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  • 1
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    Aktuelle Therapiekonzepte und Ergebnisse für Kinder mit Spinaler Muskelatrophie und Wirbelsäulendeformität
    Georg Thieme Verlag KG ; 2020
    In:  Physikalische Medizin, Rehabilitationsmedizin, Kurortmedizin Vol. 59, No. 03 ( 2020-06), p. 174-179
    Details
    In: Physikalische Medizin, Rehabilitationsmedizin, Kurortmedizin, Georg Thieme Verlag KG, Vol. 59, No. 03 ( 2020-06), p. 174-179
    Abstract: Hintergrund Kinder mit Spinaler Muskelatrophie (SMA) Typ I und II entwickeln fast immer eine progrediente Wirbelsäulendeformität, welche nach Einführung der intrathekalen Injektionstherapie mit Nusinersen durch die damit verbundenen Schwierigkeiten bei der Punktion in den letzten Monaten in den Fokus gerückt sind. Ziel der Arbeit Die vorliegende Untersuchung untersucht die Ergebnisse einer neuartigen operativen Therapie zur Wirbelsäulenkorrektur bei SMA Kindern im Vergleich zu unbehandelten SMA Jugendlichen. Methoden In einer prospektiven Studie wurden 30 SMA Kinder (Gruppe I) mit von extern auszufahrenden magnetischen Implantaten versorgt, welche parallel zur Wirbelsäule implantiert wurden. Im Wachstumsverlauf wurden die Implantate ambulant alle drei Monate distrahiert. Diesem Kollektiv wurden 15 SMA Jugendliche (Gruppe II) mit unbehandelten Deformitäten gegenübergestellt. Ergebnisse Gruppe I war bei Implantation durchschnittlich 7,6 Jahre alt u nd wurde 41 Monate nachuntersucht. Der Skoliosekrümmungswinkel konnte von 62 auf 25° (59%; p 〈 0,001) und der Beckenschiefstand von 15 auf 4° (73%; p 〈 0,001) reduziert werden. Im Gegensatz dazu hatte Gruppe II (Alter 13,7 Jahre) eine Skoliose von 101° und einen Beckenschiefstand von 22°. In Gruppe I konnten die erzielten Korrekturen im Untersuchungszeitraum gehalten werden. Schlussfolgerung Bei SMA Kindern konnte durch die vorgestellte Operationstechnik eine signifikante Skoliosereduktion und Verbesserung des Beckenschiefstandes erreichen und im Verlauf gehalten werden im Vergleich zu unbehandelten Jugendlichen. Gerade auch in Hinblick auf die intrathekale Injektionstherapie mit Nusinersen zur SMA Behandlung ist die Notwendigkeit einer Wirbelsäule ohne starke Deformierung gegeben.
    Type of Medium: Online Resource
    ISSN: 0940-6689 , 1439-085X
    URL: Article
    DOI: 10.1055/a-0965-7485
    Language: German
    Publisher: Georg Thieme Verlag KG
    Publication Date: 2020
    detail.hit.zdb_id: 2072467-6
    SSG: 31
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  • 2
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    Magnetically Controlled Devices Parallel to the Spine in Children with Spinal Muscular Atrophy
    Ovid Technologies (Wolters Kluwer Health) ; 2017
    In:  JBJS Open Access Vol. 2, No. 4 ( 2017-12-28), p. e0036-
    Details
    In: JBJS Open Access, Ovid Technologies (Wolters Kluwer Health), Vol. 2, No. 4 ( 2017-12-28), p. e0036-
    Abstract: Children with severe spinal deformity frequently are managed with growth-friendly implants. After initial surgery, externally controlled magnetic rods allow spinal deformity correction during growth without further surgical intervention. The ability to lengthen the spine without additional surgical procedures is especially beneficial in high-risk children, such as those with spinal muscular atrophy (SMA). The purpose of the present study was to assess the level of control of spinal deformity in a homogeneous group of patients with SMA who were managed with magnetically controlled implants for 2 years. Methods: This prospective, nonrandomized study included 21 non-ambulatory children with type-II SMA and progressive scoliosis who were managed bilaterally with a magnetically controlled implant that was inserted parallel to the spine with use of rib-to-pelvis hook fixation. Radiographic measurements of scoliotic curves, kyphosis, lordosis, pelvic obliquity, and spinal length were performed before and after implantation of the magnetically controlled device and during external lengthening. The mean duration of follow-up was 2 years. Results: The mean main curve of patients without prior vertical expandable prosthetic titanium rib (VEPTR) treatment decreased from 70° before implantation of the magnetically controlled device to 30° after implantation of the device. Correction was maintained during the follow-up period, with a mean curve of 31° at the time of the latest follow-up at 2.2 years. Pelvic obliquity was surgically corrected by 76% (from 17° to 4°) and remained stable during follow-up. Thoracic kyphosis could not be corrected within the follow-up period. Spinal length of children without prior spinal surgery increased by 〉 50 mm immediately after device implantation and steadily increased at a rate of 13.5 mm/yr over the course of treatment. During treatment, 4 general complications occurred and 6 lengthening procedures failed, with 3 patients requiring surgical revision. Conclusions: Bilateral implantation of an externally controlled magnetic rod with rib-to-pelvis fixation represents a safe and efficient method to control spinal deformity in children with SMA, achieving sufficient and stable curve correction as well as increased spinal length. The complication rate was lower than those that have been described for VEPTR and other growing rod instrumentation strategies. Level of Evidence: Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.
    Type of Medium: Online Resource
    ISSN: 2472-7245
    URL: Article
    DOI: 10.2106/JBJS.OA.17.00036
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2017
    detail.hit.zdb_id: 2873843-3
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  • 3
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    Spinal Deformities after Childhood Tumors
    MDPI AG ; 2020
    In:  Cancers Vol. 12, No. 12 ( 2020-11-28), p. 3555-
    Details
    In: Cancers, MDPI AG, Vol. 12, No. 12 ( 2020-11-28), p. 3555-
    Abstract: Childhood tumors of the central nervous system (CNS) and other entities affecting the spine are rare. Treatment options vary from surgical biopsy to partial, subtotal, and total resection, to radiation, to chemotherapy. The aim of this study is to investigate spinal deformity and subsequent surgical interventions in this patient cohort. A retrospective review at our institution identified children with CNS tumors, spinal tumors, and juxta-spinal tumors, as well as spinal deformities. Tumor entity, treatment, mobilization, and radiographic images were analyzed relative to the spinal deformity, using curve angles in two planes. Conservative or surgical interventions such as orthotic braces, growth-friendly spinal implants, and spinal fusions were evaluated and analyzed with respect to treatment results. Tumor entities in the 76 patients of this study included CNS tumors (n = 41), neurofibromatosis with spinal or paraspinal tumors (n = 14), bone tumors (n = 12), embryonal tumors (n = 7), and others (n = 2). The initial treatment consisted of surgical biopsy (n = 5), partial, subtotal, or total surgical resection (n = 59), or none (n = 12), followed by chemotherapy, radiotherapy, or both (n = 40). Out of 65 evaluated patients, 25 revealed a moderate or severe scoliotic deformity of 71° (range 21–116°), pathological thoracic kyphosis of 66° (range 50–130°), and lordosis of 61° (range 41–97°). Surgical treatment was performed on 21 patients with implantation of growth-friendly spinal implants (n = 9) as well as twelve dorsal spinal fusions (two with prior halo distraction). Surgical interventions significantly improved spinal deformities without additional neurological impairment. With the increasing number of children surviving rare tumors, attention should be focused on long-term problems such as spinal deformities and consequent disabilities. A significant number of children with CNS tumors, spinal tumors or juxta-spinal tumors required surgical intervention. Early information about spinal deformities and a close follow-up are mandatory for this patient group.
    Type of Medium: Online Resource
    ISSN: 2072-6694
    URL: Article
    DOI: 10.3390/cancers12123555
    Language: English
    Publisher: MDPI AG
    Publication Date: 2020
    detail.hit.zdb_id: 2527080-1
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  • 4
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    Surgical Treatment of Spinal Deformities in Young Paraplegic Children with Intraspinal Tumors
    S. Karger AG ; 2018
    In:  Pediatric Neurosurgery Vol. 53, No. 3 ( 2018), p. 149-152
    Details
    In: Pediatric Neurosurgery, S. Karger AG, Vol. 53, No. 3 ( 2018), p. 149-152
    Abstract: Bilateral vertical expandable prosthetic titanium rib (VEPTR) treatment using rib-to-pelvis constructs without touching the spine is a safe surgical technique to correct scoliosis while still allowing further MRI or neurosurgical interventions. In this retrospective cohort study, 4 paraplegic children with spinal deformity after intraspinal tumors and 4 children with neuromuscular diseases were compared. VEPTR treatment was able to considerably reduce the main scoliotic curve in both patient groups (41 vs. 40%). However, the tumor group constantly showed more severe curve progression over time and less favorable pelvic obliquity control. In conclusion, bilateral VEPTR can be expected to be less satisfying in children with tumors.
    Type of Medium: Online Resource
    ISSN: 1016-2291 , 1423-0305
    URL: Article
    DOI: 10.1159/000487810
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2018
    detail.hit.zdb_id: 1483546-0
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  • 5
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    Altered bone development with impaired cartilage formation precedes neuromuscular symptoms in spinal muscular atrophy
    Oxford University Press (OUP) ; 2020
    In:  Human Molecular Genetics Vol. 29, No. 16 ( 2020-09-29), p. 2662-2673
    Details
    In: Human Molecular Genetics, Oxford University Press (OUP), Vol. 29, No. 16 ( 2020-09-29), p. 2662-2673
    Abstract: Spinal muscular atrophy (SMA) is a fatal neurodegenerative disease of newborns and children caused by mutations or deletions of the survival of motoneuron gene 1 resulting in low levels of the SMN protein. While neuromuscular degeneration is the cardinal symptom of the disease, the reduction of the ubiquitously expressed SMN additionally elicits non-motoneuron symptoms. Impaired bone development is a key feature of SMA, but it is yet unknown whether this is an indirect functional consequence of muscle weakness or caused by bone-intrinsic mechanisms. Therefore, we radiologically examined SMA patients in a prospective, non-randomized cohort study characterizing bone size and bone mineral density (BMD) and performed equivalent measurements in pre-symptomatic SMA mice. BMD as well as lumbar vertebral body size were significantly reduced in SMA patients. This growth defect but not BMD reduction was confirmed in SMA mice by μCT before the onset of neuromuscular symptoms indicating that it is at least partially independent of neuromuscular degeneration. Interestingly, the number of chondroblasts in the hypertrophic zone of the growth plate was significantly reduced. This was underlined by RNAseq and expression data from developing SMA mice vertebral bodies, which revealed molecular changes related to cell division and cartilage remodeling. Together, these findings suggest a bone intrinsic defect in SMA. This phenotype may not be rescued by novel drugs that enhance SMN levels in the central nervous system only.
    Type of Medium: Online Resource
    ISSN: 0964-6906 , 1460-2083
    URL: Article
    DOI: 10.1093/hmg/ddaa145
    RVK:
    XA 10000
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
    detail.hit.zdb_id: 1474816-2
    SSG: 12
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  • 6
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    High Correlation Between Achieved and Expected Distraction Using Magnetically Controlled Growth Rods (MCGR) With Rib to Pelvis Fixation in Pediatric Spine Deformity
    Ovid Technologies (Wolters Kluwer Health) ; 2019
    In:  Journal of Pediatric Orthopaedics Vol. 39, No. 5 ( 2019-05), p. e334-e338
    Details
    In: Journal of Pediatric Orthopaedics, Ovid Technologies (Wolters Kluwer Health), Vol. 39, No. 5 ( 2019-05), p. e334-e338
    Abstract: Magnetically controlled implant systems have been established to treat severe progressive spinal deformity in children. The purpose of this study was to evaluate (1) the ratio between achieved and expected distraction length, (2) the complication rate and its risk factors as well as (3) the correlation of the distraction length and the length of the spine. Methods: A total of 40 patients with an average follow-up of 34 (14 to 57) months were prospectively included in the study. Children underwent lengthening procedures every three months. The ratio between the distraction lengths was determined by comparing the measured distraction length of the rod on radiographs with the distraction length displayed on the external remote controller for the magnetically controlled growing rod (MCGR). Age, weight, height, and complications were repeatedly recorded. Results: The analysis of 746 procedures showed the actual distraction to be 94.4% of the expected one. No difference between implants on the concave and convex spinal side was observed. The overall complication rate was 4.6% mainly because of failure of the implant or lack of implant extension, which was directly related to an increased BMI. There was also a strong correlation between achieved implant distraction length and gain in spinal length. Conclusions: Our study demonstrates a high ratio (0.94) between achieved and expected distraction length of magnetically controlled spinal rods. The complication rate was low (4.6%) and correlated to a high BMI. The correlation between the achieved implant distraction length and spinal length indicates the efficiency of the MCGR therapy. Level of Evidence: Therapeutic Level IV.
    Type of Medium: Online Resource
    ISSN: 0271-6798
    URL: Article
    DOI: 10.1097/BPO.0000000000001303
    Language: English
    Publisher: Ovid Technologies (Wolters Kluwer Health)
    Publication Date: 2019
    detail.hit.zdb_id: 2049057-4
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  • 7
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    Continuous lengthening potential after four years of magnetically controlled spinal deformity correction in children with spinal muscular atrophy
    Springer Science and Business Media LLC ; 2020
    In:  Scientific Reports Vol. 10, No. 1 ( 2020-12-30)
    Details
    In: Scientific Reports, Springer Science and Business Media LLC, Vol. 10, No. 1 ( 2020-12-30)
    Abstract: Magnetically controlled growing rods (MCGR) are commonly implanted for the treatment of early-onset scoliosis. While most authors report favorable short-term results, little is known about long-term deformity correction. This prospective cohort study assesses spinal deformity control in a homogeneous spinal muscular atrophy (SMA) patient group treated with MCGR implants, a standardized lengthening protocol and a minimum follow-up of four years. 17 SMA patients with progressive scoliosis were treated with MCGR implanted parallel to the spine with rib-to-pelvis fixation. Radiologic measurements were performed before and after MCGR implantation and during external lengthening procedures. These included measurements of the scoliotic curve, kyphosis, lordosis, pelvic obliquity and the spinal length. Additional clinical data of the complications were also analyzed. 17 children (mean age 7.4 years) were surgically treated and underwent a total of 376 lengthenings. Complication rates were 3.5% in respect to all interventions or 41% of the patients had complications during 3.5% of the lengthening sessions. The initial implantation significantly reduced the main scoliotic curve by 59%, with the correction remaining constant throughout the follow-up. Pelvic obliquity was also significantly and permanently corrected by 72%, whereas kyphosis and lordosis were not influenced. The spinal length could be significantly increased mostly during the first year of treatment. Bilateral implantation of MCGRs for correction of spinal deformity in children with SMA showed no decrease of the lengthening potential during a four-year follow-up. Therefore, the previously described ‘law of diminishing returns’ could not be applied to this patient population. Level of Evidence/Clinical relevance: Therapeutic Level IV.
    Type of Medium: Online Resource
    ISSN: 2045-2322
    URL: Article
    DOI: 10.1038/s41598-020-79821-x
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2020
    detail.hit.zdb_id: 2615211-3
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  • 8
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    Influence of Paraspinal Growth-Friendly Spinal Implants in Children with Spinal Muscular Atrophy on Parasol Deformity, Rib-Vertebral Angles, Thoracic, and Lung Volumes
    S. Karger AG ; 2023
    In:  Pediatric Neurosurgery Vol. 58, No. 4 ( 2023), p. 185-196
    Details
    In: Pediatric Neurosurgery, S. Karger AG, Vol. 58, No. 4 ( 2023), p. 185-196
    Abstract: 〈 b 〉 〈 i 〉 Introduction: 〈 /i 〉 〈 /b 〉 Children with spinal muscular atrophy (SMA) and progressive neuromuscular scoliosis often require early growth-friendly spinal implant (GFSI) treatment for deformity correction with implant fixation either through pedicle screws or bilateral to the spine using ribto pelvis fixation. It has been proposed that the latter fixation may change the collapsing parasol deformity via changes in the rib-vertebral angle (RVA) with a positive effect on thoracic and lung volume. The purpose of this study was to analyze the effect of paraspinal GFSI with bilateral rib-to-pelvis fixation on the parasol deformity, RVA, thoracic, and lung volumes. 〈 b 〉 〈 i 〉 Methods: 〈 /i 〉 〈 /b 〉 SMA children with ( 〈 i 〉 n 〈 /i 〉 = 19) and without ( 〈 i 〉 n 〈 /i 〉 = 18) GFSI treatment were included. Last follow-up was before definite spinal fusion at puberty. Scoliosis and kyphosis angles, parasol deformity, and index, as well as convex and concave RVA, were measured on radiographs, whereas computed tomography images were used to reconstruct thoracic and lung volumes. 〈 b 〉 〈 i 〉 Results: 〈 /i 〉 〈 /b 〉 In all SMA children ( 〈 i 〉 n 〈 /i 〉 = 37; with or without GFSI), convex RVA was smaller than concave values at all times. GFSI did not crucially influence the RVA over the 4.6-year follow-up period. Comparing age- and disease-matched adolescents with and without prior GFSI, no effect of GFSI treatment could be detected on either RVA, thoracic, or lung volumes. Parasol deformity progressed over time despite GFSI. 〈 b 〉 〈 i 〉 Conclusion: 〈 /i 〉 〈 /b 〉 Despite different expectations, implantation of GFSI with bilateral rib-to-pelvis fixation did not positively influence parasol deformity, RVA and/or thoracic, and lung volumes in SMA children with spinal deformity directly and over time.
    Type of Medium: Online Resource
    ISSN: 1016-2291 , 1423-0305
    URL: Article
    DOI: 10.1159/000531549
    Language: English
    Publisher: S. Karger AG
    Publication Date: 2023
    detail.hit.zdb_id: 1483546-0
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  • 9
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    Foot Typology, Dynamic and Static Weight Distribution, and Radiographic Changes After Subtalar Arthroereisis in Juvenile Symptomatic Flexible Flat Feet
    Elsevier BV ; 2022
    In:  The Journal of Foot and Ankle Surgery Vol. 61, No. 2 ( 2022-03), p. 272-278
    Details
    In: The Journal of Foot and Ankle Surgery, Elsevier BV, Vol. 61, No. 2 ( 2022-03), p. 272-278
    Type of Medium: Online Resource
    ISSN: 1067-2516
    URL: Article
    DOI: 10.1053/j.jfas.2021.07.021
    Language: English
    Publisher: Elsevier BV
    Publication Date: 2022
    detail.hit.zdb_id: 2102633-6
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  • 10
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    Smaller Intervertebral Disc Volume and More Disc Degeneration after Spinal Distraction in Scoliotic Children
    MDPI AG ; 2021
    In:  Journal of Clinical Medicine Vol. 10, No. 10 ( 2021-05-14), p. 2124-
    Details
    In: Journal of Clinical Medicine, MDPI AG, Vol. 10, No. 10 ( 2021-05-14), p. 2124-
    Abstract: In recent decades, magnetically controlled growing rods (MCGR) were established to treat progressive early-onset scoliosis. The aim of this investigation was to assess the effect of long-term MCGR with continuous distraction on intervertebral discs in scoliotic children. Magnetic resonance imaging (MRI) of 33 children with spinal muscular atrophy was analyzed by grading intervertebral disc degeneration (IDD) and measuring intervertebral disc volume. Cohort I (n = 17) were children who had continuous spinal distraction with MCGRs for 5.1 years and MRI before (av. age 8.1) and after (av. age 13.4) MCGR treatment. Cohort II (n = 16, av. age 13.7) were patients without prior surgical treatment. Lumbar intervertebral disc volume of cohort I did not change during 5.1 years of MCGR treatment, whereas disc volumes were significantly larger in age- and disease-matched children without prior treatment (cohort II). Cohort I showed more IDD after MCGR treatment in comparison to early MRI studies of the same patients and children without surgical treatment. MRI data showed a volume reduction and disc degeneration of lower thoracic and lumbar intervertebral discs in scoliotic children after continuous spinal distraction with MCGRs. These effects were confirmed in the same subjects before and after treatment as well as in surgically untreated controls.
    Type of Medium: Online Resource
    ISSN: 2077-0383
    URL: Article
    DOI: 10.3390/jcm10102124
    Language: English
    Publisher: MDPI AG
    Publication Date: 2021
    detail.hit.zdb_id: 2662592-1
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