteogenesis. Based on the capacity of mesenchymal stromal cells (MSCs) to induce osteogenesis, we develop an osteogenic organoid (OstO) constituted by these cells, and other components of the bone niche, for inducing bone formation in a child diagnosed with CPT. AIM: To evaluate the capacity of an OstO to induce bone formation in a patient with CPT. METHODS: The OstO was fabricated with allogeneic bone marrow MSCs from a healthy donor, collagen microbeads (CM) and PRP clot. The CM and PRP function as extracellular matrix and scaffolds for MSC. The OstO was placed at the site of non-union. Internal and external fixation was placed in the tibia. Radiological evaluation was performed after MSCs transplantation. RESULTS: After 4 months of MSCs transplantation, radiographic imaging showed evidence of osteogenesis at the site of CPT lesion. The tibia showed bone consolidation and complete healing of the non-union CPT lesion after 6 months. Functional improvement was observed after 1 year of MSC transplantation. CONCLUSIONS: The OstO is a bone-like niche which promote osteogenesis in patients with failure in bone formation, such as CPT. To our knowledge, these results provide the first evidence showing CPT healing induced by an OstO constituted by allogeneic MSCs. Future studies incorporating a larger number of patients may confirm these results.

[Long term follow-up evaluation of combined surgery for congenital tibial pseudarthrosis in children].

Objective: To explore the long-term effect of combined surgery for the treatment of congenital tibial pseudarthrosis in children. Methods: The clinical data of 44 children with congenital tibial pseudarthrosis who underwent combined surgery (tibial pseudarthrosis tissue resection, intramedullary rod fixation, Ilizarov external fixator fixation, wrapped autologous iliac bone graft) from August 2007 to October 2011 at the Department of Pediatric Orthopedics, Hunan Children's Hospital were collected retrospectively. There were 33 males and 11 females. The age at the time of surgery was (3.7±2.2)years (range:0.6 to 12.4 years), including 25 cases under 3 years old and 19 cases above 3 years old.Among them, 37 cases were complicated with neurofibromatosis type 1.The operation status, postoperative complications and follow-up results were recorded. Results: The follow-up time after surgery was (10.9±0.7)years (range:10 to 11 years).Thirty-nine out of 44 patients (88.6%) achieved initial healing of tibial pseudarthrosis, with an average healing time of (4.3±1.1)months (range:3 to 10months).In the last follow-up, 36 cases (81.8%) had unequal tibial length, 20 cases (45.4%) had refractures, 18 cases (40.9%) had ankle valgus, 9 cases (20.4%) had proximal tibial valgus, and 11 cases (25.0%) had high arched feet.Nine cases (20.4%) developed distal tibial epiphyseal plate bridging.17 cases (38.6%) had abnormal tibial mechanical axis.Seven cases (15.9%) developed needle infection, and one case (2.3%) developed tibial osteomyelitis. 21 patients (47.7%) had excessive growth of the affected femur.Five patients (11.3%) had ankle stiffness, and 34 patients (77.2%) had intramedullary rod displacement that was not in the center of the tibial medullary cavity.Among them, 8 cases (18.1%) protruded the tibial bone cortex and underwent intramedullary rod removal.18 children have reached skeletal maturity, while 26 children have not been followed up until skeletal maturity. Conclusion: Combined surgery for the treatment of congenital pseudarthrosis of the tibia in children has a high initial healing rate, but complications such as unequal tibia length, refracture, and ankle valgus occur during long-term follow-up, requiring multiple surgical treatments.

Management of congenital pseudoarthrosis using Ilizarov Device-Delsuth, oghara experience.

Background: Congenital pseudoarthrosis of the tibia (CPT) is a rare and very difficult pediatric orthopedic condition to manage. It occurs spontaneously or from a trivial trauma. It results from a dysplastic periosteum that is more osteoclastic and less osteoblastic in nature. Successful treatment targets excision of the dysplastic periosteum and replacement with viable one and bone graft ± bone morphogenic proteins to aid union. Stabilization of the union site with intramedullary implants is advised to prevent refracture. Aims: To highlight the outcome of treatment of CPT with the Ilizarov procedure, bone graft, and use of bisphosphonate (sodium alendronate). Patients and Methods: This is a retrospective study of five patients treated for CPT using the Ilizarov procedure, bone graft, and use of bisphosphonate in the Delta State University Teaching Hospital, Oghara in Delta State, Nigeria. Inclusion criteria were patients that had a fracture of the tibia ± fibula spontaneously or from minor trauma. Exclusion criteria were patients that had pathological fractures or fractures from significant trauma. The outcome was graded into good, fair and poor. Results: Five patients were seen in this study. The male/female ratio was 2:3. The age was 6-18 years (mean = 11.5 years). The limb length discrepancy was 6-12 cm (mean = 7.2 cm) pre-operation and zero after completion of distraction. There was the loss of some regeneration in two patients at the end of consolidation. Two patients had hypertrophic CPT, while three patients had atrophic. Union was good in four and fair in one. Complications seen were ankle stiffness bending of the regenerate and loss of length of regenerate. Conclusion: CPT is a rare condition worldwide that is associated with non-union and complicated by post-operation refracture or non-union has been successfully treated with a procedure that entails use of Ilizarov technique, bone graft bisphosphonate and support with intramedullary nails.

Congenital Pseudarthrosis of the Tibia: Long-term Outcome of Treatment With Intramedullary Vascularized Fibular Graft Combined With Ilizarov Distraction.

BACKGROUND: Late presenting cases of congenital pseudarthrosis of the tibia, are further complicated with severe shortening. Limb length discrepancy (LLD) cannot be corrected by vascularized fibular grafting and the use of Ilizarov distraction is associated with a high rate of complications. The aim of this study was to report the long-term follow-up of a combined technique previously published under the name "telescoping vascularized fibular graft". MATERIALS AND METHODS: Eleven patients operated at an average age of 10.2±3.2 years were reviewed. All cases were Crawford type IV affected by neurofibromatosis 1. Nine patients had an average of 4.3 previous operations. Preoperative LLD averaged 7.9±2.5 cm. RESULTS: Follow-up averaged 10±5.4 years. Seven cases (63.6%) reached skeletal maturity before final follow-up. Primary union was achieved in all cases after an average of 7.2±1.3 months. Full weightbearing was possible after an average of 10.6±2.2 months. Recurrent stress fractures occurred in 9 cases (81.8%) and healed with casting in 6 cases and required internal fixation in 3 cases. Eight cases (72.8%) developed tibial shaft deformities, mainly procurvatum that required corrective osteotomy in 2 cases. Final LLD averaged 2.7±1.3 cm. Complete tibialization of the graft was achieved after an average of 17.0 ± 3.6 months. Valgus deformity of the ipsilateral ankle averaged 12.4 degrees±7.5. CONCLUSION: The presented technique avoids osteotomy of the diseased bone and allows simultaneous treatment of the pseudarthrosis and correction of shortening. Compared with conventional bone transport, it requires shorter time of frame application and therefore better patient tolerability because there is no waiting for consolidation of the regenerate. The dis-impaction of the doweled fibula occurs proximally, allowing the less active site located at the distal aspect of the pseudarthrosis to heal without displacement. A shortcoming of the presented technique is the more propensity for axial deviation and refractures that seldom require surgery. LEVEL OF EVIDENCE: Level-IV.

Clinical study of a new type of telescopic rod for the treatment of congenital pseudarthrosis of the tibia in children.

The purpose of this study was to investigate the initial efficacy of a new telescopic rod in the treatment of congenital pseudarthrosis of tibia (CPT) in children. A new type of telescopic rod for children was designed and its clinical results were evaluated. There were nine cases of CPT on the right and six cases on the left; there were six females and nine males. The average age at operation was 35.5 months. There were 12 cases with neurofibromatosis type 1 and 4 cases with proximal tibial dysplasia. The initial healing of tibial pseudarthrosis, proximal tibial valgus deformity, tibial length, ankle valgus, ankle function, and intramedullary rod displacement were evaluated during follow-up. All 15 cases were followed up for an average of 18.2 months, 14 cases achieved initial healing, with an average healing time of 4.3 months. Ten cases had unequal lengths of the tibia, with an average of 0.9 cm. Proximal tibial valgus occurred in seven cases and ankle valgus occurred in one child. The average range of motion of the ankle joint was 23° and the average plantar flexion was 42°. The average extension of the new telescopic rod was 1.6 cm. The displacement of the telescopic rod occurred in seven cases, there was no epiphyseal plate tethering and re-fracture. The new children's telescopic rod has a reasonable design and can extend with the growth of tibia. There are no complications of epiphyseal plate tethering and re-fracture. It provides a new choice of intramedullary fixation for the treatment of CPT.

The incidence of congenital pseudarthrosis of the clavicle and review of the literature.

The aim of this study was to identify the hitherto unknown incidence of congenital pseudarthrosis of the clavicle (CPC), based on a cohort of continuous livebirths born in our hospital, to review the literature and investigate if there is evidence supporting the published association between left-sided CPC and dextrocardia. From our electronic medical record and radiology databases, we identified all live births and patients with the diagnosis of CPC born from 2000 to 2016. We reviewed the imaging which included one or both clavicles to search for unrecorded CPC cases and reviewed all retrievable CPC publications listed in PubMed and publications quoted within these publications going back to 1910. We identified 87 407 livebirths of which 41 800 had radiological studies done, 14 885 showing one or both clavicles. We found five cases of CPC, two from the electronic database and three from our imaging review, giving an incidence of 1 of 17 481 livebirths. We identified 138 publications reporting paediatric and adult CPC cases and 12 review articles, including 429 patients (187 female; 159 male; 83 unknown) with 456 CPCs and a minimum of 24 additional patients from case reports for which we could not retrieve details. Two publications reported one case of left-sided CPC with dextrocardia, either not showing left/right marking or only showing the CPC with the aortic knob on the same side. We report the first CPC incidence of 0.0057%, provide the by far most inclusive CPC epidemiology based on 429 patients and could not find reliable proof that there has ever been a patient with left-sided CPC which was associated with dextrocardia.

EXPERIMENTAL STUDY OF BONE DENSITY IN PATIENTS WITH CONGENITAL PSEUDOARTHROSIS OF THE TIBIA BEFORE AND AFTER SURGERY.

OBJECTIVE: The aim: Determine the changes in bone density that occur in the cortical layer of the tibia in patients with CPT after surgical treatment due to radiography data. PATIENTS AND METHODS: Materials and methods: The study was performed on X-rays of the tibia of three patients with CPT, aged 5 to 7 years. X-rays were taken before surgery and 1 year after surgery. The optical density of the cortical layer of both tibia bones was determined, for which they were conditionally divided into 7 zones. RESULTS: Results: In all patients with CPT before treatment, there is significant decrease in the optical density of cortical bone tissue of the damaged tibia compared with contralateral. One year after surgery, there was an increase in the optical density of the cortical layer of both tibia of the studied patients. The increase in the density of the nonoperated tibia can be attributed to the active growth of patients. On the operated limb, the increase in the density of the cortical layer became statistically significant in all studied areas, which can be attributed not only to the growth of patients, but also to the possibility of active loading of the operated limb. CONCLUSION: Conclusions: In all patients with CPT before treatment, a statistically significant decrease in the density of cortical bone tissue of the damaged tibia was observed. As a result of the treatment there is an approximation of the bone density of the operated limb to the indicators of intact contralateral bone.

Functional and radiological outcomes after treatment of congenital pseudarthrosis of the tibia using the Ilizarov technique: a retrospective single-center study.

BACKGROUND: Congenital pseudarthrosis of the tibia (CPT) is a challenging problem in orthopedic practice, with high rates of non-union, refracture, and residual deformities. After union, long-term follow-up is required to manage late post-union complications. This study aimed to assess the outcomes of the Ilizarov technique in the management of CPT. MATERIALS AND METHODS: This retrospective study included patients with CPT treated with the Ilizarov method between 2005 and 2018. Intramedullary rods were used in 9 cases and iliac bone graft was used in 12 cases. An orthosis was applied till the end of follow-up in all cases. The American Orthopaedic Foot and Ankle Society (AOFAS) scale was used for the evaluation of the functional outcomes. RESULTS: This study included 16 patients, 11 males and 5 females, with an average age of 5.4 ± 2.8 years. Seven cases had multiple previous surgeries. Six patients had neurofibromatosis. The mean follow-up period was 5.8 ± 3.4 years. The average AOFAS score improved significantly from 47.5 ± 7.6 preoperatively to 78.9 ± 8.9 at the latest follow-up. Union was achieved in 15 cases, and persistent non-union occurred in one case. The clinical results were excellent in one patient, good in seven cases, fair in 6, and poor in 2 cases. The radiological results were excellent in one patient, good in seven cases, fair in seven, and poor in one case. CONCLUSIONS: The Ilizarov technique combined with intramedullary rod and primary or secondary bone graft provides a high union rate of CPT and can achieve simultaneous effective management of problems related to pseudarthrosis, including non-union, deformity, limb shortening, and adjacent joint contracture and subluxation. Level of evidence Level IV.

New insights into pathogenesis of congenital pseudarthrosis of tibia in children using periosteum proteomics analysis.

RATIONALE: The exact etiology and pathogenesis of congenital pseudarthrosis of tibia (CPT) are not clear. Quantitative proteomics analysis plays a vital role in disease pathology research. Tandem mass tag (TMT)-based proteomics techniques were employed to identify and analyze the differentially expressed proteins (DEP) in the tibia periosteum tissues of CPT patients. METHODS: The samples were divided into three groups: CPT with NF1 group, CPT without NF1 group (non-NF1-CPT), and control group (patients with open tibial fracture). A fold change ≥1.5 or ≤0.66 and P-value <0.05 were used as the thresholds to screen DEPs. Subsequently, bioinformatics resources such as online tools DAVID and String were used to generate gene ontology (GO) annotation, KEGG pathways enrichment, and protein-protein interaction (PPI) network for these DEPs. RESULTS: The results show that a total of 347 proteins were differentially expressed in NF1-CPT groups, 212 of which were upregulated and 135 were downregulated. There were more DEPs in non-NF1-CPT groups; we identified 467 DEPs, including 281 upregulated and 186 downregulated. Among them, NF1-CPT groups and non-NF1-CPT groups shared 231 DEPs, and the remaining 230 DEPs showed the same expression trend in the two disease groups, with 117 upregulated and 113 downregulated. In particular, 116 proteins were altered only in NF1-CPT groups (94 were upregulated and 22 were downregulated), whereas 236 proteins were altered only in non-NF1-CPT groups (164 were upregulated and 72 were downregulated). Finally, compared with non-NF1-CPT groups, 47 proteins changed 1.5-fold and P-value < 0.05 in NF1-CPT groups. CONCLUSIONS: To sum up, we found that common DEPS in periosteum of NF1-CPT and non-NF1-CPT groups are mainly involved in cell matrix assembly, cell adhesion, AKT-PI3K signal pathway activation, and vascular agglutination, which indicate that these are the pathological characteristics of CPT. The osteogenic ability is weak, the osteoclastic ability is strong, the vascular lumen is narrow, the invasive growth and the proliferation of fibroblasts are enhanced in CPT patients.

Analysis of related factors of behavioral problems in children with congenital pseudarthrosis of tibia.

OBJECTIVE: This study aimed to investigate the factors associated with behavioral problems in children with congenital pseudarthrosis of the tibia. METHODS: Random sampling is utilized to obtain a sample of 90 patients. The behavioral problems of the patients are detected by Achenbach Children's Behavior Scale. Parental emotional problems are investigated by the Self-Rating Depression Scale and Self-Rating Anxiety Scale. RESULTS: The results demonstrate that the detection rate of behavioral problems in children with congenital pseudarthrosis of the tibia is 53.3% (48/90). Among these behavioral problems, an abnormal rate is higher in the four dimensions: thinking, violation of discipline, social interaction, and aggression. The anxiety and depression scores of caregivers are statistically higher in the abnormal group than in the normal group. The results of the multivariate analysis show that the anxiety degree of the parents had a significant impact on the behavior of the children. CONCLUSIONS: Children with congenital pseudarthrosis of the tibia are facing the issues of high rates of behavioral problems. Parents of children with congenital pseudarthrosis of the tibia had higher levels of anxiety and depression than parents of normal children. The anxiety and depressive state of mind of parents or caregivers had a significant impact on the behavior of children with congenital pseudarthrosis of the tibia.

Design of a New "U"-shaped Staple and Its Clinical Application in Postoperative Ankle Valgus of Congenital Pseudarthrosis of the Tibia in Children.

OBJECTIVE: There has been a lack of suitable epiphysis blocking materials due to the characteristics of less tissue coverage and narrow epiphysis in children's distal tibial medial malleolus. Therefore, this study is to investigate the clinical efficacy and safety of a new "U"-shaped staple in the treatment of postoperative ankle valgus of congenital pseudarthrosis of the tibia (CPT). METHOD: According to the inclusion and exclusion criteria, 33 patients with postoperative ankle valgus of CPT were treated with new "U"-shaped staples from May 2013 to September 2019. The deformity of ankle valgus was gradually corrected by implanting a new "U"-shaped staple on the medial side of the distal tibia. Clinical indexes such as the operation time, intraoperative bleeding and hospital stay were observed. Tibiotalar angle was selected as the evaluation index of ankle valgus. American Orthopedic Foot & Ankle Society (AOFAS) scale was used for clinical evaluation of ankle function. The tibiotalar angle, deformity correction rate and complications were evaluated by preoperative, postoperative and last follow-up imaging data. Student's t-test was used for statistical analysis. RESULTS: Thirty-three patients, including 12 males and 21 females were included. All the patients were followed up for at least 14 months, with an average of 35 months. The average operation time was 23 (15-40) min, the average amount of intraoperative bleeding was 7.5 (4-10) mL, and the average hospital stay was 4.2 (3-6) days. The intraoperative tibiotalar angles of all patients were 74.2° ± 4.6°, the tibiotalar angle were 86.8° ± 4.9° when internal fixation was removed, and the tibiotalar angles at the last follow-up were 84.3° ± 5.9°. The average orthopedic rate was 0.68° per month. No patients suffered from serious complications such as screw prolapse, osteomyelitis, wound infection, etc. Postoperative wound pain complications occurred in two patients, which were relieved after conservative treatment. The AOFAS score improved from 46.2 ± 9.4 before the operation to 74.6 ± 5.7 at the last follow-up (P < 0.01). The ankle movement was good without joint stiffness. There was no epiphyseal plate injury after the removal of internal fixation. CONCLUSION: The new "U"-shaped staple is characterized by simple implantation, low notch, lower risk of fixation failure and close fitting with cortical bone. It is a safe and effective internal fixation system for the treatment of ankle valgus in children.

Congenital pseudarthrosis of the clavicle: a systematic review.

PURPOSE: There is no consensus on the type of surgical treatment of congenital pseudarthrosis of the clavicle due to its rarity. The purpose of this study is to provide evidence in favor of a surgical technique and to explore factors correlated with bone consolidation. METHODS: Systematic review of the literature and an analysis of the data for each subject, including all available subjects from the published series and clinical cases since 1990. Fisher's exact tests or T-tests were used to evaluate the effect of independent variables (age at surgery and type of treatment) on bone healing. RESULTS: The literature search provides 305 articles; 30 were selected, reporting 191 patients and 194 clavicles. One hundred and fifty-one clavicles were operated on at a mean age of nine years and four months (from 8 months to 21 years). Thirteen clavicles (8, 6%) had not consolidated at the last follow-up. Concerning the type of fixation, the rate of healing was similar for plates and pins (p = 0.27). The rate of consolidation was higher with autograft than with allograft, xenograft, or no graft (p = 0.00001), and was 100% for vascularized graft. The mean age at surgery was higher for patients who healed at the last follow-up (118 vs. 61 months, p = 0.001). CONCLUSION: In the event of surgical indication for congenital pseudarthrosis of the clavicle, it is recommended to perform autograft and stable fixation (level 4) after seven years old (level 4).

Analysis of risk factors affecting union and refracture after combined surgery for congenital pseudarthrosis of the tibia: a retrospective study of 255 cases.

BACKGROUND: Congenital pseudarthrosis of the tibia (CPT) is a rare disease occurring in children. The aim of this study is to identify the factors affecting bone union and re-fracture after surgery for CPT and to provide reliable evidence for clinics. METHODS: We collected the detailed information of 255 cases with Crawford IV CPT treated by combined surgery in our hospital from 2013 to 2020. Basic characteristics were recorded. Univariate variance and logistic regression analysis were used to compare the correlations between factors and outcomes. RESULTS: 92.5% of patients achieved primary union, 7.5% of patients had non-union and 13.3% of patients had re-fracture. Logistic regression analysis showed that age at index surgery (Coef. = 0.171, 95%CI 0.015-0.327, P = 0.032), and CPT location (Coef. = - 1.337, 95%CI - 2.218-0.456, P = 0.003) had statistical differences, while no factors had significant correlation with re-fracture. Furthermore, ROC curve showed that the optimal age threshold for first surgery was 2.37 years old. CONCLUSIONS: For patients with Crawford IV CPT treated by combined surgery, the younger the age at index surgery and the closer the CPT location to the distal end, the easier to achieve bone union.

Reconstruction of congenital pseudarthrosis of the clavicle with a barrel-shaped mono-cortical iliac crest autograft: A case report.

BACKGROUND: Congenital pseudarthrosis of the clavicle (CPC) is a rare congenital entity with unresolved aetiology and pathogenesis. Nearly 250 cases have been reported to date. CPC is characterized by a definite defect in the mid-clavicle at birth and is usually diagnosed when the deformity becomes evident in late childhood or adolescence. Surgical management is controversial, especially in asymptomatic children, with various techniques reported in the literature. CASE REPORT: We report a case of a 6-year-old boy who was diagnosed with CPC during a medical examination for primary school enrollment. Operative treatment included debridement of pseudoarthrosis, internal fixation with third tube plate, and barrel-shaped mono-cortical iliac crest autograft. RESULTS: A complete bone union was obtained 9 months after the operation, and satisfactory function and cosmetic appearance were observed 4 years and 3 months postoperatively. CONCLUSION: In our opinion, reconstruction with barrel-shaped mono-cortical iliac crest autograft was an effective and reproducible surgical technique to treat CPC.

Does An Osteotomy Performed in Congenital Pseudarthrosis of the Tibia Heal?

BACKGROUND: Shortening and deformity of the tibia commonly occur during the treatment of congenital pseudarthrosis of the tibia (CPT). The role of osteotomies in lengthening and deformity correction remains controversial in CPT. This study evaluates the approach to and outcome after osteotomy performed in CPT. METHODS: We performed an IRB approved retrospective review of consecutive patients with CPT treated at our institution from 2010 through 2019. Patients who underwent osteotomies were included in this study. RESULTS: Nine patients (10 osteotomies-5 proximal metaphyseal and 5 diaphyseal) with a median age at osteotomy of 8.9 years (range: 4 to 21 y) were included. Six patients had neurofibromatosis-1, 1 had cleidocranial dysplasia, and 2 patients had idiopathic CPT. Four osteotomies were performed for deformity correction, 3 osteotomies to allow intramedullary instrumentation, and 3 osteotomies for lengthening. Five osteotomies were preceded by zolendronate treatment before surgery. Nine were fixed with a rod supplemented with external fixation (7) or locking plates (2). One osteotomy was stabilized with locked intramedullary nailing alone. Four osteotomies were supplemented with autologous bone graft, and bone morphogenic protein-2 was utilized in 3 osteotomies. Median time to healing was 222.5 days (range: 124 to 323 d). One osteotomy (locked intramedullary nailing) required grafting at 5.5 months and then healed uneventfully. Median healing index for patients undergoing lengthening was 57.9 days/cm (range: 35 to 81 d/cm). All 3 osteotomies performed for lengthening required a second osteotomy for preconsolidation at a mean of 34 days. Other complications included compartment syndrome requiring fasciotomy (n=2), tibial osteomyelitis (n=1), and fracture distal to cross-union (n=1). CONCLUSIONS: Contrary to much of the established practice, osteotomies may be safely performed in CPT for various indications. All osteotomies healed with only 1 osteotomy requiring secondary bone grafting. Although time to healing of the osteotomy was generally prolonged, this study suggests, somewhat surprisingly, that preconsolidation can occur frequently in lengthening procedures. LEVEL OF EVIDENCE: Level IV-case series.

Combination Treatment by Cross-Union of the Tibia and Fibula, Autogenic Iliac Bone Grafting, Reliable Fixation and Bone Morphogenetic Proteins for the Treatment of Refractory Congenital Pseudarthrosis of the Tibia.

BACKGROUND: The treatment of congenital pseudarthrosis of the tibia (CPT) remains a challenge because of the difficulties of achieving and maintaining bone union, as well as complications of joint deformity and limb-length discrepancy. The purpose of this study was to evaluate the efficacy of cross-union of the tibia and fibula in achieving union and preventing refracture for patients with refractory CPT as a complementary approach to improve upon conventional surgical treatments. METHODS: A retrospective study including patients with refractory CPT who attended our department between June 2014 and August 2020. Eighteen CPT patients, who had sustained refracture that required cast immobilization or secondary surgery, and were managed by pseudarthrosis resection, cross-union of the tibia and fibula, bone morphogenetic protein-2 and autogenous iliac bone grafting, were included. Clinical outcomes of the bone union rate and the frequency of refracture after performing cross-union of the tibia and fibula were assessed during the follow-up period. RESULTS: The mean follow-up period was 4.3 years (range: 1.5 to 6.25 y). The mean age of the patients at surgery was 5.4 years (range: 2.6 to 10 y), and all 18 (100%) of the 18 patients had final healing at the site of pseudarthrosis. The average time spent to achieve radiologic bone union of the pseudarthrosis after operation was 2.96 months (range: 2.2 to 4.1 mo). Two (11.1%) patients had an average 2.5 cm limb-length discrepancy, none (0%) sustained refracture which needed cast immobilization or secondary surgery. Patients were all pain-free and move actively. CONCLUSIONS: Cross-union of the tibia and fibula is a promising complementary procedure for treating refractory CPT patients. LEVEL OF EVIDENCE: Level IV-case series.

Evaluation of the efficacy of ipsilateral fibular transfer for reconstruction of large tibial defects in children: a retrospective study.

BACKGROUND: Reconstruction of large tibial defects is often a major challenge in limb salvage. This study aimed to evaluate initial follow-up results of ipsilateral fibula transfer for the treatment of large tibial defects in children. METHODS: A retrospective study was performed between September 2014 and April 2021. Ten children were identified as having large tibial defects. The children underwent ipsilateral fibula transfer. We then evaluated initial healing, tibial length discrepancy, ankle varus/valgus, fibular position, refracture, infection, and function. RESULTS: Five boys and five girls, with an average age of 7.2 years, were evaluated. The transferred fibula was united in the patients. The mean follow-up period after fibular transposition was 43 months. The patients achieved primary bone union; the mean time to union was 8.4 months (range, 4-18 months). Complications included refracture (30%), infection (40%), tibia malunion (30%), ankle varus (30%), sensory loss of toes (10%), and ankle valgus (10%). No other major complications were observed. All 10 patients were able to perform activities of daily living and return to their normal activities. CONCLUSION: Ipsilateral fibula transfer is a salvage surgery for the treatment of large tibial defects in children with congenital pseudoarthrosis of the tibia, traumatic nonunion of the tibia, and/or tibial defect after chronic osteomyelitis. However, long-term results still need to be followed up.