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1.
Eur J Med Genet ; 68: 104916, 2024 Apr.
Article in English | MEDLINE | ID: mdl-38296035

ABSTRACT

There is no universally accepted definition for rare diseases: in Europe a disease is considered to be rare when affecting fewer than 1 in 2000 people. European Reference Networks (ERNs) have been the concrete response to address the unmet needs of rare disease patients and many pan-European issues in the field, reducing inequities, and significantly increasing accessibility to high-quality healthcare across Europe. ERNs are virtual networks, involving centres and patient representatives with the general scope to facilitate discussion on complex cases requiring highly specialised competences and trained expertise. ERN BOND - the European Reference Network on rare BONe Diseases - is one of these 24 approved networks with the specific ongoing mission to implement measures facilitating multidisciplinary, holistic, continuous, patient-centred, and participative care provision to patients, and supporting them in the full realisation of their fundamental human rights. ERN BOND includes in 2023 a total of 53 centres of expertise from 20 European countries. Its governing structure installed in March 2017 includes decision-making, operative and consultative committees, which comprise experts in the field and patient representatives ensuring patient's voice and perspectives are taken into account. Over the years, ERN BOND has worked hard to achieve its mission and valuably contribute to the advancement of diagnosis, management, treatment, and research in rare diseases. The network activities are mainly related to (i) the provision of care which collectively involves averagely 2800 patients diagnosed per year, (ii) the development of education for and training of the healthcare personnel consisting until now in the realisation of 7 thematic workshops and 19 webinars, (iii) the dissemination and exchange and spread of knowledge via network's website (https://ernbond.eu/), social media channels, and newsletters, (iv) the management of related data through a disease registry currently mapping over 2300 cases and recording over 600 reported cases, and (v) the enhancement of research which now include two clinical trials endorsed by the network. ERN BOND represents therefore an unprecedented move to improve the healthcare management of patients suffering from rare bone diseases through European collaborations. This network, through the support from the European Health Programme, will continue to pursue its efforts to achieve its goals, always maintaining the patients and their families at the centre of healthcare services.


Subject(s)
Bone Diseases , Rare Diseases , Humans , Rare Diseases/diagnosis , Rare Diseases/therapy , Europe
3.
J Pediatr Orthop ; 43(6): 386-391, 2023 Jul 01.
Article in English | MEDLINE | ID: mdl-36941111

ABSTRACT

BACKGROUND: Bone age (BA) has been shown to be superior to chronological age (CA) when predicting remaining growth. However, it is not known whether the calculations are more accurate when BA is assessed by the Greulich and Pyle (GP) or the Sauvegrain (SG) methods. The aim of our study was to identify the method which gives an estimate closest to actual growth in the lower extremities. METHODS: Leg length radiographs, hand radiographs, and elbow radiographs were simultaneously obtained during the adolescent growth spurt (10 to 16 years) in 52 children treated for LLD, with radiographic follow-up of segmental length (femur, tibia, and foot) until skeletal maturity, were randomly selected from a local institutional register. BA, according to GP and SG, were manually rated, and BA based on the GP method was additionally assessed by the automated BoneXpert (BX) method. The remaining growth was calculated based on the White-Menelaus method for both BA methods (GP, SG), the combination of the 2 methods, GP by BX, CA, and the combination of CA and GP by BX. Estimated growth was compared with the actual growth in the distal femur and proximal tibia from the time of BA determination until skeletal maturity. RESULTS: For all included methods, the average calculated remaining growth was higher compared with the actual growth. The mean absolute difference between calculated remaining growth and actual growth in the femur and tibia was lowest using GP by BX [0.66 cm (SD 0.51 cm) and 0.43 cm (SD 0.34 cm)] and highest using CA [1.02 (SD 0.72) and 0.67 (SD 0.46)]. It was a significant association between calculated growth and the difference between actual and calculated growth for the SG method ( P =<0.001). CONCLUSION: During the adolescent growth spurt, the GP method compared with the SG method and CA gives the most accurate estimate of remaining growth around the knee according to our results. CLINICAL RELEVANCE: In calculations of remaining growth around the knee, BA assessment by the GP atlas or BX method should be used as the parameter of biological maturity.


Subject(s)
Age Determination by Skeleton , Lower Extremity , Adolescent , Child , Humans , Age Determination by Skeleton/methods , Knee Joint/diagnostic imaging , Radiography , Tibia/diagnostic imaging
4.
J Child Orthop ; 16(5): 401-408, 2022 Oct.
Article in English | MEDLINE | ID: mdl-36238144

ABSTRACT

Purpose: The purpose of this study was to systematically analyze the presence of secondary angular deformities after percutaneous epiphysiodesis based on long-standing radiographs, and to see if the occurrence and magnitude of angular deformities after percutaneous epiphysiodesis correlated with the amount of remaining growth at the time of surgery. Methods: From a local Health Register consisting of patients investigated using the Moseley Straight-Line Graph, we identified 269 patients who had undergone percutaneous epiphysiodesis from 2002 until 2020. Radiographic analysis included the measurement of mechanical axis and joint orientation angles on long-standing anterior-posterior radiographs. Remaining growth was analyzed based on the Menelaus method. Results: One hundred and forty epiphysiodeses (71 femurs and 69 tibiae) in 88 patients (39 girls and 49 boys) could be included in the study. Mean age at surgery was 13.2 (10-16.8) years, and mean skeletal age at surgery was 13.0 (9.8-15.7) years. A change of the MA (Mechanical axis) ≥10 mm was found in eight patients (9%). Secondary frontal plane deformities after percutaneous epiphysiodesis correlated significantly with the remaining growth at the time of surgery (p = 0.003). Conclusion: We found a high rate of secondary angular deformities after percutaneous epiphysiodesis, and the magnitude of the deformities correlated with the amount of remaining growth at the time of surgery. A modification of the original surgical method for percutaneous epiphysiodesis to also include ablation of central parts of the growth plate might be considered. Patients should be enrolled in a systematic follow-up scheme which allows for the early detection of possible angular deformities. Level of evidence: level III study.

5.
Cureus ; 14(9): e29164, 2022 Sep.
Article in English | MEDLINE | ID: mdl-36258983

ABSTRACT

Compartment syndrome is a rare manifestation of vaso-occlusive crisis, a serious complication of sickle cell disease (SCD), which is an inherited hemoglobinopathy. During a visit to Norway, an otherwise healthy, 20-year-old male from Ghana was admitted to Oslo University Hospital (Day 1) because of increasing pain in the hip and thighs that did not respond adequately to non-opioid painkillers. Despite initial treatment with intravenous fluids and opioids, his pain intensified. Careful clinical inspection supported by an MRI examination revealed focal, high-signal-intensity muscle edema of the anterior compartment of the thigh, almost exclusively limited to the vastus intermedius muscles. There were no MRI findings or blood biochemistry evidence for myonecrosis or rhabdomyolysis, and a diagnosis of deep compartment syndrome appeared to be the most likely explanation for his pain. We decided to continue with a conservative treatment approach, and the patient did not undergo a fasciotomy or blood transfusion therapy. On Day 7 after admission, his condition improved markedly, and he was discharged on Day 11 whereupon he returned to Ghana. This case is a reminder that, although rare, deep compartment syndrome can be a severe manifestation of vaso-occlusive crisis in SCD and should be considered in patients with severe, deep muscular pain in the absence of other explanatory factors.

6.
J Child Orthop ; 16(4): 306-312, 2022 Aug.
Article in English | MEDLINE | ID: mdl-35992522

ABSTRACT

Purpose: The aim was to evaluate which clinical and radiographic variables are independent (true) risk factors for hip subluxation in nonambulatory children below 5 years of age with cerebral palsy. Methods: Patients were recruited from a population-based hip surveillance program. Inclusion criteria were birth during 2002-2006, age below 5 years, and gross motor function classification system levels III-V. In all, 121 children (71 boys) met these criteria. Gross motor function classification system was level III in 29 patients, level IV in 28, and level V in 64. Anteroposterior radiographs at diagnosis and during follow-up were assessed, and only the worst hip of each patient was used for the analyses. The mean age at the initial radiograph was 2.5 years (range: 0.7-4.9 years), and the mean follow-up time was 4.0 years (range: 0.5-11.8 years). Results: At the last follow-up, 67 children had a clinically significant hip displacement, defined as migration percentage ≥40%. Univariable regression analysis defined these risk factors: gross motor function classification system level V, spastic bilateral cerebral palsy, initial migration percentage, yearly rate of migration percentage progression, and initial acetabular index. When these variables were analyzed with multivariable regression in 107 patients with initial migration percentage <50% and follow-up ≥1.0 year, the independent risk factors were initial migration percentage (p = 0.003) and yearly rate of migration percentage progression (p < 0.001). Conclusion: The parameters that need to be assessed in hip surveillance in children below 5 years of age are initial migration percentage and rate of migration percentage progression. Acetabular index and femoral head-shaft angle might be useful later for decision-making regarding choice of treatment. Level of evidence: Level II, development of diagnostic criteria.

7.
Acta Orthop ; 93: 222-228, 2022 01 11.
Article in English | MEDLINE | ID: mdl-35019143

ABSTRACT

Background and purpose - Skeletal maturity is a crucial parameter when calculating remaining growth in children. We compared 3 different methods, 2 manual and 1 automated, in the radiological assessment of bone age with respect to precision and systematic difference. Material and methods - 66 simultaneous examinations of the left hand and left elbow from children treated for leg-length discrepancies were randomly selected for skeletal age assessment. The radiographs were anonymized and assessed twice with at least 3 weeks' interval according to the Greulich and Pyle (GP) and Sauvegrain (SG) methods by 5 radiologists with different levels of experience. The hand radiographs were also assessed for GP bone age by use of the automated BoneXpert (BX) method for comparison. Results - The inter-observer intraclass correlation coefficient (ICC) was 0.96 for the GP and 0.98 for the SG method. The inter- and intra-observer standard error of the measurement (SEm) was 0.41 and 0.32 years for the GP method and 0.27 and 0.21 years for the SG method with a significant difference (p < 0.001) between the methods and between the experienced and the less experienced radiologists for both methods (p = 0.003 and p < 0.001). In 25% of the assessments the discrepancy between the GP and the SG method was > 1 year. There was no systematic difference comparing either manual method with the automatic BX method. Interpretation - With respect to the precision of skeletal age determination, we recommend using the SG method or preferably the automated BX method based on GP assessments in the calculation of remaining growth.


Subject(s)
Age Determination by Skeleton , Leg , Age Determination by Skeleton/methods , Child , Hand/diagnostic imaging , Humans , Radiography , Reproducibility of Results
8.
J Pediatr Orthop ; 41(8): e659-e663, 2021 Sep 01.
Article in English | MEDLINE | ID: mdl-34101699

ABSTRACT

BACKGROUND: The aim of this study was to evaluate whether the femoral head-shaft angle (HSA) is a predictor of hip displacement in children with cerebral palsy (CP). METHODS: The patients were recruited from a population-based hip surveillance program. Inclusion criteria were age under 5 years, bilateral CP, Gross Motor Function Classification System (GMFCS) levels III-V, and migration percentage (MP) of both hips <40% at the primary radiograph. With these criteria, 101 children (61 boys) were included. GMFCS was level III in 26 patients, level IV in 23, and level V in 52. An anteroposterior radiograph of the pelvis was taken at diagnosis and at the last follow-up. Only the worst hip of each patient (the hip with the largest MP) was used for the analyses. RESULTS: The mean age at the primary radiograph was 2.4 years (range, 0.8 to 4.9 y). The mean primary HSA was 171.0 degrees (range, 152 to 190 degrees). The mean follow-up time was 4.3 years (range, 0.9 to 11.8 y). The mean MP at the primary radiograph was 17.5% (range, 0% to 39%) and at the last follow-up 41.9% (range, 0% to 100%). At that point, MP was <40% in 54 hips and ≥40% in 47 hips. There was no significant difference in primary HSA between patients with final MP<40% and those with final MP≥40% (170.8 and 171.3 degrees, respectively; P=0.761). At the last follow-up, the mean HSA was significantly larger in hips with final MP≥40% than in hips with final MP<40% (171.1 vs. 167.4 degrees; P=0.029). CONCLUSIONS: There was a markedly increased valgus position of the proximal femur in nonambulatory children with CP. However, the primary HSA in children below 5 years of age was not a predictor of later hip displacement, defined as MP≥40%. CLINICAL RELEVANCE: Measurement of HSA is not necessary in routine hip surveillance in children below 5 years. LEVEL OF EVIDENCE: Level I-investigating a diagnostic test.


Subject(s)
Cerebral Palsy , Hip Dislocation , Cerebral Palsy/diagnostic imaging , Cerebral Palsy/epidemiology , Child , Child, Preschool , Femur , Femur Head/diagnostic imaging , Hip Dislocation/diagnostic imaging , Hip Dislocation/epidemiology , Hip Dislocation/etiology , Humans , Male , Radiography
9.
Acta Orthop ; 92(5): 615-620, 2021 Oct.
Article in English | MEDLINE | ID: mdl-34082661

ABSTRACT

Background and purpose - In Norway all compensation claims based on healthcare services are handled by a government agency (NPE, Norsk Pasientskade Erstatning). We provide an epidemiological overview of claims within pediatric orthopedics in Norway, and identify the most common reasons for claims and compensations.Patients and methods - All compensation claims handled by NPE from 2012 to 2018 within pediatric orthopedics (age 0 to 17 years) were reviewed. Data were analyzed with regard to patient demographics, diagnoses, type of injury, type of treatment, reasons for granted compensation, and total payouts.Results - 487 compensation claims (259 girls, 228 boys) within orthopedic surgery in patients younger than 18 years at time of treatment were identified. Mean age was 12 years (0-17). 150 out of 487 claims (31%) resulted in compensation, including 79 compensations for inadequate treatment, 58 for inadequate diagnostics, 12 for infections, and 1 based on the exceptional rule. Total payouts were US$8.45 million. The most common primary diagnoses were: upper extremity injuries (26%), lower extremity injuries (24%), congenital malformations and deformities (12%), spine deformities (11%), disorders affecting peripheral joints (9%), chondropathies (6%), and others (12%).Interpretation - Most claims were submitted and granted for mismanagement of fractures in the upper and lower extremity, and mismanagement of congenital malformations and disorders of peripheral joints. Knowledge of the details of malpractice claims should be implemented in educational programs and assist pediatric orthopedic surgeons to develop guidelines in order to improve patient safety and quality of care.


Subject(s)
Compensation and Redress , Malpractice/economics , Musculoskeletal Diseases/economics , Musculoskeletal Diseases/surgery , Musculoskeletal System/injuries , Musculoskeletal System/surgery , Orthopedic Procedures/economics , Adolescent , Child , Child, Preschool , Female , Humans , Infant , Infant, Newborn , Male , Norway , Surveys and Questionnaires
10.
Acta Orthop ; 92(3): 329-334, 2021 06.
Article in English | MEDLINE | ID: mdl-33410356

ABSTRACT

Background and purpose - Literature describing long-term functional outcome and osteoarthritis (OA) in adjacent joints after femoral lengthening is rare. We evaluated physical function and the presence of radiographic OA in adjacent joints in 10 patients ≥ 27 years after femoral lengthening.Patients and methods - We conducted a cross-sectional study of 10 patients treated by unilateral femoral lengthening. Follow-up was between 27 and 34 years. Physical function was evaluated by the 30-second sit-to-stand (30sSTS) and a stair test and was compared with reference values. 4 single-legged hop tests were used to assess difference in physical function between the lengthened and contralateral limb. Radiographic OA was evaluated by joint space width (JSW) and Kellgren and Lawrence (KL) classification.Results - The patients scored worse compared with reference values on the 30sSTS and stair test, and worse on the lengthened limb on the single- and triple-hop test. Radiographic OA was found in the hip or knee in the lengthened limb in 3 of 10 patients based on JSW and 4 of 10 based on KL. No radiographic OA was found in unlengthened limbs.Interpretation - Our results showed impaired physical function both in general and of the lengthened limb. Additionally, we found a possible association between femoral lengthening and radiographic OA in adjacent joints in the long term. However, the sample size of the current study is small.


Subject(s)
Bone Lengthening , Femur/surgery , Leg Length Inequality/surgery , Motor Activity/physiology , Osteoarthritis, Hip/epidemiology , Osteoarthritis, Knee/epidemiology , Adolescent , Adult , Cross-Sectional Studies , Female , Follow-Up Studies , Humans , Leg Length Inequality/complications , Leg Length Inequality/physiopathology , Male , Middle Aged , Osteoarthritis, Hip/diagnostic imaging , Osteoarthritis, Knee/diagnostic imaging , Radiography , Time Factors , Young Adult
12.
Bone Jt Open ; 1(4): 55-63, 2020 Apr.
Article in English | MEDLINE | ID: mdl-33215108

ABSTRACT

AIMS: When the present study was initiated, we changed the treatment for late-detected developmental dislocation of the hip (DDH) from several weeks of skin traction to markedly shorter traction time. The aim of this prospective study was to evaluate this change, with special emphasis on the rate of stable closed reduction according to patient age, the development of the acetabulum, and the outcome at skeletal maturity. METHODS: From 1996 to 2005, 49 children (52 hips) were treated for late-detected DDH. Their mean age was 13.3 months (3 to 33) at reduction. Prereduction skin traction was used for a mean of 11 days (0 to 27). Gentle closed reduction under general anaesthesia was attempted in all the hips. Concurrent pelvic osteotomy was not performed. The hips were evaluated at one, three and five years after reduction, at age eight to ten years, and at skeletal maturity. Mean age at the last follow-up was 15.7 years (13 to 21). RESULTS: Stable closed reduction was obtained in 36 hips (69%). Open reduction was more often necessary in patients ≥ 18 months of age at reduction (50%) compared with those under 18 months (24%). Residual hip dysplasia/subluxation occurred in 12 hips and was significantly associated with avascular necrosis (AVN) and with high acetabular index and low femoral head coverage the first years after reduction. Further surgery, mostly pelvic and femoral osteotomies to correct subluxation, was performed in eight hips (15%). The radiological outcome at skeletal maturity was satisfactory (Severin grades 1 or 2) in 43 hips (83%). CONCLUSIONS: Gentle closed reduction can be attempted in children up to three years of age, but is likely to be less successful in children aged over 18 months. There is a marked trend to spontaneous improvement of the acetabulum after reduction, even in patients aged over 18 months and therefore simultaneous pelvic osteotomy is not always necessary.

13.
J Child Orthop ; 14(4): 266-272, 2020 Aug 01.
Article in English | MEDLINE | ID: mdl-32874358

ABSTRACT

PURPOSE: The aims of this study were to evaluate the reliability of three classifications of severity of dislocation in late-detected developmental dysplasia of the hip (DDH), especially to assess whether they are predictive of long-term outcomes. METHODS: Two groups of patients were analyzed. Group 1 (57 patients, 69 hips) underwent closed reduction between 1958 and 1962. Mean age at reduction was 20.3 months (4 to 65). Group 2 (50 patients, 54 hips) treated between 1996 and 2005, was used for analysis of the association between severity of dislocation and treatment (open or closed reduction). The primary radiographs were graded according to the Tönnis classification, the classification of the International Hip Dysplasia Institute and a new method based on the position of the most lateral point of the proximal femoral metaphysis (lateral metaphysis height classification, LMH). The outcome at a mean age of 51.2 years (55 to 60) was graded according to the occurrence of osteoarthritis (OA) and total hip arthroplasty (THA). RESULTS: There were significant associations between the classifications, and the intra- and interobserver agreements were high. More severe grades of DDH were significantly associated with age ≥ 18 months and with open reduction. None of the classifications were significantly associated with long-term OA. The LMH method was significantly associated with the need for THA, whereas the other classifications were not. CONCLUSION: All the classifications were reliable in grading severity of DDH. The LMH method seems preferable in clinical practice because the main landmarks were easy to define and because it had long-term prognostic value. LEVEL OF EVIDENCE: III.

14.
Dtsch Arztebl Int ; 117(24): 405-411, 2020 06 12.
Article in English | MEDLINE | ID: mdl-32865491

ABSTRACT

BACKGROUND: Many people have leg-length discrepancies of greater or lesser severity. No evidence-based studies on the need for treatment are currently available. METHODS: This review is based on publications retrieved by a selective search in the PubMed database, as well as on published recommendations from Germany and abroad and on the authors' own clinical experience. RESULTS: If the two legs are of different lengths, this is generally because one leg is too short. It is debated whether leg-length discrepancy causes pain or long-term musculoskeletal disturbances. A direct connection to back pain is questionable, but a mildly elevated incidence of knee arthritis seems likely. The evidence base on the indications for treatment of leg-length discrepancy is poor; only informal consensus recommendations are available. There are a wide variety of conservative and surgical treatment options. The final extent of a leg-length discrepancy first noted during the growing years can be estimated with predictive algorithms to within 2 cm. The treatments that can be considered include a shoe insert, a high shoe, or an orthosis, surgically induced slowing of growth by blockade of the epiphyseal plates around the knee joint, or leg lengthening with osteotomy and subsequent distraction of the bone callus with fully implanted or external apparatus. Changes in leg length exert marked mechanical stress on the soft tissues. If the predicted leg-length discrepancy exceeds 5 cm, initial leg-lengthening treatment can already be considered during the patient's growing years. CONCLUSION: It must be discussed with each patient individually whether the treatment should be conservative or surgical. The extent of the discrepancy is not the sole determining factor for the mode of treatment. The decision to treat is always elective.


Subject(s)
Leg Length Inequality , Leg , Germany , Humans , Leg/anatomy & histology , Prostheses and Implants , Treatment Outcome
15.
Acta Orthop ; 90(1): 81-87, 2019 02.
Article in English | MEDLINE | ID: mdl-30371122

ABSTRACT

Background and purpose - Limb lengthening with an intramedullary motorized nail is a relatively new method. We investigated if lengthening nails are reliable constructs for limb lengthening and deformity correction in the femur and the tibia. Patients and methods - 50 lengthenings (34 Precice and 16 Fitbone devices) in 47 patients (mean age 23 years [11-61]) with ≥12 months follow-up are included in this study. 30 lengthenings were done due to congenital and 20 because of posttraumatic deformity (21 antegrade femora, 23 retrograde femora, 6 tibiae). Initial deformities included a mean shortening of 42 mm (25-90). In 15 patients, simultaneous axial correction was done using the retrograde nailing technique. Results - The planned amount of lengthening was achieved in all but 2 patients. 5 patients who underwent simultaneous axial correction showed minor residual deformity; unintentionally induced minor deformities were found in the frontal and sagittal plane. The consolidation index was 1.2 months/cm (0.6-2.5) in the femur and 2.5 months/cm (1.6-4.0) in the tibia. 2 femoral fractures occurred in retrograde femoral lengthenings after consolidation due to substantial trauma. There were 8 complications, all of which were correctable by surgery, with no permanent sequelae. Interpretation - Controlled acute axial correction of angular deformities and limb lengthening can be achieved by a motorized intramedullary nail. A thorough preoperative planning and intraoperative control of alignment are required to avoid residual and unintentionally induced deformity. In the femur relatively fast consolidation could be observed, whereas healing was slower in the tibia.


Subject(s)
Bone Lengthening , Femur/surgery , Foot Deformities, Acquired , Foot Deformities, Congenital/surgery , Fracture Fixation, Intramedullary , Tibia/surgery , Adolescent , Adult , Bone Lengthening/adverse effects , Bone Lengthening/instrumentation , Bone Lengthening/methods , Bone Nails , Female , Femoral Fractures/complications , Femur/pathology , Foot Deformities, Acquired/etiology , Foot Deformities, Acquired/surgery , Fracture Fixation, Intramedullary/adverse effects , Fracture Fixation, Intramedullary/methods , Humans , Intraoperative Care/methods , Male , Middle Aged , Norway , Outcome and Process Assessment, Health Care , Prosthesis Design , Tibia/pathology
16.
Acta Orthop ; 88(3): 334-340, 2017 Jun.
Article in English | MEDLINE | ID: mdl-28464755

ABSTRACT

Background and purpose - Hexapod ring fixators such as the Taylor Spatial Frame (TSF) have shown good outcomes. However, there have only been a few studies comparing the use of TSF with various etiologies of the deformity. We compared the use of TSF in congenital and acquired deformities in children. Patients and methods - We reviewed 213 lower extremity reconstructive procedures with the TSF in 192 patients who were operated between October 2000 and October 2015. 128 procedures (67 proximal tibiae, 51 distal femora, and 10 distal tibiae) in 117 children (median age 14 (4-18) years; 59 girls) fulfilled the inclusion criteria. 89 procedures were done in children with congenital deformities (group C) and 39 were done in children with acquired deformities (group A). Outcome parameters were lengthening and alignment achieved, lengthening index, complications, and analysis of residual deformity in a subgroup of patients. Results - Mean lengthening achieved was 3.9 (1.0-7.0) cm in group C and 3.7 (1.0-8.0) cm in group A (p = 0.5). Deformity parameters were corrected to satisfaction in all but 3 patients, who needed further surgery for complete deformity correction. However, minor residual deformity was common in one-third of the patients. The mean lengthening index was 2.2 (0.8-10) months/cm in group C and 2.0 (0.8-6) months/cm in group A (p = 0.7). Isolated analysis of all tibial and femoral lengthenings showed similar lengthening indices between groups. Complication rates and the need for secondary surgery were much greater in the group with congenital deformities. Interpretation - The TSF is an excellent tool for the correction of complex deformities in children. There were similar lengthening indices in the 2 groups. However, congenital deformities showed a high rate of complications, and should therefore be addressed with care.


Subject(s)
Bone Lengthening/methods , External Fixators , Lower Extremity Deformities, Congenital/surgery , Lower Extremity/surgery , Adolescent , Bone Lengthening/adverse effects , Bone Transplantation/methods , Child , Child, Preschool , Female , Femur/abnormalities , Femur/diagnostic imaging , Femur/surgery , Humans , Knee Joint/physiopathology , Leg Length Inequality/diagnostic imaging , Leg Length Inequality/surgery , Lower Extremity/diagnostic imaging , Lower Extremity Deformities, Congenital/diagnostic imaging , Male , Osteotomy/methods , Radiography , Range of Motion, Articular , Retrospective Studies , Tibia/abnormalities , Tibia/diagnostic imaging , Tibia/surgery , Treatment Outcome
17.
J Child Orthop ; 10(6): 487-492, 2016 Dec.
Article in English | MEDLINE | ID: mdl-27838825

ABSTRACT

Distraction osteogenesis biologically resembles fracture healing with distinctive characteristics notably in the distraction phase of osteogenesis. In the latency phase of bone lengthening, like in the inflammatory phase of fracture repair, interleukines are released and act with growth factors released from platelets in the local haematoma, leading to attraction, proliferation and differentiation of mesenchymal stem cells into osteoblasts and other differentiated mesenchymal cells. These in turn produce matrix, collagen fibers and growth factors. A callus containing cells, collagen fibers, osteoid and cartilage matrix is formed. Provided stable fixation, distraction will trigger intramembranous bone formation. As distraction proceeds, the distraction gap develops five distinctive zones with unmineralized bone in the middle, remodelling bone peripherally, and mineralizing bone in between. During consolidation, the high concentration of anabolic growth factors in the regenerate diminishes with time as remodelling takes over to form mature cortical and cancellous bone. Systemic disease, congenital bone deficiencies, medications and substance abuse can influence the quality and quantity of regenerate bone, usually in a negative way. The regenerate bone can be manipulated when needed by using injection of mesenchymal stem cells and platelets, growth factors (BMP-2 and -7), and systemic medications (bisphosphonates and parathyroid hormone). Growth factors and systemic anabolic and antiresorptive drugs are prescribed on special indications, while distraction osteogenesis is not an authorized indication. To some extent, however, these compounds can be used off-label. Use in children presents special problems since growth factors and specific anabolic medications may involve a risk of inducing cancer.

18.
Clin Orthop Relat Res ; 474(5): 1189-98, 2016 May.
Article in English | MEDLINE | ID: mdl-26290341

ABSTRACT

BACKGROUND: Despite considerable changes in the treatment of of late-detected congenital or developmental hip dislocation (DDH) during the last 50 years, it is unclear whether and to what degree these changes have led to better long-term outcome for the patients. QUESTIONS/PURPOSES: The aims of this study were to see whether decreasing use of skin traction and instead a more aggressive approach to open reduction resulted in (1) reduced use of secondary procedures; (2) improved radiographic appearance of the hips at long-term followup; and (3) changes in the proportion of patients developing avascular necrosis. METHODS: Two groups of patients were compared retrospectively. Inclusion criteria were patient age older than 3 months and younger than 5 years at the initiation of treatment, no associated anomaly, no previous treatment in other hospitals, and available radiographs from the time of diagnosis to skeletal maturity. Group A consisted of 56 patients (51 girls [91%]; 74 hips) primarily treated during the period 1958 to 1962. Group B comprised 38 patients (36 girls [95%]; 40 hips) treated during the period 1996 to 2002. The mean age at the time of hip reduction was 20 months (SD 9.6) in Group A and 17 months (SD 11.9) in Group B. The mean time in skin traction had decreased from 35 days (SD 12.5) to 11 days (SD 5.7) over the years (p < 0.001). Open reduction was performed in six of 74 hips (8%) in Group A and 15 of 40 hips (37%) in Group B (p < 0.001). The immobilization time in the hip spica had decreased from 9 to 6 months (p < 0.001). The indication for secondary procedures to correct residual dysplasia was center-edge angle < 20° and was similar in both groups. A modified version of the radiographic classification of Severin was used to compare the results. RESULTS: Secondary procedures to correct residual dysplasia were performed in 28 of 74 hips (38%) in Group A and seven of 40 hips (18%) in Group B (odds ratio [OR], 0.35; 95% confidence interval [CI], 0.14-0.89; p = 0.025) At skeletal maturity, the proportion of patients with satisfactory radiographic results (Severin Grades I/II) was larger in Group B (33 of 40 hips [82%]) than in Group A (46 of 74 hips [62%]; OR, 0.35; CI, 0.14-0.89; p = 0.025). Femoral head coverage, assessed as the center-edge angle, was greater in Group B than in Group A (mean 26° versus 22°; CI, 0.8-7.9; p = 0.016). There was no difference in the proportion of avascular necrosis of the femoral head (seven of 74 hips [9%] in Group A and five of 40 [13%] in Group B; OR, 1.4; CI, 0.4-4.6; p = 0.614). CONCLUSIONS: The move away from prolonged use of skin traction and toward more frequent open reduction for children with a late diagnosis of DDH appears to result in fewer secondary procedures and a better radiographic appearance of the hip at skeletal maturity. Based on the present results, we cannot conclude whether preliminary traction is needed; this question should be evaluated in future long-term studies with a prospective, randomized design. LEVEL OF EVIDENCE: Level III, therapeutic study.


Subject(s)
Arthrography , Delayed Diagnosis , Hip Dislocation, Congenital/surgery , Hip Joint/surgery , Orthopedic Procedures/methods , Age Factors , Chi-Square Distribution , Child Development , Child, Preschool , Female , Hip Dislocation, Congenital/diagnostic imaging , Hip Dislocation, Congenital/physiopathology , Hip Joint/abnormalities , Hip Joint/diagnostic imaging , Hip Joint/physiopathology , Humans , Infant , Male , Odds Ratio , Orthopedic Procedures/adverse effects , Predictive Value of Tests , Reoperation , Retrospective Studies , Risk Factors , Time Factors , Traction , Treatment Outcome
20.
Waste Manag Res ; 33(2): 165-74, 2015 Feb.
Article in English | MEDLINE | ID: mdl-25649405

ABSTRACT

Hexabromocyclododecane containing polystyrene foam obtained from the building and construction market has been co-incinerated in a full-scale waste incineration plant. The co-feeding of 1 and 2 wt% of polystyrene foam had no influence on the operation of the plant. The bromine content increased the raw gas hydrogen bromide concentration slightly. The air emission, including that of dioxins and bromine, was not altered and so was the quality of the solid residues. The hexabromocyclododecane concentrations in the solid residues were almost identical, regardless of whether or not and how much polystyrene foam was added. The obtained destruction efficiency was >99.999% independent of the amount of added polystyrene foam. This finding indicates a virtually total destruction of hexabromocyclododecane.


Subject(s)
Flame Retardants , Hydrocarbons, Brominated/chemistry , Incineration , Polystyrenes/analysis , Air Pollutants , Environmental Monitoring , Hydrocarbons, Brominated/analysis , Hydrocarbons, Chlorinated/analysis , Solid Waste/analysis
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