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Child's Nervous System : ChNS :... Oct 2020The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia....
UNLABELLED
The skeletal system is affected in up to 60% of patients with neurofibromatosis type 1. The most commonly observed entities are spinal deformities and tibial dysplasia. Early recognition of radiologic osseous dystrophy signs is of utmost importance because worsening of the deformities without treatment is commonly observed and surgical intervention is often necessary. Due to the relative rarity and the heterogenic presentation of the disease, evidence regarding the best surgical strategy is still lacking.
PURPOSE
To report our experience with the treatment of skeletal manifestations in pediatric patients with (neurofibromatosis type 1) NF-1 and to present the results with our treatment protocols.
MATERIALS AND METHODS
This is a retrospective, single expert center study on children with spinal deformities and tibial dysplasia associated with NF-1 treated between 2006 and 2020 in a tertiary referral institution.
RESULTS
Spinal deformity: Thirty-three patients (n = 33) were included. Mean age at index surgery was 9.8 years. In 30 patients (91%), the deformity was localized in the thoracic and/or lumbar spine, and in 3 patients (9%), there was isolated involvement of the cervical spine. Eleven patients (33%) received definitive spinal fusion as an index procedure and 22 (67%) were treated by means of "growth-preserving" spinal surgery. Halo-gravity traction before index surgery was applied in 11 patients (33%). Progression of deformity was stopped in all patients and a mean curve correction of 60% (range 23-98%) was achieved. Mechanical problems with instrumentation requiring revision surgery were observed in 55% of the patients treated by growth-preserving techniques and in none of the patients treated by definitive fusion. One patient (3%) developed a late incomplete paraplegia due to a progressive kyphotic deformity. Tibial dysplasia: The study group comprised of 14 patients. In 5 of them (36%) pathological fractures were present on initial presentation. In the remaining 9 patients (64%), anterior tibial bowing without fracture was observed initially. Four of them (n = 4, 28%) subsequently developed a pathologic fracture despite brace treatment. Surgical treatment was indicated in 89% of the children with pathological fractures. This involved resection of the pseudarthrosis, autologous bone grafting, and intramedullary nailing combined with external fixation in some of the cases. In 50% of the patients, bone morphogenic protein was used "off-label" in order to promote union. Healing of the pseudarthrosis was achieved in all of the cases and occurred between 5 to 13 months after the index surgical intervention. Four of the patients treated surgically needed more than one surgical intervention in order to achieve union; one patient had a re-fracture. All patients had a good functional result at last follow-up.
CONCLUSION
Early surgical intervention is recommended for the treatment dystrophic spinal deformity in children with NF-1. Good and sustainable curve correction without relevant thoracic growth inhibition can be achieved with growth-preserving techniques alone or in combination with short spinal fusion at the apex of the curve. Preoperative halo-gravity traction is a safe and very effective tool for the correction of severe and rigid deformity in order to avoid neurologic injury. Fracture union in tibial dysplasia with satisfactory functional results can be obtained in over 80% of the children by means of surgical resection of the pseudarthrosis, intramedullary nailing, and bone grafting. Wearing a brace until skeletal maturity is achieved is mandatory in order to minimize the risk of re-fracture.
Topics: Child; Humans; Kyphosis; Neurofibromatosis 1; Pseudarthrosis; Retrospective Studies; Spinal Fusion; Treatment Outcome
PubMed: 32613421
DOI: 10.1007/s00381-020-04775-4 -
Acta Orthopaedica Et Traumatologica... 2013The aim of this study was to evaluate union rates and complications of Hyalonect, a knitted mesh composed of HYAFF, a benzyl ester of hyaluronic acid, and a naturally...
OBJECTIVE
The aim of this study was to evaluate union rates and complications of Hyalonect, a knitted mesh composed of HYAFF, a benzyl ester of hyaluronic acid, and a naturally occurring constituent of the extracellular matrix, for the treatment of pseudarthrosis.
METHODS
The study included 11 patients (8 male, 3 female; mean age: 44.6 years; range: 23 to 57 years) operated for pseudarthrosis using Hyalonect. Average time between initial treatment and surgical procedure was 12.9 (range: 8 to 48) months. Pseudarthrosis of the tibia, femur and humerus was present in 4, 2 and 5 patients, respectively. All patients had undergone prior surgery (1 to 6 times). Each patient underwent open reduction and internal fixation. Allograft was applied to the pseudarthrosis area and covered with Hyalonect. Mean follow up period was 31 (range: 12 to 48) months.
RESULTS
Union was achieved in all patients after an average of 6 (range: 4 to 8) months. One patient had a discharge for two weeks. Another developed an infection which responded well to appropriate antibiotic treatment. No malunion or implant failure was observed. One patient with pre-existing radial nerve palsy maintained the condition.
CONCLUSION
Use of Hyalonect appears to be a safe method with a positive impact on union in the surgical treatment of pseudarthrosis, particularly in the absence of any infection.
Topics: Adult; Allografts; Female; Femur; Follow-Up Studies; Fracture Fixation, Internal; Fracture Healing; Humans; Humerus; Hyaluronic Acid; Male; Middle Aged; Pseudarthrosis; Radial Nerve; Range of Motion, Articular; Reoperation; Risk Factors; Surgical Mesh; Tibia; Treatment Outcome; Viscosupplements
PubMed: 24509216
DOI: 10.3944/aott.2013.2875 -
Frontiers in Genetics 2023To analyze the lncRNA-mRNA differential expression and co-expression network of periosteal stem cells (PSCs) from congenital pseudarthrosis of the tibia (CPT) and...
To analyze the lncRNA-mRNA differential expression and co-expression network of periosteal stem cells (PSCs) from congenital pseudarthrosis of the tibia (CPT) and normal patients, and to explore the role of key lncRNAs. Differentially expressed lncRNAs and mRNAs in PSCs were obtained by sequencing, and biological functions of differentially expressed mRNAs were detected by gene ontology (GO), Kyoto encyclopedia of genes and genomes (KEGG) pathway and protein -protein interaction (PPI) analysis. The co-expression network of lncRNA-mRNA was constructed by correlation analysis of differentially expressed lncRNAs and mRNAs, and the key lncRNAs were screened according to the connectivity degree. After that, the cis-regulated target genes of differential expressed lncRNAs and mRNAs were predicted. A total of 194 differentially expressed lncRNAs were identified, including 73 upregulated and 121 downregulated genes. A total of 822 differentially expressed mRNAs were identified, including 311 upregulated and 511 downregulated genes. GO, KEGG and PPI enrichment analysis showed that the regulatory function of differentially expressed mRNAs were mainly gathered in skeletal system development and tissue morphogenesis. The co-expression network with 226 nodes and 3,390 edges was constructed based on correlation analysis. A total of 10 key lncRNAs, including FAM227B, POM121L9P, AF165147 and AC103702, were screened according to connectivity degree. Prediction of target genes indicated that FAM227B-FGF7 and AC103702-HOXB4/5/6 may play an important role in the pathogenesis of CPT. A total of 10 key lncRNAs, including FAM227B, POM121L9P, AF165147, and AC103702, occupy the core position in the co-expression network, suggesting that these lncRNAs and their target genes may play an important role in the pathogenesis of CPT.
PubMed: 36814904
DOI: 10.3389/fgene.2023.1094298 -
Global Spine Journal Apr 2015Study Design Systematic literature review and meta-analysis. Objective This study is a meta-analysis assessing the fusion rate and the clinical outcomes of cervical... (Review)
Review
Study Design Systematic literature review and meta-analysis. Objective This study is a meta-analysis assessing the fusion rate and the clinical outcomes of cervical pseudarthrosis treated with either a posterior or a revision anterior approach. Methods A literature search of PubMed, Cochrane, and Embase was performed. Variables of interest included fusion rate and clinical success. The effect size based on logit event rate was calculated from the pooled results. The studies were weighted by the inverse of the variance, which included both within- and between-study error. The confidence intervals were reported at 95%. Heterogeneity was assessed using the Q statistic and I (2), where I (2) is the estimate of the percentage of error due to between-study variation. Results Sixteen studies reported fusion outcomes; 10 studies reported anterior and/or posterior results. The pooled fusion success was 86.4% in the anterior group and 97.1% in the posterior group (p = 0.028). The anterior group demonstrated significant heterogeneity with Q value of 34.2 and I (2) value of 73.7%; no heterogeneity was seen in the posterior group. The clinical outcomes were reported in 10 studies, with eight reporting results of anterior and posterior approaches. The pooled clinical success rate was 77.0% for anterior and 71.7% for posterior (p = 0.55) approaches. There was significant heterogeneity in both groups (I (2) 16.1; 19.2). Conclusions Symptomatic cervical pseudarthrosis can be effectively managed with either an anterior or a posterior approach. The posterior approach demonstrates a significantly greater fusion rate compared with the anterior approach, though the clinical outcome does not differ between the two groups.
PubMed: 25844290
DOI: 10.1055/s-0035-1544176 -
Asian Spine Journal Oct 2018Retrospective cohort study (level of evidence: 4).
STUDY DESIGN
Retrospective cohort study (level of evidence: 4).
PURPOSE
To describe the potential comorbid, operative, and radiographic risk factors for the development of clinically-relevant pseudarthrosis following minimally-invasive transforaminal lumbar interbody fusion (MIS-TLIF).
OVERVIEW OF LITERATURE
MIS-TLIF has shown long-term clinical outcomes with decreased perioperative morbidity and earlier return to work, similar to those of open TLIF. However, unsuccessful fusion still remains a concern. The impacts of various patient, operative, and radiographic risk factors have not been evaluated for their potential association with pseudarthrosis related to MIS-TLIF.
METHODS
Between 2012 and 2015, 204 consecutive patients underwent one or two-level MIS-TLIF at St. Joseph's University Medical Center, Paterson, NJ, USA; they had a minimum of 1 year of follow-up. The patients were divided into two cohorts: those who developed clinically-relevant pseudarthrosis and those who did not. Clinically-relevant pseudarthrosis was determined by both evidence on computed tomography and presence of continued clinical symptoms at 1-year follow-up.
RESULTS
Revision surgery was the only identified non-radiographic factor associated with pseudarthrosis. Disc angle had the highest (R 2=0.8), followed by anterior disc height (R 2=0.79). Although posterior disc height and the ratio of anterior to posterior disc height showed a marked relationship with the outcome, the R 2-values were <0.3, thus indicating a less-strong correlation. The overall pseudarthrosis rate was 8%. No statistically significant differences were identified between the two cohorts with respect to mean age, sex, medical comorbidities, smoking status, or number of levels fused.
CONCLUSIONS
Clinically-relevant pseudarthrosis is not uncommon following MIS-TLIF. In the current study, undergoing revision surgery, disc angle, and anterior disc height were observed to be associated with clinically-relevant pseudarthrosis. This study demonstrated that the patient population may benefit from an alternate approach.
PubMed: 30213165
DOI: 10.31616/asj.2018.12.5.830 -
Scientific Reports May 2022Our study aimed to evaluate differences in outcomes of patients submitted to spinal fusion using different grafts measuring the effectiveness of spinal fusion rates,... (Meta-Analysis)
Meta-Analysis
Our study aimed to evaluate differences in outcomes of patients submitted to spinal fusion using different grafts measuring the effectiveness of spinal fusion rates, pseudarthrosis rates, and adverse events. Applying the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement, this systematic review and meta-analysis identified 64 eligible articles. The main inclusion criteria were adult patients that were submitted to spinal fusion, autologous iliac crest (AIC), allograft (ALG), alloplastic (ALP; hydroxyapatite, rhBMP-2, rhBMP-7, or the association between them), and local bone (LB), whether in addition to metallic implants or not, was applied. We made a comparison among those groups to evaluate the presence of differences in outcomes, such as fusion rate, hospital stay, follow-up extension (6, 12, 24, and 48 months), pseudarthrosis rate, and adverse events. Sixty-four studies were identified. LB presented significantly higher proportions of fusion rates (95.3% CI 89.7-98.7) compared to the AIC (88.6% CI 84.8-91.9), ALG (87.8% CI 80.8-93.4), and ALP (85.8% CI 75.7-93.5) study groups. Pseudarthrosis presented at a significantly lower pooled proportion of ALG studies (4.8% CI 0.1-15.7) compared to AIC (8.6% CI 4.2-14.2), ALP (7.1% CI 0.9-18.2), and LB (10.3% CI 1.8-24.5). ALP and AIC studies described significantly more cases of adverse events (80 events/404 patients and 860 events/2001 patients, respectively) compared to LB (20 events/311 patients) and ALG (73 events/459 patients). Most studies presented high risk-of-bias scores. Based on fusion rates and adverse events proportions, LB showed a superior trend among the graft cases we analyzed. However, our review revealed highly heterogeneous data and a need for more rigorous studies to better address and assist surgeons' choices of the best spinal grafts.
Topics: Adult; Bone Transplantation; Humans; Ilium; Pseudarthrosis; Spinal Diseases; Spinal Fusion; Treatment Outcome
PubMed: 35534520
DOI: 10.1038/s41598-022-11551-8 -
Journal of Children's Orthopaedics Jun 2022Congenital pseudarthrosis of the tibia is a rare disease that is particularly difficult to treat; the most difficult complications include nonunion of the tibia,...
PURPOSE
Congenital pseudarthrosis of the tibia is a rare disease that is particularly difficult to treat; the most difficult complications include nonunion of the tibia, refracture, and failed surgery. This study aimed to evaluate the efficiency of transposing gastrocnemius flaps for the treatment of congenital pseudarthrosis of the tibia.
METHODS
Nine patients (aged 6.2 ± 3.6 years) diagnosed with congenital pseudarthrosis of the tibia in our hospital between March 2013 and March 2018 were enrolled. The tibial pseudarthrosis and thickened periosteum were completely removed, and intramedullary nails were used to fix the tibia. Bone harvest from the iliac, mixed with allogenic bone, was filled in the gap created by excision of the pseudarthrosis site and the surrounding periosteum; the gastrocnemius flap was then used to wrap the pseudoarthrosis site. The plaster cast was fixed postoperatively. The tibial union was evaluated via radiograph, and the plaster cast was removed after 12-24 weeks. Patients began walking approximately 12-14 weeks postoperatively.
RESULTS
Anatomical reduction was achieved in all the patients; the mean bone healing time was 10.1 ± 2.1 months. Bone nonunion was observed in one patient, and no neurovascular injury or wound infection occurred. Limb length discrepancy was in the range 3.2 ± 1.8 cm at 1 year and 4.7 ± 2.7 cm at 2 years after surgery. Two patients underwent replacement of the intramedullary nail, and eight patients exhibited good functional and radiographic outcomes.
CONCLUSION
This preliminary study proved that using the gastrocnemius muscle flap to cover the pseudarthrosis site was an effective method to promote the tibial union and treat congenital pseudarthrosis of the tibia.
PubMed: 35800657
DOI: 10.1177/18632521221097525 -
Journal of Children's Orthopaedics Apr 2019Congenital pseudarthrosis of the tibia (CPT) is likely to be a primary periosteal disease and secondary bone disease. The primary goal of treatment is to obtain union,...
UNLABELLED
Congenital pseudarthrosis of the tibia (CPT) is likely to be a primary periosteal disease and secondary bone disease. The primary goal of treatment is to obtain union, correct the diaphyseal deformity, correct any proximal fibular migration and prevent refracture. The pathobiology demonstrates increased osteoclasis by the surrounding fibrous hamartoma and reduced osteogenesis and bone morphogenic protein production by the bone. This leads to a loss of remodelling potential and gradual bowing and atrophy of the bone with eventual fracture of the tibia and or fibula. This recommends the synergistic use of bisphosphonates and bone morphogenic protein. The pathomechanics of CPT implicate the anterolateral bowing, narrow diameter of the atrophic bone ends and proximal fibular migration. These biomechanical factors can be addressed by means of straightening of the deformity, intramedullary support of both bones, stable fixation and reduction of proximal migration of the fibula. A summary of the literature on CPT shows that the mean probability of achieving primary union without refracture, by most treatments is 50% (12% to 80%). Two recent studies have shown a much higher success rate approaching 100%, by creating a cross-union between the tibia and fibula. The cross-union with intramedullary reinforcement of the bone makes refracture unlikely due to the cross-sectional area of union with its two-bar linkage. A new classification to guide such treatment is also proposed.
LEVEL OF EVIDENCE
V - expert opinion.
PubMed: 30996736
DOI: 10.1302/1863-2548.13.180147 -
Journal of Orthopaedics and... Sep 2022Congenital pseudarthrosis of the tibia (CPT) is a challenging problem in orthopedic practice, with high rates of non-union, refracture, and residual deformities. After...
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.
Topics: Child; Child, Preschool; Female; Humans; Ilizarov Technique; Male; Pseudarthrosis; Retrospective Studies; Tibia
PubMed: 36149607
DOI: 10.1186/s10195-022-00667-2 -
BMC Surgery May 2022Symptomatic pseudarthrosis and cage migration/protrusion are difficult complications of transforaminal or posterior lumbar interbody fusion (TLIF/PLIF). If the patient...
BACKGROUND
Symptomatic pseudarthrosis and cage migration/protrusion are difficult complications of transforaminal or posterior lumbar interbody fusion (TLIF/PLIF). If the patient experiences severe radicular symptoms due to cage protrusion, removal of the migrated cage is necessary. However, this procedure is sometimes very challenging because epidural adhesions and fibrous union can be present between the cage and vertebrae. We describe a novel classification and technique utilizing a navigated osteotome and the oblique lumbar interbody fusion at L5/S1 (OLIF51) technique to address this problem.
METHODS
This retrospective study investigated consecutive patients with degenerative lumbar diseases who underwent TLIF/PLIF. Symptomatic cage migration was evaluated by direct examination, radiography, and/or computed tomography (CT) at 1, 3, 6, 12, and 24 months of follow-up. Cage migration/protrusion was defined as symptomatic cage protrusion > 5 mm from the posterior border of the over and underlying vertebral body compared with initial CT. We evaluated patient characteristics including body mass index, smoking history, fusion level, and cage type. A total of 113 patients underwent PLIF/TLIF (PLIF n = 30, TLIF n = 83), with a mean age of 71.1 years (range, 28-87 years). Mean duration of follow-up was 25 months (range, 12-47 months).
RESULTS
Cage migration was identified in 5 of 113 patients (4.4%). All cases of symptomatic cage migration involved the L5/S1 level and the TLIF procedure. Risk factors for cage protrusion were age (younger), sex (male), and level (L5/S1). The mean duration to onset of cage protrusion was 3.2 months (range, 2-6 months). We applied a new classification for cage protrusion: type 1, only low back pain without new radicular symptoms; type 2, low back pain with minor radicular symptoms; or type 3, cauda equina syndrome and/or severe radicular symptoms. According to our classification, one patient was in type 1, three patients were in type 2, and one patient was in type 3. For all cases of cage migration, revision surgery was performed using a navigated high-speed burr and osteotome, and the patient in group 1 underwent additional PLIF without removal of the protruding cage. Three revision surgeries (group 2) involved removal of the protruding cage and PLIF, and one revision surgery (group 3) involved anterior removal of the cage and OLIF51 fusion.
CONCLUSIONS
The navigated high-speed burr, navigated osteotome, and OLIF51 technique appear very useful for removing a cage with fibrous union from the disc in patients with pseudarthrosis. This new technique makes revision surgery after cage migration much safer, and more effective. This technique also reduces the need for fluoroscopy.
Topics: Aged; Humans; Low Back Pain; Lumbar Vertebrae; Male; Pseudarthrosis; Reoperation; Retrospective Studies; Spinal Fusion; Treatment Outcome
PubMed: 35546229
DOI: 10.1186/s12893-022-01620-0