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1.
Bone Joint J ; 106-B(5): 508-514, 2024 May 01.
Artigo em Inglês | MEDLINE | ID: mdl-38688504

RESUMO

Aims: The aim of this study is to evaluate the surgical treatment with the best healing rate for patients with proximal femoral unicameral bone cysts (UBCs) after initial surgery, and to determine which procedure has the lowest adverse event burden during follow-up. Methods: This multicentre retrospective study was conducted in 20 tertiary paediatric hospitals in France, Belgium, and Switzerland, and included patients aged < 16 years admitted for UBC treatment in the proximal femur from January 1995 to December 2017. UBCs were divided into seven groups based on the index treatment, which included elastic stable intramedullary nail (ESIN) insertion with or without percutaneous injection or grafting, percutaneous injection alone, curettage and grafting alone, and insertion of other orthopaedic hardware with or without curettage. Results: A total of 201 patients were included in the study. The mean age at diagnosis was 8.7 years (SD 3.9); 77% (n = 156) were male. The mean follow-up was 9.4 years (SD 3.9). ESIN insertion without complementary procedure had a 67% UBC healing rate after the first operation (vs 30% with percutaneous injection alone (p = 0.027), 43% with curettage and grafting (p = 0.064), and 21% with insertion of other hardware combined with curettage (p < 0.001) or 36% alone (p = 0.014)). ESIN insertion with percutaneous injection presented a 79% healing rate, higher than percutaneous injection alone (p = 0.017), curettage and grafting (p = 0.028), and insertion of other hardware combined with curettage (p < 0.001) or alone (p = 0.014). Patients who underwent ESIN insertion with curettage had a 53% healing rate, higher than insertion of other hardware combined with curettage (p = 0.009). The overall rate of postoperative complications was 25% and did not differ between groups (p = 0.228). A total of 32 limb length discrepancies were identified. Conclusion: ESIN insertion, either alone or combined with percutaneous injection or curettage and grafting, may offer higher healing rates than other operative procedures. Limb length discrepancy remains a major concern, and might be partly explained by the cyst's location and the consequence of surgery. Therefore, providing information about this risk is crucial.


Assuntos
Cistos Ósseos , Curetagem , Fêmur , Humanos , Criança , Masculino , Estudos Retrospectivos , Feminino , Cistos Ósseos/cirurgia , Cistos Ósseos/diagnóstico por imagem , Curetagem/métodos , Fêmur/cirurgia , Transplante Ósseo/métodos , Pinos Ortopédicos , Adolescente , Pré-Escolar , Resultado do Tratamento , Seguimentos
2.
Pediatr Radiol ; 54(2): 324-336, 2024 02.
Artigo em Inglês | MEDLINE | ID: mdl-38238598

RESUMO

BACKGROUND: In the presence of pain over the lateral aspect of the foot or recurrent ankle sprain in children, medical imaging is often employed to investigate potential causes, such as a calcaneonavicular coalition or a too-long anterior process (TLAP) of the calcaneus. Diagnosis and categorization of calcaneonavicular coalitions (synostosis, synchondrosis, or synfibrosis) is generally facilitated through imaging, in contrast to TLAP, which lacks well-defined semiological characteristics, apart from a calcaneonavicular space measurement of less than 5 mm. However, this measurement initially performed on an oblique view radiograph can be subject to a lack of precision due to positional variations of the foot and overlapping bones. Furthermore, the differentiation between TLAP as an anatomical variant and TLAP syndrome (characterized by symptomatic presentation), remains a subject of uncertainty. OBJECTIVE: The objective of our retrospective study was to investigate the imaging diagnosis of TLAP syndrome. MATERIALS AND METHODS: A retrospective unmatched case-control study was conducted, covering data from February 2014 to January 2021. All patients, included retrospectively and consecutively, were initially managed in our hospital with radiography and/or computed tomography (CT) and/or magnetic resonance imaging (MRI). Two radiologists independently reviewed the images taken (radiographs, CT scans, and MRIs) of patients undergoing treatment in pediatric orthopedics for TLAP syndrome and control subjects, utilizing a standardized questionnaire. The control group consisted of subjects with no features suggestive of TLAP syndrome. The questionnaire included measurements of the calcaneonavicular space and identification of indirect signs associated with calcaneonavicular coalitions, as described in the related literature. RESULTS: A total of 128 patients who met the inclusion criteria were included in the analysis, including 38 patients and 90 controls. The prevalence of TLAP was 71.5% in the study population and 62.6% among controls. A threshold measurement of the calcaneonavicular space at 3.2 mm favored TLAP syndrome (sensitivity=97%, specificity=70%, area under the curve [95% confidence interval] =0.881[0.812-0.949]), with better precision using CT. Three indirect signs were significant: the "anteater nose" sign, the talar beak, and the tapered anterior calcaneal process. These signs demonstrated an even stronger association with TLAP syndrome when observed in conjunction with a reduction in the calcaneonavicular space, particularly in CT scans. CONCLUSION: TLAP is common among control subjects. Therefore, a variant appears to be the most plausible explanation and it can be considered a mild form of calcaneonavicular coalition. However, in conjunction with symptoms suggestive of TLAP syndrome, the diagnosis is further supported by imaging, specifically with a calcaneonavicular space measurement of less than 3.2 mm. This measurement is most accurately obtained using CT with 2-dimensional reconstructions in all three planes. The simultaneous presence of the "anteater nose" sign, the talar beak, or the tapered anterior calcaneal process provides additional diagnostic evidence. In the diagnostic approach of calcaneonavicular coalition, oblique foot radiography seems useful in initially detecting abnormal coalition (bony or not), and complementarily, CT emerges as the best modality to characterize TLAP syndrome.


Assuntos
Sinostose , Ossos do Tarso , Criança , Humanos , Adolescente , Estudos Retrospectivos , Estudos de Casos e Controles , Vermilingua
4.
Orthop Traumatol Surg Res ; : 103636, 2023 Jun 01.
Artigo em Inglês | MEDLINE | ID: mdl-37268156

RESUMO

BACKGROUND: Treatment of scaphoid waist fractures is generally conservative in children but surgical in adults, given the relatively high risk of non-union in adults. In adolescents, the required therapeutic strategy is less well defined. The objective of this study was to compare the radiographic and clinical parameters, and the rate of complications, between non-surgical orthopedic treatment (OT) and surgical treatment (ST) by percutaneous screw fixation of these fractures in adolescents approaching skeletal maturity. HYPOTHESIS: ST of non-displaced scaphoid waist fractures in adolescents allows radiographic union, a functional result and a complication rate comparable to that of ST. METHODS: This single-center retrospective study included patients who presented with a non-displaced scaphoid waist fracture, with a chronological age (CA) and a bone age (BA) between 14 and 18 years. Clinical and radiographic parameters and complications were analyzed during the trauma and at one year, including functional scores, between two groups of patients; OT and ST. RESULTS: Thirty-seven patients had OT (63.8%) and 21 had ST (36.2%). The median CA was 16 years [14.25-16]. The median BA was 16 years [15;17] according to the Greulich and Pyle method and corresponded to R9 [R7-R10] and U7 [U7;U8] according to the Distal Radius and Ulnar (DRU) classification system. All non-unions were found in the OT group (23.4% vs. 0%, p=0.019). The duration of immobilization (8 weeks) and the number of consultations were higher after OT than ST. Functional scores were lower in patients with non-union after OT (p≤0.002) CONCLUSION: OT of scaphoid waist fractures in adolescents results in a higher rate of non-union than ST, similar to the rate found in adults. Findings from this study recommend a surgical approach by percutaneous screw fixation. LEVEL OF EVIDENCE: III; comparative retrospective study.

5.
Eur Spine J ; 32(9): 3118-3132, 2023 09.
Artigo em Inglês | MEDLINE | ID: mdl-37382696

RESUMO

PURPOSE: The aim of this study was to evaluate the effects of definitive arthrodesis on frontal and sagittal spine balance in EOS patients treated with MCGR, as well as the complications associated with this procedure and the outcome at last follow-up. METHODS: This was a multicentre study in 10 French centres. All patients who underwent posterior spinal arthrodesis after completion of MCGR treatment, regardless of age, etiology of scoliosis between 2011 and 2022, were included. RESULTS: A total of 66 patients who had a final fusion after the lengthening programme were included in the study. The mean follow-up time was 5.5 ± 1.7 years (range: 2.1-9). The mean follow-up time after arthrodesis was 24 ± 18 months (range: 3-68) and the mean age at arthrodesis was 13.5 ± 1.5 years (range: 9.5-17). The main and secondary curves were significantly (p < 0.005 and p = 0.03) improved by arthrodesis (16.4° and 9° respectively) and stabilised at the last follow-up. The T1-T12 and T1-S1 distances increased by 8.4 mm and 14 mm with spinal fusion, with no significant difference (p = 0.096 and p = 0.068). There was no significant improvement in the rest of the parameters with arthrodesis, nor was there any significant deterioration at last follow-up. After final fusion, there were a total of 24 complications in 18 patients (27.3%) that routinely led to repeat surgery. CONCLUSION: Final fusion after MCGR provides satisfactory additional correction of the main and secondary curves and a moderate increase in the T1-T12 distance but has no impact on sagittal balance and other radiological parameters. The post-operative complication rate is particularly high in patients at risk of complications. LEVEL OF EVIDENCE: Level 4.


Assuntos
Escoliose , Coluna Vertebral , Humanos , Criança , Adolescente , Seguimentos , Resultado do Tratamento , Coluna Vertebral/cirurgia , Escoliose/diagnóstico por imagem , Escoliose/cirurgia , Radiografia , Estudos Retrospectivos
6.
Eur Spine J ; 32(7): 2558-2573, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37227519

RESUMO

PURPOSE: The purpose of this study was to investigate patients with early-onset scoliosis (EOS) who completed their electromagnetic lengthening rod program to assess the demographics of this population and to analyze the evolution of clinical and radiological parameters and the occurrence of complications. METHODS: This was a multicenter study with 10 French centers. We collected all patients with EOS who had undergone electromagnetic lengthening between 2011 and 2022. They had to have reached the end of the procedure (graduate). RESULTS: A total of 90 graduate patients were included. The mean follow-up time over the entire period was 66 months (25.3-109). Of these, only 66 patients (73.3%) underwent definitive spinal arthrodesis at the end of the lengthening phase, whereas 24 patients (26.7%) kept their hardware in situ with a mean follow-up time from the last lengthening of 25 months (3-68). Patients had an average of 2.6 surgeries (1-5) over the entire follow-up. Patients had an average of 7.9 lengthenings for a mean total lengthening of 26.9 mm (4-75). Analysis of the radiological parameters showed a percentage reduction in the main curve of 12 to 40%, depending on the etiology, with an average reduction of 73-44°, and an average thoracic height of 210 mm (171-214) for an average improvement of 31 mm (23-43). There was no significant difference in the sagittal parameters. During the lengthening phase, there were a total of 56 complications in 43 patients (43.9%; n = 56/98), of which 39 in 28 patients (28.6%) resulted in unplanned surgery. In the graduate patients, there were a total of 26 complications in 20 patients (22.2%), all of which resulted in unscheduled surgery. CONCLUSION: MCGR, allow to decrease the number of surgeries, to progressively improve the scoliotic deformity and to reach a satisfactory thoracic height at the price of an important complication rate linked in particular to the complexity of the management of patients with an EOS.


Assuntos
Escoliose , Fusão Vertebral , Humanos , Seguimentos , Escoliose/diagnóstico por imagem , Escoliose/cirurgia , Radiografia , Estudos Retrospectivos , Resultado do Tratamento
7.
Eur Spine J ; 31(9): 2287-2294, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35593932

RESUMO

PURPOSE: To determine the midterm outcome of lateral thoracotomy (LT) in skeletally immature patients concerning thoracogenic scoliosis development and lung parenchyma resection (LPR) extent. METHODS: In total, 129 children met the inclusion criteria: (1) LT during the study period; (2) skeletally immature at the time of LT; (3) clinical and radiographic follow-up of at least 3 years; and (4) no spinal or thoracic deformity on radiographs before LT. Patients were grouped according to their underlying disease, age at LT, and LPR extent. Radiographic parameters were assessed. Kaplan-Meier survival curves and univariate and multivariate analysis were performed. RESULTS: Of 129, 108 patients underwent pneumonectomy (9; 9.1%), lobectomy (79; 61.2%), segmentectomy (20; 15.5%) and 21 patients LT without LPR. The mean age at LT and at last radiological follow-up was 5.5 years (birth-17.8) and 15.2 years (3.4-33.2). The mean follow-up was 10 years (3-28.1). Scoliosis developed on average 5.3 years after LT. The mean Cobb angle was 22.1° (11-90°); > 10° in 37/129 patients (28.7%), of whom 5/129 (3.9%) had > 45°. The average vertebral rotation was 16.2° (2-43°; grade 0-II). RVAD was 26.5° (8°-33°) and 15.3° (2-43°) in patients with Cobb > 45° and ≤ 45°. Gender, age at surgery, and extent of LPR were not risk factors for post-thoracotomy scoliosis (p > 0.05), although younger patients developed a more severe deformity. CONCLUSION: Although post-thoracotomy scoliosis is not associated with significant rotation, the risk of curve progression > 45° is relatively high. Regular follow-up is required as scoliosis may develop several years after LT with or without LPR.


Assuntos
Escoliose , Fusão Vertebral , Criança , Seguimentos , Humanos , Estudos Retrospectivos , Escoliose/complicações , Escoliose/diagnóstico por imagem , Escoliose/cirurgia , Fusão Vertebral/efeitos adversos , Coluna Vertebral , Resultado do Tratamento
8.
Medicina (Kaunas) ; 58(5)2022 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-35630027

RESUMO

Background and objectives: The epidemiology and distribution of pediatric fractures change over time and are influenced by a multitude of factors including geography, climate, and population characteristics. The aims of our work were to study the distribution of traumatic pediatric orthopedic injuries admitted to the Lille University Hospital (LUH) Pediatric Emergency Department in 1999 and in 2019 and to analyze the epidemiological differences 20 years apart. Materials and methods: This was a retrospective, comparative, monocentric, and epidemiological study involving all children between 0 and 15 years and 3 months of age who consulted the pediatric emergencies of LUH from 1 January 1999 to 31 December 1999 and from 1 January 2019 to 31 December 2019. On admission, the following data were collected: sex, age at the time of injury, month and time of the day the trauma occurred (4:00 a.m to 11:59 a.m, 12:00 p.m. to 19:59 p.m, and 20:00 p.m to 3:59 a.m.), mechanism of injury, laterality (right or left), anatomical location, type of injury, and whether the fracture was closed or open. The type of treatment (orthopedic or surgical) was collected from the medical records. Results: A total of 939 children were included in 1999 compared with 781 in 2019 (21% decrease); the average age of children with fractures was significantly higher in 1999 (8.81 years) than in 2019 (7.19 years). This difference was explained by the majority involvement of older children (10-15 years) in 1999 (43% of fractures in 1999 versus 25% of fractures in 2019). Conversely, small children (1-5 years) had significantly more fractures in 2019 (36%) than in 1999 (24%). Conclusions: Overall, the types and sites of fractures did not change over the studied time despite a change in the population and mechanism of injury. This suggested that the reflexes of breaking a fall still tended to implicate and damage the same bone segments. Finally, the proportion of fractures managed surgically versus orthopedically has not evolved since 1999. Exploring this is a possible area of further research that would complement our study.


Assuntos
Fraturas Ósseas , Acidentes por Quedas , Adolescente , Criança , Fraturas Ósseas/epidemiologia , Hospitais Universitários , Humanos , Estudos Retrospectivos , Centros de Atenção Terciária
9.
Foot Ankle Surg ; 28(7): 1076-1082, 2022 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-35346595

RESUMO

INTRODUCTION: The too-long anterior process (TLAP) increases mechanical stress on the hindfoot and could lead to osteochondral lesions of the talus (OLT) by localized hyper-pressure. The purpose of this study is to investigate an association between TLAP and OLT in children. METHODS: This is a retrospective, multicenter, case-control study conducted between 2010 and 2020. The OLT group was compared to a control group (CoG). TLAP is characterized by a distance between the anteromedial process of the calcaneus and the navicular bone (CN) of< 5 mm. RESULTS: Forty-three feets were included in the OLT group and 92 in the CoG. The OLT group had a lower CN distance on CT than the CoG, a median of 2.8 mm versus 3.75 mm (p = 0.002); 86% of patients (37 feet/43) in the OLT group had a CN distance of< 5 mm (OR=3.0 [1.1; 9.5], p = 0.023) compared to 67% in the CoG. DISCUSSION: The OLT group had an increased risk of developing TLAP compared to the CoG. LEVEL OF EVIDENCE: III.


Assuntos
Calcâneo , Tálus , Estudos de Casos e Controles , Criança , Humanos , Imageamento por Ressonância Magnética , Estudos Retrospectivos , Tálus/cirurgia
10.
European J Pediatr Surg Rep ; 10(1): e1-e5, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35047327

RESUMO

Ewing's sarcoma is the second most frequent primary malignant bone tumor in adolescents and young adults. Locations on the thoracic wall represent up to 20% of primary and secondary locations. We present the case of a 13-year-old patient treated with the use of a radiolucency porous bioceramic prosthesis as a sternal replacement for a wide tumor resection in an oncologic context. Focal radiation therapy was not possible due to the high risk of severe myocardial injuries caused by the sternal location of the tumor. The sternum CERAMIL® (I.CERAM, Limoges, France), in porous alumina (Al 2 O 3 ) has already been implanted into adults in sternal replacement during its invasion by a tumor or its infectious destruction. There were no complication concerning the surgery. The last follow-up at 2 years postoperatively reveals a satisfactory clinical situation with any functional thoracic complaint and nor any functional respiratory symptoms. The porous alumina sternal prosthesis offers a reliable alternative for sternal replacement indications for children in an oncologic context.

11.
Orthop Traumatol Surg Res ; 108(1): 103167, 2022 02.
Artigo em Inglês | MEDLINE | ID: mdl-34871794

RESUMO

BACKGROUND: There is little consensus on the best treatment algorithm for unstable severe slipped capital femoral epiphysis (SCFE). Subcapital osteotomy, which is one of the surgical options, is performed either anteriorly (anterior cuneiform osteotomy, CO) or laterally with trochanteric osteotomy (Dunn procedure, DP). The CO is technically easier and decreases operating time. Moreover, because the DP was the standard in our department before it was replaced by the CO, we had a series of consecutive patients. Therefore, we did a retrospective case-control study in unstable, severe SCFEs treated by CO versus DP, which is to our knowledge the first one aiming to compare: (1) postoperative complications and in particular avascular necrosis, (2) functional outcome, (3) radiologic findings. HYPOTHESIS: CO is less or just as likely to cause avascular necrosis and has the same clinical and radiologic findings as DP. METHODS: A total of 41 patients (24 girls, i.e. 58.5%) were included between 2005 and 2018: 23 in the CO group and 18 in the DP group. The median age was 12.9 years (range, 11.5-14.9) and the median slip angle 70̊ (range, 62.5̊-80̊) with a median follow-up of 3 years (range, 2-4). Preoperative, intraoperative, and postoperative clinical and radiologic parameters (Southwick and alpha angles, and femoral head-neck offset) were analyzed, and all complications were documented. RESULTS: Two (8.7%) cases of avascular necrosis were reported in the CO group and 6 (33.3%) in the DP group (p=.11), with an overall rate of avascular necrosis of 19.5% (8/41). Five out of the 41 patients (12.2%) underwent a total hip arthroplasty: 1/23 (4.3%) in the CO group and 4/18 (22.2%) in the DP group (p=.16). Two (9.5%) patients in the CO group and 7 (38.9%) in the DP group developed postoperative limping before any arthroplasty was performed (p=.055). The alpha angle at follow-up (54±6.1̊ vs. 59.1±7.2̊; p=.027), Oxford hip score at follow-up (17/60 [range, 14-20] vs. 23.5 [range, 19-27]) (p=.021), operating time (132 min [range, 103-166] vs. 199.5 min [range, 142-215]) (p=.011) and intraoperative bleeding (250 mL [range, 100-350] vs. 300 mL [range, 197-450]) (p=.088) were more favorable in the CO group than in the DP group. CONCLUSIONS: The CO has similar results to DP in the surgical management of unstable severe SCFE. LEVEL OF EVIDENCE: III; retrospective comparative study.


Assuntos
Escorregamento das Epífises Proximais do Fêmur , Estudos de Casos e Controles , Criança , Epífises , Feminino , Seguimentos , Humanos , Masculino , Necrose , Osteotomia/métodos , Radiografia , Estudos Retrospectivos , Escorregamento das Epífises Proximais do Fêmur/diagnóstico por imagem , Escorregamento das Epífises Proximais do Fêmur/cirurgia , Resultado do Tratamento
12.
Orthop Traumatol Surg Res ; 106(1): 155-157, 2020 02.
Artigo em Inglês | MEDLINE | ID: mdl-31948876

RESUMO

In pectus excavatum, minimally invasive endoscopic repair is the current gold standard in adolescents. The main postoperative pitfall is bar displacement, making fixation a major issue. We report an original transcostal technique using absorbable USP-2 suture in 36 patients. There was a single case of bar displacement (2.78%; 95% CI: 0.07-14.53). Bar stability was comparable to literature reports; but the present technique avoided the complications potentially incurred by use of metal wire, pericostal fixation points and stabilizers.


Assuntos
Tórax em Funil , Adolescente , Tórax em Funil/diagnóstico por imagem , Tórax em Funil/cirurgia , Humanos , Procedimentos Cirúrgicos Minimamente Invasivos , Estudos Retrospectivos , Suturas , Resultado do Tratamento
13.
Eur Spine J ; 26(6): 1567-1576, 2017 06.
Artigo em Inglês | MEDLINE | ID: mdl-28040873

RESUMO

PURPOSE: Preliminary results of magnetically controlled growing rods (MCGR) are encouraging. However, only short case series of MCGR for the treatment of early onset scoliosis (EOS) have been reported. Our aim was to evaluate its effectiveness and complications. METHODS: We report a 30-case retrospective, consecutive, multicenter series of MCGR. Effectiveness was judged upon: deformity correction and difficulties to achieve desired distraction. Secondary endpoints included complications and revision surgeries. RESULTS: Median age at surgery was 9.1 years (5-13). Mean follow-up was 18.4 months (12-33.9). Mean Cobb angle was 66° preoperatively and 44° at latest follow-up. MCGR has avoided an average of 2.03 scheduled surgical procedures per patient compared to traditional growing rod (GR). The intended total length gain was 40.1 mm per patient (5-140) and the total measured length gain was 21.9 mm (45.5% discrepancy). There were 24 complications: 7 proximal pull-outs of the hooks, 3 rod breakages, 6 failures of the lengthening of which 4 complete blockages and 2 complete blockages followed by backtracking, 1 proximal junctional kyphosis, 1 wound dehiscence, 1 superficial infection, 1 deep infection requiring implant removal, 1 pulmonary embolism, 1 pulmonary insufficiency, 1 secondary lumbar scoliosis, and 1 painful outpatient distraction. Eight patients had a gradual loss of effectiveness of distractions. There were 13 revision surgeries in 9 patients. CONCLUSIONS: MCGR provides satisfactory deformity correction and avoids repeated surgical procedures for lengthening. However, it has substantial complication rate. Although less frequent than in GR, the law of diminishing returns also applies to MCGR.


Assuntos
Imãs , Osteogênese por Distração/instrumentação , Escoliose/cirurgia , Criança , Feminino , Seguimentos , Humanos , Masculino , Osteogênese por Distração/métodos , Complicações Pós-Operatórias/epidemiologia , Reoperação/estatística & dados numéricos , Estudos Retrospectivos , Resultado do Tratamento
14.
Am J Med Genet A ; 167A(12): 3204-8, 2015 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-26345137

RESUMO

Constitutive COL2A1 mutations are associated with a wide variety of clinical manifestations known as type II collagenopathies. Among them is Kniest dysplasia, which is phenotypically variable and includes both skeletal (short trunk and limbs, kyphoscoliosis, prominent joints, and osteoarthritis) and craniofacial characteristics. Kniest dysplasia mutations primarily arise in the triple-helicoidal region of the alpha 1 (II) chain in COL2A1 between exons 12 and 24. Somatic COL2A1 mutations have been identified in chondrosarcoma, a rare cartilage forming neoplasm, with a hypermutability of the gene reported in 37% of cases. However, to the best of our knowledge, there is no reported increase in predisposition to chondrosarcoma in human collagenopathies, and no reported clinical association between these congenital diseases and cartilaginous tumors. In the case study presented here, we report the first description of an association between these two rare diseases involving COL2A1, in a child presenting with Kniest dysplasia and a grade I sphenoethmoidal chondrosarcoma. We also describe a new constitutive mutation in COL2A1.


Assuntos
Neoplasias Ósseas/genética , Condrossarcoma/genética , Fissura Palatina/genética , Doenças do Colágeno/genética , Colágeno Tipo II/genética , Nanismo/genética , Face/anormalidades , Doença da Membrana Hialina/genética , Mutação/genética , Osteocondrodisplasias/genética , Neoplasias Ósseas/patologia , Condrossarcoma/patologia , Fissura Palatina/patologia , Doenças do Colágeno/patologia , Nanismo/patologia , Face/patologia , Humanos , Doença da Membrana Hialina/patologia , Recém-Nascido , Masculino , Osteocondrodisplasias/patologia , Prognóstico
15.
Spine (Phila Pa 1976) ; 38(25): E1589-99, 2013 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-24048087

RESUMO

STUDY DESIGN: Multicenter retrospective study of 54 children. OBJECTIVE: To describe the complication rate of the French vertical expandable prosthetic titanium rib (VEPTR) series involving patients treated between August 2005 and January 2012. SUMMARY OF BACKGROUND DATA: Congenital chest wall and spine deformities in children are complex entities. Most of the affected patients have severe scoliosis often associated with a thoracic deformity. Orthopedic treatment is generally ineffective, and surgical treatment is very challenging. These patients are good candidates for VEPTR expansion thoracoplasty. The aim of this study was to evaluate the potential complications of VEPTR surgery. METHODS: Of the 58 case files, 54 were available for analysis. The series involved 33 girls and 21 boys with a mean age of 7 years (range, 20 mo-14 yr and 2 mo) at primary VEPTR surgery. During the follow-up period, several complications occurred. RESULTS: Mean follow-up was 22.5 months (range, 6-64 mo). In total, 184 procedures were performed, including 56 VEPTR implantations, 98 expansions, and 30 nonscheduled procedures for different types of complications: mechanical complications (i.e., fracture, device migration), device-related and infectious complications, neurological disorders, spine statics disturbances. Altogether, there were 74 complications in 54 patients: a complication rate of 137% per patient and 40% per surgery. Comparison of the complications in this series with those reported in the literature led the authors to suggest solutions that should help decrease their incidence. CONCLUSION: The complication rate is consistent with that reported in the literature. Correct determination of the levels to be instrumented, preoperative improvement of nutritional status, and better evaluation of the preoperative and postoperative respiratory function are important factors in minimizing the potential complications of a technique that is used in weak patients with complex deformities.


Assuntos
Complicações Pós-Operatórias , Próteses e Implantes/efeitos adversos , Costelas/cirurgia , Coluna Vertebral/cirurgia , Parede Torácica/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Masculino , Pediatria , Complicações Pós-Operatórias/classificação , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Escoliose/congênito , Escoliose/cirurgia , Coluna Vertebral/anormalidades , Coluna Vertebral/patologia , Toracoplastia/métodos , Titânio , Resultado do Tratamento
16.
Pediatr Radiol ; 42(4): 503-7, 2012 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21965143

RESUMO

Dysplasia epiphysealis hemimelica (DEH), or Trevor disease, is a rare skeletal dysplasia characterised by an osteocartilaginous mass arising from an epiphysis. It affects single or multiple epiphyses in children, usually in the lower limb, predominantly the knee and ankle. Bilateral involvement is extremely rare, with only four cases reported in the literature to date. We report an additional case of bilateral DEH of the talus in a 14-year-old girl with an associated, not previously reported, intramuscular cartilaginous mass of the left lower leg. Clinical, radiological and pathological findings are presented. We emphasise the role of MRI and US in the diagnosis of DEH.


Assuntos
Doenças do Desenvolvimento Ósseo/reabilitação , Cartilagem/anormalidades , Cartilagem/diagnóstico por imagem , Perna (Membro)/diagnóstico por imagem , Tálus/diagnóstico por imagem , Adolescente , Feminino , Fêmur/anormalidades , Humanos , Radiografia , Tíbia/anormalidades
17.
Clin Orthop Relat Res ; 469(4): 1141-7, 2011 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-21086195

RESUMO

BACKGROUND: Numerous reports of THAs in patients younger than 30 years indicate a high risk of revision. Although risk factors for revision have been reported for older patients, it is unclear whether these risk factors are the same as those for patients younger than 30 years. QUESTIONS/PURPOSES: We therefore (1) determined function and survivorship of revision THAs performed in patients younger than 30 years, and (2) assessed the risk factors for revision THAs in this younger population by comparison with a group of patients younger than 30 years who did not undergo revision. PATIENTS AND METHODS: We retrospectively reviewed the clinical records and radiographs of 55 patients younger than 30 years (average age at revision, 24.3 years; range, 14-30 years) who underwent 77 hip revisions. Revision was performed, on average, 4.6 years (range, 0.4-12 years) after the primary THA. The results for these 55 patients (77 revision THAs) were compared with results for a nonrevised group, including 819 THAs in patients younger than 30 years. Minimum followup of the revision group was 1 year (mean, 6.2 years; range, 1-15 years). RESULTS: At followup after the revision, the Merle d'Aubigné-Postel score improved from 12.2 to 14.6. The rates of dislocation, neurologic lesions, and fractures were 15%, 7.8%, and 14%, respectively. The 10-year survival rate was 36% (95% confidence interval [CI], 21%-51%). Compared with the nonrevised group, the independent revision risk factors were young age at primary THA (OR 1.14 [1.07-1.19]), high number of previous surgeries (OR 5.41 [2.67-10.98]), and occurrence of at least one dislocation (OR 3.98 [1.74-9.07]). Hard-on-soft bearings had a higher risk (OR 3.42 [1.91-6.1]) of revision compared with hard-on-hard bearings. CONCLUSIONS: Revision THAs are likely in patients younger than 30 years, and the complication rate is high. The survivorship of hip revision in this population is low and alternative solutions should be advocated whenever possible. LEVEL OF EVIDENCE: Level III, therapeutic study, case control study. See the Guidelines for Authors for a complete description of levels of evidence.


Assuntos
Artroplastia de Quadril/instrumentação , Articulação do Quadril/cirurgia , Prótese de Quadril , Falha de Prótese , Adolescente , Adulto , Fatores Etários , Análise de Variância , Artroplastia de Quadril/efeitos adversos , Estudos de Casos e Controles , Distribuição de Qui-Quadrado , Feminino , França , Articulação do Quadril/diagnóstico por imagem , Articulação do Quadril/fisiopatologia , Humanos , Estimativa de Kaplan-Meier , Modelos Logísticos , Masculino , Estudos Multicêntricos como Assunto , Razão de Chances , Radiografia , Reoperação , Estudos Retrospectivos , Medição de Risco , Fatores de Risco , Fatores de Tempo , Resultado do Tratamento , Adulto Jovem
18.
J Bone Joint Surg Am ; 92(14): 2419-26, 2010 Oct 20.
Artigo em Inglês | MEDLINE | ID: mdl-20962192

RESUMO

BACKGROUND: Total hip arthroplasty in patients younger than thirty years of age represents a long-term challenge. As polyethylene wear secondary to a high activity level could be problematic, hard-on-hard bearings have been proposed to reduce wear. The aim of this retrospective case series was to assess the clinical and radiographic results of primary metal-on-metal total hip arthroplasty in patients thirty years of age or younger. METHODS: We retrospectively studied thirty-four patients (forty-seven hips) who had undergone metal-on-metal total hip arthroplasty and analyzed the radiographic and clinical measurements after a mean duration of follow-up of 108 months (range, 62.4 to 153.6 months). The mean age of the patients at the time of surgery was twenty-five years (range, fifteen to thirty years). The diameter of the head of the femoral component was 28 mm in all hips except five, in which it was 32 mm. The metal-on-metal bearing was the same in all patients. RESULTS: The mean Merle d'Aubigné score increased from 10.6 (range, 1 to 14) to 17.1 (range, 12 to 18). No wear was found on the latest radiograph, but osteolysis was noted in three femora and two acetabula. Two revisions were performed, one because of impingement secondary to cup malorientation and the other because of acetabular loosening with osteolysis. Kaplan-Meier analysis with revision of either component as the end point revealed a ten-year survival rate of 94.5% (95% confidence interval, 80% to 98.6%). The survival rate of the femoral stem was 100%. CONCLUSIONS: These encouraging intermediate-term results indicate that hip arthroplasty with metal-on-metal bearing components may be a suitable solution for young and active patients with hip osteoarthritis or osteonecrosis of the femoral head.


Assuntos
Artroplastia de Quadril , Prótese de Quadril , Adolescente , Adulto , Feminino , Necrose da Cabeça do Fêmur/cirurgia , Humanos , Masculino , Metais , Osteoartrite do Quadril/cirurgia , Desenho de Prótese , Falha de Prótese , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
19.
J Child Orthop ; 1(2): 135-41, 2007 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-19308486

RESUMO

PURPOSE: Purpose This study reports the results of surgical treatment of thumb duplication in the Clinique d'Orthopédie et de Chirurgie de l'Enfant de l'Hôpital Jeanne de Flandre in Lille (France). Methods Thirty patients (33 thumbs) operated on between 1995 and 2003 are clinically reviewed. Results The mean postoperative follow-up was 3 years and 11 months. According to Wassel's classification, the series included 12 type II duplications, two type III, 14 type IV, two type V, one type VI and two type VII. The surgical approaches consisted of simple resection of the most hypoplastic thumb (16 thumbs), the Bilhaut-Cloquet procedure (ten thumbs) and resection associated with reconstructive surgery (seven thumbs). The Bilhaut-Cloquet procedure was used in three cases for treatment of type IV duplication On the basis of the Tada scoring system, we obtained 24 good results, eight fair results and one poor result. Conclusion Based on our results, we recommend that the Bilhaut-Cloquet procedure be used not only for the treatment of type II duplication when the thumbs are both hypoplastic and symmetric but also for type IV duplication with the same clinical parameters. For the other types of duplications, we consider that resection of the most hypoplastic thumb associated with reconstructive surgery is the best surgical approach. For type VII duplication, ablation of the triphalangeal thumb remains the best option. We do not recommend osteotomy at the first surgery.

20.
Am J Med Genet A ; 124A(4): 417-22, 2004 Feb 01.
Artigo em Inglês | MEDLINE | ID: mdl-14735595

RESUMO

This is a 3-year retrospective study of 107 cases presenting with limb anomalies detected either on prenatal ultrasound scan, or after birth. These limb malformations are developmental anomalies, and can be isolated, syndromic, or associated with multiple malformations. Cases were ascertained through the prenatal diagnosis center, the pediatrics department, and the feto-pathology department. Several criteria were analyzed including sex ratio, prenatal diagnosis, karyotype, termination of pregnancies, clinical or pathological examination, pediatric or surgical and/or genetic assessment, and whether or not a diagnosis was made. Positional deformities and syndactyly were excluded. Limb anomalies were detected prenatally in 45% of the cases, and a diagnosis was made in 78%, including isolated, syndromic, or chromosomal anomalies. Sixty-one per cent of the infants had follow-up, either pediatric, surgical, or genetic. Prenatal multidisciplinary assessment is fundamental to assist with counseling, as is the post-natal follow-up of the infant. The diagnosis, if made, will obviously influence the information that will be given to the parents and the management of the malformation. If the pregnancy is terminated, feto-pathological examination is essential to help make a diagnosis, and guide recurrence risks. We are currently undertaking a prospective study, and we will develop a protocol of investigations in the future, depending on the type of the malformation identified.


Assuntos
Deformidades Congênitas dos Membros/diagnóstico por imagem , Ultrassonografia Pré-Natal , Feminino , Humanos , Recém-Nascido , Deformidades Congênitas dos Membros/diagnóstico , Masculino , Gravidez
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