Pelvic Fixation for Non-Ambulatory Patients with Neuromuscular Scoliosis Treated with Magnetically Controlled Growing Rods-A 4-Year Comparison of Two Different Fixation Methods.

Background/Objectives: This study aims to analyze the efficacy and safety of the two pelvic fixation systems, S-hooks (SH) and lumbar-sacral-alar-iliac (SAI) screws, when used in association with magnetically controlled growing rods (MCGRs) in non-ambulatory children with severe neuromuscular scoliosis (NMS). Methods: Forty-one patients were retrospectively examined and subdivided corresponding to ilium hook fixation or SAI screws. The major curve correction (%) and pelvic obliquity (PO) correction (%) were assessed utilizing scoliosis plain film radiographs over time. Complications and unplanned return to the operating room (UPROR) were recorded. Patient-specific pre- and postoperative values were included in a backward stepwise regression model to assess UPROR. Results: Mean age at index intervention was 9.4 years. Preoperative main curve was 81° and PO was 22°. 21 and 20 patients were categorized into the SH and SAI subgroups, respectively. Initial curve correction was significantly better in the SAI subgroup (63 vs. 42% in the SH, p = 0.045), while PO correction was equally good. Curve and PO correction were maintained throughout the follow-up period of 55 months. UPROR rate was 38% in the SH subgroup, and 5% in the SAI subgroup (p = 0.010). Regression analysis identified postoperative curve correction as predictive value for UPROR (p = 0.006). Conclusions: SAI screw fixation has a low UPROR rate and achieves superior curve correction. S-hooks are a viable option to correct PO and NMS in children with high operative risk to reduce operative time, but revision surgery is not uncommon.

Localization and Likelihood of Chondral and Osteochondral Lesions After Patellar Dislocation in Surgically Treated Children and Adolescents.

Background: Patellar dislocation is a common injury in children and adolescents. There is a lack of information about concomitant osteochondral lesions in these patients. Hypothesis: We hypothesized that the likelihood of chondral or osteochondral lesions would increase with each prior patellar dislocation and that the appearance of lesions in surgically treated children and adolescents would be influenced by age, sex, injury mechanism, and body mass index (BMI). Study Design: Case-control study; Level of evidence, 3. Methods: Inclusion criteria were (1) age <18 years, (2) surgical treatment with diagnostic arthroscopy of the knee including description of chondral and osteochondral lesions, and (3) maximum time period between the last patellar dislocation and presentation in our department of 6 weeks and maximum of 4 weeks between presentation and surgery. A total of 153 children (173 knees) with a mean age of 13.0 ± 2.1 years were included. All surgical reports and intraoperative arthroscopic imaging were analyzed retrospectively for the location and severity of lesions. The likelihood of lesions was calculated with multiple stepwise regression models regarding injury mechanism, number of dislocations, sex, age, physeal closure, and BMI. Results: The regression model to predict femoral lesions was statistically significant (χ2[5] = 26.55; P < .001) and identified male sex, BMI ≥25, traumatic injury mechanism, and physeal closure as independent factors associated with the appearance of femoral lesions. The second regression model predicting the appearance of patellar lesions was also statistically significant (χ2[4] = 26.07; P < .001) and identified the traumatic injury mechanism as a single independent predictor for patellar lesions. Conclusion: BMI ≥25, male sex, and physeal closure were factors significantly associated with femoral chondral and osteochondral lesions in our cohort. In case of traumatic lateral patellar dislocation (LPD), the likelihood of patellar and femoral chondral and osteochondral lesions is significantly higher than in nontraumatic LPD. Early magnetic resonance imaging and surgical treatment should be considered in obese patients, male patients, and patients with physeal closure and after traumatic LPD.

Vertebral column growth in children with early onset scoliosis treated with magnetically controlled growing rods - Effects of distraction on vertebral and disc morphology.

OBJECTIVES: To report changes in vertebral and disc morphology following treatment of early onset scoliosis (EOS) with magnetically controlled growing rods (MCGR). METHODS: 30 Patients, 21 girls and 9 boys, treated with MCGR for EOS were compared to a matched control group of 19 patients (12 girls, 7 boys) treated with bracing or observation. Age at surgery in the MCGR group was 8.75 (range: 4.6-11) years compared to 7.75 (range: 3.5-10.3) years in the control group at the time of onset of treatment. Mean follow-up was 45 (range: 24-65) months in the MCGR group vs 42 (range: 24-65) months in the control group. Calibrated radiographs were used to digitally measure disc height, vertebral body height, depth and width after surgery and at latest follow-up. Height, width and depth of lumbar and thoracic vertebrae and discs under distraction were compared to the control group and to vertebrae below instrumentation. T1-T12, T1-S1 length and Cobb angles were also measured. RESULTS: (1) There is a significant increase of lumbar vertebral height under distraction. (2) Lumbar disc height as well as lumbar vertebral width within distraction is significantly reduced. (3) Depth of lumbar vertebrae is not significantly affected by distraction. (4) Morphology of thoracic vertebrae and adjacent discs is not significantly changed with distraction. (5) T1-T12 distance did not show any significant changes between surgical and control group, while T1-S1 growth was significantly reduced compared to the control group. CONCLUSIONS: Significant changes of morphology of lumbar vertebra and discs are observed under distraction with MCGR compared to segments below instrumentation and the control group. LEVEL OF EVIDENCE: Level III - retrospective comparative series.

Hip pathologies in mucopolysaccharidosis type III.

BACKGROUND: Mucopolysaccharidosis type III (MPS III) comprises a group of rare lysosomal storage diseases. Although musculoskeletal symptoms are less pronounced than in other MPS subtypes, pathologies of hip and spine have been reported in MPS III patients. The purpose of this study was to describe hip pathologies and influencing parameters in MPS III patients. METHODS: A retrospective chart review was performed for 101 MPS III patients. Thirty-two patients met the inclusion criteria of enzymatically or genetically confirmed diagnosis and anteroposterior radiograph of the hips. Modified Ficat classification, Wiberg's center-edge angle, and Reimer's migration percentage were measured. RESULTS: The mean age at data assessment was 11.0 years (SD 5.7). Osteonecrosis of the femoral head was observed in 17/32 patients. No statistically significant association was found between these changes and age, sex, or MPS III subtype. Patients with a severe phenotype showed significantly higher rates of osteonecrosis (14/17) than patients with an intermediate phenotype. Hip dysplasia was present in 9/32 patients and was significantly associated with osteonecrosis of the femoral head (p = 0.04). CONCLUSIONS: The present study demonstrates a high rate of hip pathologies in MPS III patients. Hip dysplasia and severe phenotype were significantly correlated with osteonecrosis of the femoral head. Therefore, radiographs of the hips are highly recommended in baseline and follow-up assessments of MPS III patients. TRIAL REGISTRATION: Retrospectively registered.

Electromagnetic navigation reduces radiation exposure for retrograde drilling in osteochondrosis dissecans of the talus.

BACKGROUND: Retrograde drilling in osteochondrosis dissecans (OCD) is a widely used surgical intervention. A radiation-free electromagnetic navigation system (ENS)-based method was compared with the standard freehand fluoroscopic (SFF) method regarding clinical applicability. METHODS: We performed a clinical cohort study at a department of Orthopaedics in a Level 1 children's hospital with 40 patients (20 SFF and 20 ENS). Retrograde drilling of the talar dome was used in patients with unstable medial OCD (MRI stage 2 according to Hepple's revised classification; stage 2 according to the International Cartilage Repair Society). The outcome measurements were: (a) Intraoperative fluoroscopy exposure and length of surgery and (b) Postoperative serial follow-up MRIs every 6 months. RESULTS: 22 female and 18 male patients aged 13.8 ± 1.6 years (range: 11-17 years) were included. Using the ENS technique, length of surgery was significantly reduced to 20.2 ± 6.4 min compared to 36.1 ± 11.8 min (p < 0.01) for the SFF technique. The average x-ray radiation time for the SFF technique was 23.5 ± 13.5 sec and 1.9 ± 1.7 sec for the ENS technique (p < 0.01). Radiation exposure was significantly reduced from 44.6 ± 19.7 mSv (SFF technique) to 5.6 ± 2.8 mSv (ENS technique) (p < 0.01). Intraoperative perforation of cartilage occurred once in the SFF group. Correct placement of the drilling channel was verified in all patients on follow-up MRI after six months and a timely healing was seen after two years. CONCLUSIONS: The ENS method provides for a significant reduction in length of surgery and radiation exposure. ENS was without intraoperative cartilage perforation. The clinical and radiological follow-up parameters are comparable for SFF- and ENS-guided retrograde drilling. TRIAL REGISTRATION: WF - 085/20, 05/2020 "retrospectively registered" https://www.aerztekammer-hamburg.org/ethik_kommission.html .

Validity of Ultrasound Compared with Magnetic Resonance Imaging in Evaluation of Osteochondritis Dissecans of the Distal Femur in Children.

OBJECTIVE: Magnetic resonance imaging (MRI) is currently the gold standard to diagnose and monitor osteochondritis dissecans (OCD) of the knee. The purpose of this study was to evaluate for the first time if ultrasound imaging can be used to visualize osteochondritis dissecans of the distal femur. DESIGN: From May 2008 to December 2013, 44 children (26 boys and 18 girls) presenting with OCD of the knee in our department were examined and evaluated by ultrasound imaging. Mean age at diagnosis was 11.8 ± 2.2 years. Two independent experienced orthopedic surgeons analyzed the localization, stage, and the size of the OCD via ultrasound and compared the results with the MRI findings. RESULTS: Ultrasonic examination has limitations in assessing the OCD stage I and therefore is not suitable for evaluating this stage of the disease. In stages II to IV, a good correlation to MRI regarding defect localization and size can be found, when the defect is localized in a region that is accessible to ultrasonic examination. CONCLUSION: Ultrasonic scan is an appropriate tool for the screening and monitoring of OCDs stages II to IV. It provides an inexpensive and readily available alternative to MRI. In addition, the healing process of higher grade defects as well as the screening of the opposite side can also be performed by ultrasound. Detection of defects being localized close to the intercondylar notch or far posterior on the lateral condyle are limitations for the use of ultrasound.

Fixation of Unstable Osteochondritis Dissecans Lesions and Displaced Osteochondral Fragments Using New Biodegradable Magnesium Pins in Adolescents.

OBJECTIVE: Fixation of unstable osteochondritis dissecans (OCD) lesions and displaced osteochondral fragments are frequently performed procedures in pediatric orthopedic surgery. Since 2018, CE-certified MAGNEZIX pins are used in our institution in these cases. The aim of this study was (1) to analyze safety, efficiency, and limitations of magnesium-pin-based fixation of unstable OCD lesions and displaced osteochondral fragments and (2) to report clinical and radiological outcomes at short-term follow-up (FU). DESIGN: In this prospective cohort study, 19 patients (10 girls and 9 boys) were included. Inclusion criteria were (1) magnetic resonance imaging-confirmed unstable OCD lesion or displaced osteochondral fragment, (2) fixation with magnesium-based pins, and (3) minimum FU of 6 months. X-rays were taken 6 weeks and 6 months after operation and magnetic resonance imaging scans every 4 to 6 months to assess the healing progress. RESULTS: In total 67 pins were used, with a mean of 3.6 ± 1.4 per patient. Average age at surgery was 13.7 years (11-17 years). Mean time of operation was 56 ± 31 minutes, including arthroscopy, fixation, and patellar realignment (n = 6). No intraoperative complications occurred. Average FU was 11.3 ± 4.2 months (6-20 months). No redislocation or new dislocation occurred. Until now a complete radiographic healing occurred in 12 cases. Due to an implant failure in one case 11 weeks after the index surgery a revision became necessary. CONCLUSIONS: In short-term FU of 11 ± 4 months MAGNEZIX pins provide high stability after fixation of unstable OCDs and displaced osteochondral fragments leading to uncomplicated and timely healing.

The Use of Bovine Xenogeneic Bone Graft for Dega Pelvic Osteotomy in Children with Hip Dysplasia: A Retrospective Study of 147 Treated Hips.

BACKGROUNDS: Dega pelvic osteotomy is commonly used to correct acetabular dysplasia in children with open triradiate cartilage. The use of bovine xenogeneic bone graft (Tutobone®) for Dega osteotomy has not been reported so far. This study aimed to determine the clinical and radiological outcome in a large series of children with hip dysplasia who were treated by Dega osteotomy using a bovine xenogeneic block for stabilisation. METHODS: A retrospective, single-centre study was conducted including 101 patients (147 hips) with different underlying diseases. The acetabular angle of Hilgenreiner (AA) and the lateral center-edge angle (LCA) were analysed to quantify the correction of acetabular indices. Graft incorporation was assessed using the Goldberg scoring system. RESULTS: the mean preoperative AA improved from 28.1 (SD: 6.7) to 14.7 (SD: 5.1) after surgery (p < 0.001). The mean preoperative LCA improved from 9.9 (SD: 6.7) to 21.8 (SD: 6.8) postoperatively (p < 0.001). Both indices remained stable at the one-year follow-up examination. Graft incorporation was excellent with a mean Goldberg score of 6.6. Heterotopic ossification occurred in one hip without clinical relevance. Graft-related complications were not noted. CONCLUSIONS: Dega osteotomy using Tutobone® is safe and effective in the treatment of acetabular dysplasia in children independent of the underlying disease.

Correction of Recurrent Equinus Deformity in Surgically Treated Clubfeet by Anterior Distal Tibial Hemiepiphysiodesis.

BACKGROUND: Residual or recurrent equinus deformity is a common problem in surgically treated clubfeet. This deformity may occur due to soft tissue-related reasons or due to bony deformity. An increased anterior distal tibial angle (ADTA) was previously found in 48% of the operated clubfeet. This study aimed to determine the efficacy and safety of anterior distal tibial hemiepiphysiodesis (ADTE) in the treatment of recurrent equinus deformity in patients with an increased ADTA. METHODS: Eighteen children (23 feet) treated by ADTE to correct recurrent equinus deformity in surgically treated clubfeet were included in this retrospective, single-center study. ADTE using 8 plates was performed in children with an increased ADTA (>82 degrees) and inability to dorsiflex the ankle (≤0 degree of dorsiflexion). The mean patient age was 11.3 years (range: 10.2 to 12.9 y). All patients had completed treatment with implant removal after an average of 20.3 months (range: 9 to 37 mo). RESULTS: The mean preoperative ankle dorsiflexion significantly improved from -3.3 degrees (range: -20 to -0 degrees) to 6.1 degrees (range: -15 to 10 degrees) at the time of 8-plate removal (correction rate: 9.4 degrees; P<0.0001). The ADTA was a mean of 87.5 degrees (range: 83 to 110 degrees) before surgery and significantly improved to 75.8 degrees (range: 63 to 106 degrees) at the time of implant removal (correction rate: 11.7 degrees; P<0.0001). Average follow-up was 43.9 months (range: 10 to 76 mo). Follow-up examinations were continued in 11 patients (13 feet) after implant removal. Changes of ankle dorsiflexion (mean: -2.5 degrees) and ADTA (mean: 3.6 degrees) occurred in this group. CONCLUSIONS: ADTE was safe and effective in the treatment of recurrent equinus deformity in surgically treated clubfeet with increased ADTA. Deterioration of ankle dorsiflexion and ADTA occurred after implant removal in some cases. The results of this study have to be analyzed with caution due to the limited number of included patients and its retrospective nature. LEVEL OF EVIDENCE: Level IV-retrospective case series.

Vertical Expandable Rib-based Distraction Device for Correction of Congenital Scoliosis in Children of 3 Years of Age or Younger: A Preliminary Report.

BACKGROUND: This study was designed to evaluate the treatment outcome of very young children with congenital scoliosis aged 3 years or under after surgery with a vertical expandable prosthetic titanium rib (VEPTR)-based distraction device. METHODS: A retrospective study of 13 children undergoing implantation of a vertical expandable rib-based distraction device. From September 2007 to June 2018, 13 children (7 male and 6 female patients) with congenital scoliosis were followed after treatment with a VEPTR. The outcome parameters were complications, thoracic height, kyphosis, lordosis, and coronal major scoliosis curve. In addition, the American Society of Anaesthesiologists (ASA) score, assisted ventilation rating, and hemoglobin and body mass index were analyzed. Data were examined separately by 2 investigators. RESULTS: The mean age at initial surgical treatment was 24.4±10.6 months, follow-up was 91.5±23.1 months. The mean number of surgical procedures per patient was 14±3.9 (total 182 operations). Apart from planned operations every 6 months, 5 unplanned operations (2.7%) were performed. The major scoliosis curve improved significantly from 55.2±21.9 degrees to 40.5±18.7 degrees, thoracic spine height T1-T12 significantly from 111±12 mm to 137±23 mm, T1-S1 height significantly from 211±13 mm to 252±36 mm and space available for the lung from 80.9%±11.3% to 84.4%±8.8% (preoperatively and at 5-y follow-up, respectively). Kyphosis increased significantly from 30±21.4 degrees to 42.6±23.5 degrees and lordosis from 32.4±20.9 degrees to 37.6±10.4 degrees. Hemoglobin levels were virtually unchanged. Weight increased from 10.6±2.8 kg to 20.2±5.0 kg at final follow-up, but body mass index decreased from 16.6±3.7 kg/m² to 15.7±2.9 kg/m². CONCLUSIONS: The expandable rib-based distraction device is an effective method to treat severe congenital scoliosis in very young children. It can control the deformity and promotes spinal and thoracic growth. LEVEL OF EVIDENCE: Level IV.

Hip Morphology in Mucolipidosis Type II.

Mucolipidosis type II (MLII) is a rare lysosomal storage disorder caused by defective trafficking of lysosomal enzymes. Severe skeletal manifestations are a hallmark of the disease including hip dysplasia. This study aims to describe hip morphology and the natural course of hip pathologies in MLII by systematic evaluation of plain radiographs, ultrasounds and magnetic resonance imaging (MRI). An international two-centered study was performed by retrospective chart review. All MLII patients with at least one pelvic radiograph were included. A total of 16 patients were followed over a mean of 3.5 years (range 0.2-10.7 years). Typical age-dependent radiographic signs identified were femoral cloaking (7/16), rickets/hyperparathyroidism-like changes (6/16) and constrictions of the supra-acetabular part of the os ilium (16/16) and the femoral neck (7/16). The course of acetabular and migration indexes (AI, MI) significantly increased in female patients. However, in the overall group, there was no relevant progression of acetabular dysplasia with a mean AI of 23.0 (range 5°-41°) and 23.7° (range 5°-40°) at the first and last assessments, respectively. Better knowledge on hip morphology in MLII could lead to earlier diagnosis, improved clinical management and enables assessment of effects of upcoming therapies on the skeletal system.

Pedicle screw instrumentation with or without pelvic fixation in neuromuscular scoliosis: Outcome and complications in a series of 37 patients with a minimum 2-year follow-up.

OBJECTIVE: Neuromuscular scoliosis (NMS) is often associated with rapid progressive spinal deformities. Indications, when to extend the instrumentation to the pelvis for pelvic obliquity are not generally accepted at this time. This study reports on the indications, surgical technique and results using pedicle screw instrumentation exclusively with or without pelvic fixation for spine fusion in patients with NMS. PATIENTS AND METHODS: Thirty-seven NMS patients were treated with pedicle screw instrumentation (PSI) between 2007 and 2013 with a minimum follow-up of 24 months. The mean age at the time of operation was 14.91 ± 2.03 years (range, 11.17-18.58). Posterior spine fusion (PSF) was conducted in 33 patients; 4 patients underwent a combined anterior spinal release followed by PSF during one-stage surgery. Pelvic fixation was achieved by ilium screws combined with S-1 screws in 4 cases and by sacral-alar-iliac (SAI) technique by Sponseller in 9 cases, respectively. RESULTS: The mean primary Cobb angle was 65.5° (range, 14-103°) and improved significantly to 19.8° (range, 1-50°, p < 0.001) after surgery and 20.5° (range, 3-57, p = 0.47) at 2 years FU, respectively. Besides, an improvement of pelvic obliquity and T1 tilt angle could be detected. Major complications occurred in 19% and minor complications in 32%. CONCLUSION: Pedicle screw fixation only for spine fusion in patients with NMS can be applied safely with reasonable complication rates. An excellent correction in all planes, a significant improvement of the pelvic obliquity and almost no loss of correction at 2 years FU were observed.

Outcome of the modified Dunn procedure in severe chronic or acute on chronic slipped capital femoral epiphysis.

BACKGROUND: In recent years, the modified Dunn osteotomy has gained popularity to treat slipped capital femoral epiphysis (SCFE) with various complication rates. Most studies included patients with different severities. This study aimed to determine (1) the radiological and clinical outcome, (2) the health-related quality of life, and (3) the incidence of avascular necrosis of the femoral head (AVN) in patients with severe chronic or acute on chronic SCFE treated by the modified Dunn procedure. METHODS: Out of 150 patients with SCFE treated at our institution between 2001 and 2014, 15 patients (mean age 12.9 years (range 11.8-15)) were treated by the modified Dunn procedure. Eight SCFE were chronic and 7 acute on chronic. All slips were severe with a mean Southwick slip angle (SSA) of 67° (range 60-80). Radiographic and clinical outcomes were measured. Mean time of follow-up was 3.8 years (range 1-10). RESULTS: Anatomical reduction was achieved in all cases. Good radiological results according to the Stulberg Classification (grade 1 + 2) and the Sphericity Deviation Score (< 30) were found in 9 out of 13 patients at the last follow-up. Clinical and functional outcome analysis revealed good results in 8 out of 10 patients (Harris Hip Score > 80). The quality of life measured by the Nottingham Health Profile (NHP) was described good in 10 out of 10 patients. Four out of 15 patients developed an AVN. CONCLUSIONS: The modified Dunn procedure has a great potential to restore proximal femur geometry in severe chronic or acute on chronic SCFE. It should be considered only if there is no other possibility to restore proximal femur geometry, as is the case in severe slips, due to the risk of AVN.

The Lysosomal Protein Arylsulfatase B Is a Key Enzyme Involved in Skeletal Turnover.

Skeletal pathologies are frequently observed in lysosomal storage disorders, yet the relevance of specific lysosomal enzymes in bone remodeling cell types is poorly defined. Two lysosomal enzymes, ie, cathepsin K (Ctsk) and Acp5 (also known as tartrate-resistant acid phosphatase), have long been known as molecular marker proteins of differentiated osteoclasts. However, whereas the cysteine protease Ctsk is directly involved in the degradation of bone matrix proteins, the molecular function of Acp5 in osteoclasts is still unknown. Here we show that Acp5, in concert with Acp2 (lysosomal acid phosphatase), is required for dephosphorylation of the lysosomal mannose 6-phosphate targeting signal to promote the activity of specific lysosomal enzymes. Using an unbiased approach we identified the glycosaminoglycan-degrading enzyme arylsulfatase B (Arsb), mutated in mucopolysaccharidosis type VI (MPS-VI), as an osteoclast marker, whose activity depends on dephosphorylation by Acp2 and Acp5. Similar to Acp2/Acp5-/- mice, Arsb-deficient mice display lysosomal storage accumulation in osteoclasts, impaired osteoclast activity, and high trabecular bone mass. Of note, the most prominent lysosomal storage accumulation was observed in osteocytes from Arsb-deficient mice, yet this pathology did not impair production of sclerostin (Sost) and Fgf23. Because the influence of enzyme replacement therapy (ERT) on bone remodeling in MPS-VI is still unknown, we additionally treated Arsb-deficient mice by weekly injection of recombinant human ARSB from 12 to 24 weeks of age. We found that the high bone mass phenotype of Arsb-deficient mice and the underlying bone cell deficits were fully corrected by ERT in the trabecular compartment. Taken together, our results do not only show that the function of Acp5 in osteoclasts is linked to dephosphorylation and activation of lysosomal enzymes, they also provide an important proof-of-principle for the feasibility of ERT to correct bone cell pathologies in lysosomal storage disorders. © 2018 The Authors. Journal of Bone and Mineral Research Published by Wiley Periodicals Inc.

Anterior distal femoral hemiepiphysiodesis can reduce fixed flexion deformity of the knee: a retrospective study of 83 knees.

Background and purpose - Fixed knee flexion deformity in children is a common problem in various diseases including myelomeningocele and cerebral palsy. Until now, only a few studies focusing on the surgical procedure of anterior distal femoral hemiepiphysiodesis have been published. We analyzed outcome and correction rate in the largest case series to date of patients treated by staples or 8-plates. Patients and methods - We reviewed the medical records of all patients with fixed knee flexion deformity who were treated with anterior distal femoral hemiepiphysiodesis using either staples or 8-plates between the years 2002 and 2017 (73 patients; 130 knees). 49 patients (83 knees) had completed treatment with implant removal at the time of full correction of the deformity or at skeletal maturity and were included. The average age at operation was 12 years (6-20). Patients were assigned to 3 different groups based on their diagnosis: cerebral palsy, myelomeningocele, and the "other" group.d Results - Mean fixed knee flexion deformity improved from 21° (10-60°) to 8° (0-50°) (p < 0.001) with an average correction rate of 0.44° per month (range -2.14° to 1.74°). The correction rate per month was lowest for patients with cerebral palsy (0.20°), followed by the myelomeningocele group (0.50°), and the "other" group (0.58°). Implant loosening occurred in 10% of the treated knees with consecutive re-implantation in 5% of the cases. Interpretation - Anterior distal femoral hemiepiphysiodesis is an effective and safe method for the treatment of fixed knee flexion deformity in children. The optimal timing depends on the remaining individual growth potential, the underlying disease, and the extent of the deformity.

Treatment of pediatric spinal deformity with use of recombinant human bone morphogenetic protein-2.

In pediatric spine surgery nonunion is a challenging issue. Instability may cause neurological impairment and lead to numerous surgeries in order to achieve fusion. The use of rhBMP-2 for pediatric spinal fusion has not been widely reported. In this study, a series of 13 children (14 procedures) that underwent spinal rhBMP-2 application were analyzed in order to measure clinical and radiographic outcome. Therefore, patient data, diagnosis, construct of instrumentation, type of bone graft, quantity of BMP used, and fusion outcome were reviewed. The study cohort included four female and nine male patients with a mean age of 11.2 years (range 2.6-19.2 years) at the time of rhBMP-2 application. Rh-BMP-2 was used in both primary (n = 6) and revision surgery (n = 8) in patients with a high risk for the development of nonunion. The mean follow-up was 51 months (range 12-108 months). Fusion occurred in 11 patients. Complications that may be due to application of rhBMP-2 were seen after four operations. Three patients had an increased body temperature and in one case prolonged wound secretion was evident, treated by local wound care or observation. In one of these patients an extensive postoperative hematoma occurred, necessitating surgical treatment. In conclusion, we could detect high fusion rates following the use of rhBMP-2 in pediatric spine surgery without an increased complication rate attributable to its application. Therefore we consider recombinant human BMP-2 to be an option in selected pediatric spinal procedures, especially in cases with compromised bone healing due to congenital, systemic, or local conditions.

Hip Dysplasia in Mucopolysaccharidosis Type IVA (Morquio A Syndrome) Treated by Proximal Femoral Valgization Osteotomy: A Case Report.

INTRODUCTION: Morquio A syndrome or mucopolysaccharidosis type IVA (MPS IVA) is a progressive lysosomal storage disorder caused by an N-acetylgalactosamine-6-sulfatase deficiency. The abnormal metabolism of glycosaminoglycans among other medical problems leads to various skeletal disorders caused by a dysfunction of endochondral ossification of epiphyseal cartilage. Severe hip dysplasia is common and can lead to pain and impaired mobility. CASE REPORT: We report on a 15-year-old girl suffering from MPS IVA. At the age of 5 years, hip pain and a reduced walking distance were described for the 1sttime. At the age of 9 years, acetabulofemoral dysplasia associated with genuavalga was diagnosed. After pre-operative assessment of the hips including plain radiographs, magnetic resonance imaging, and arthrography with dynamic testing a valgization osteotomy of the proximal femur in combination with a shelf acetabuloplasty was performed. The patient was followed for 6 years with a stable hip joint and without any sign of redislocation. CONCLUSION: Some treatment strategies of hip dysplasia in patients with MPS IVA are described in the literature. The techniques used for congenital hip dysplasia, varisation of the femur in combination with Pemberton, Salter, or shelf acetabuloplasty, are widely reported. Nevertheless, resubluxations were described in some cases. The well-known surgical procedure with valgization of the proximal femur is not reported in literature for MPS IVA patients. In our opinion, dynamic testing with arthrography should strongly be considered for this particular problem before surgical intervention. Pathology-related decisions should be made under consideration of the different surgical techniques.

Recombinant Human Bone Morphogenetic Protein-2 (rhBMP-2) for the Treatment of Nonunion of the Femur in Children and Adolescents: A Retrospective Analysis.

Background. The aim of this study was to examine clinical and radiographic healing after rhBMP-2 application in children and adolescents presenting with nonunion of the femur and to investigate the safety of rhBMP-2 use in these cases. Materials and Methods. We reviewed the medical records of five patients with a mean age of 11 years (5.4 to 16.2) with nonunion of the femur who were treated with rhBMP-2 and internal fixation using a locking plate at a single institution. Particular attention was paid to identify all adverse events that may be due to rhBMP-2 use. Results. Union occurred in four of five patients at a mean of 12.1 months (7.9 to 18.9). The locking plates were removed after a mean of 16 months (11 to 23). One patient had nonunion due to deep infection. After a mean follow-up of 62.5 months (17 to 100), union was still evident in all four patients and they were fully weight-bearing without pain. Discussion. In this retrospective study, rhBMP-2 combined with a locking plate has been used successfully to treat children and adolescents with nonunion of the femur in four of five cases. One major complication was thought to be possibly related to its use.

Bone morphogenetic protein-2 for the treatment of congenital pseudarthrosis of the tibia or persistent tibial nonunion in children and adolescents: A retrospective study with a minimum 2-year follow-up.

There is a lack of studies reporting on rhBMP-2 application in pediatric orthopaedics, although few reports demonstrated promising results of the use of rhBMP-2 in children, especially for spine fusion and for the treatment of congenital pseudarthrosis of the tibia. The objectives of this study were (1) to examine clinical and radiographic healing after rhBMP-2 application for the treatment of congenital pseudarthrosis of the tibia (CPT) or persistent tibial nonunion in children and adolescents, and (2) to investigate the safety of rhBMP-2 use in these cases. Therefore we reviewed the medical records of ten patients with a mean age of 8.6 years (2.3-21) with CPT (n = 7) or persistent tibial nonunion for at least six months (n = 3) who had been treated with rhBMP-2. Nine of ten patients had union at final follow-up, after a mean of 72.9 months (25-127). In the CPT group, primary healing of the pseudarthrosis occurred in six of seven patients at a mean of 5.2 months (3-12). Repeat rhBMP-2 application was performed in three patients; two patients had one additional application each, and one patient had three additional applications. Complications that may be attributed to the use of rhBMP-2 were seen in two of fifteen applications, including a compartmemt syndrome and a hematoma. In this retrospective case series rhBMP-2 has been used successfully to treat CPT or persistent tibial nonunion in pediatric patients. However, prospective randomized controlled trials are warranted to investigate the long-term efficacy and safety of rhBMP-2 use in these cases.