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Most children with spinal muscular atrophy (SMA) develop spinal deformity, which may require surgical intervention. In addition to poor bone stock, vertebral body shape may hinder the placement of spinal implants resulting in complications and poor outcome. The aim of this study was to analyze whether vertebral body morphology of children and adolescents with SMA is altered in comparison to healthy age-matched controls.In this prospective cohort study, 17 children with SMA (mean age 8.7 ±1.0 years) and 13 adolescents with SMA (mean age 13.6 ±1.4 years), all with some degree of neuromuscular scoliosis, were analyzed by standardized radiographic measurements to evaluate vertebral body height and depth. Results were compared with age-matched healthy controls (n = 10 children; mean age 9.1 ± 1.6 years; n = 20 adolescents, mean age 13.1 ± 0.5 years). Computed tomography scans of 27 adolescents with SMA (13.5 ±1.2 years) and 25 healthy age-matched controls (13.8 ±2.0 years) were analyzed to define pedicle diameters.All children and adolescents with SMA had decreased vertebral height and depth in comparison to age-matched healthy controls. In adolescents, reduced depth was more pronounced than height in the thoracic spine. Pedicle size was significantly reduced in the lower thoracic and lumbar area.Reduced vertebral body height and depth and pedicle size in children and adolescents with SMA may influence surgical treatment of spinal deformity. Surgeons should be aware of anatomical differences and choose implant devices accordingly. more...

Duchenne muscular dystrophy is a progressive disease usually associated with loss of ambulation and progressive scoliosis. Immobilisation and glucocorticoid treatment are predisposing factors for reduced bone mineral density (BMD). Analysis of quantitative computed tomography revealed low BMD in thoracic and lumbar vertebrae in comparison to age- and sex-matched healthy controls.Evaluation of vertebral bone mineral density (BMD) in Duchenne Muscular Dystrophy (DMD) adolescents with untreated advanced scoliosis and comparison with the BMD values of healthy age-matched controls, based on quantitative computer tomography.Thirty-seven DMD adolescents (age 15.6 ± 2.5 years) with spinal deformity were evaluated clinically and radiologically prior to definite spinal fusion and compared to 31 male and age-matched healthy individuals (age 15.7 ± 2.3 years). Data related to previous medical treatment, physiotherapy and ambulatory status was also analysed. Scoliotic curves were measured on plain sitting radiographs of the spine. The BMD Z-scores of the thoracic and lumbar vertebrae were calculated with QCTpro® (Mindways Software Inc., USA), based on data sets of preoperative, phantom pre-calibrated spinal computed tomography scans.A statistically significant lower BMD could be found in DMD adolescents, when compared to healthy controls, showing an average value for the lumbar spine of 80.5 ± 30.5 mg/cmDMD adolescents with scoliosis have strongly reduced BMD Z-scores, especially in the lumbar spine in comparison to healthy controls. These findings support the implementation of a standardised screening and treatment protocol. Level of evidence/clinical relevance: therapeutic level III. more...

Die kongenitale Pseudarthrose der Tibia (CPT) ist eine seltene Erkrankung, die Unfallchirurgen und Orthopaden seit langer Zeit herausfordert. Klinisch imponieren eine meist zunehmende Antekurvations- und Varusfehlstellung der Tibia im Sauglings- und im Kleinkindalter. Oft ist die CPT mit einer Neurofibromatose oder fibrosen Dysplasie vergesellschaftet. Eine Diskontunitat der Tibia und/oder Fibula kann bei Unkenntnis des Krankheitsbildes als einfache Fraktur fehlgedeutet werden. Histopathologische Untersuchungen lassen vermuten, dass eine pathologische Veranderung des Periosts im Bereich der Pseudarthrose, das Hamartom, im Wesentlichen fur dieses Krankheitsbild verantwortlich zu sein scheint. Somit muss zur Heilung der CPT auch eine Resektion des Hamartoms erfolgen. Multimodale Therapieansatze mit Vereinigung medikamentoser und operativer Ansatze wie z. B. die X‑Union-Technik von Paley scheinen im Verlauf eine gunstigere Prognose aufzuzeigen. more...

Background. Brace treatment is frequently used in adolescent idiopathic scoliosis (AIS). However, due to different brace models, long-term results on spinal deformity development at the end of Chneau brace treatment are not often described and differ in results. Aim. The aim of this work was to analyze clinical and radiological data of AIS patients treated with Chneau braces from the beginning of treatment until the end of growth and brace therapy in order to define realistic treatment results and expectations in an everyday setting. Materials and methods. 52 AIS patients with Chneau brace treatment were followed from the beginning of treatment until the end of growth. Clinical data such as the initial Risser sign, age at treatment, gender, curve patterns and body mass index were analyzed. Results. At the beginning of brace therapy, the average age was 13.1 years and patients showed a mean scoliotic curve angle of 30.9. Four months of brace use reduced the scoliotic curve to 20.1. Nine months after the end of brace treatment and an average treatment duration of 17 months, scoliosis has increased up to 30.3 again. In children with a lower maturity status, the initial scoliotic curve was less than in more mature patients leading to less spinal deformity at the end of treatment. In addition, obese children had less scoliosis correction during brace therapy than normalweight children. Conclusion. In patients with AIS treated with a Chneau brace, the initial curvature correction was 35%. Nine months after the end of brace treatment, scoliotic curves identical to the deformities at the beginning of treatment could be observed. more...

Skoliosen im Kindes- und Jugendalter benotigen haufig aufwendige konservative und operative Therapieverfahren. Zur Vermeidung unerfreulicher Verlaufe ist die detaillierte Kenntnis des Wachstumsverhaltens, der Prognose und von Begleitproblematiken unerlasslich. Kenntnisse zu Atiologie, Prognose, Diagnostik und Therapie von Wirbelsaulendeformitaten im Wachstumsalter sollen vermittelt werden. Die vorliegende Arbeit beruht auf einer selektiven Literaturrecherche und auf den Ergebnissen mehrerer eigener prospektiver und retrospektiver Untersuchungen. Abhangig von Atiologie und Patientenalter ist eine detaillierte und interdisziplinare Diagnostik notig. Konservative Therapien, z. B. die Versorgung mithilfe eines Cheneau-Korsetts, konnen Krummungen bestenfalls aufhalten, aber nicht auf lange Sicht umkehren. Operative Therapien mit wachstumsfreundlichen Implantaten sind aufwendig sowie mit Komplikationen und Mehrfacheingriffen verbunden. Von extern zu verlangernde, magnetische Implantate vermindern die Zahl notwendiger operativer Eingriffe, tragen aber nicht zur Erhohung der Lebensqualitat bei. Unterschiedliche Formen der Skoliose benotigen verschiedene Therapieansatze. Wichtig ist die Kenntnis von der Art der Skoliose und dem wahrscheinlichen Verlauf. Abhangig davon muss die Familie entsprechend beraten und ein Therapiekonzept entwickelt werden. Wachstumsfreundliche Implantate werden als Ubergangslosung bis zur Wirbelsaulenversteifung eingesetzt. more...

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. more...

Magnetically controlled growing rods (MCGRs) are an effective treatment method for early-onset scoliosis (EOS). In recent years, increasing titanium wear was observed in tissue adjacent to implants and in blood samples of these patients. This study aims to investigate the potential correlation between amount of metal loss and titanium levels in blood during MCGR treatment as well as influencing factors for metal wear. In total, 44 MCGRs (n = 23 patients) were retrieved after an average of 2.6 years of implantation and analyzed using a tactile measurement instrument and subsequent metal loss calculation. Titanium plasma levels (n = 23) were obtained using inductively coupled plasma-mass spectrometry (ICP-MS). The correlation of both parameters as well as influencing factors were analyzed. Titanium abrasion on MCGRs was observed in the majority of implants. There was no correlation of metal implant wear or titanium plasma values to the duration of MCGR implantation time, number of external lengthening procedures, patient’s ambulatory status, gender, weight or height. Material loss on the MCGRs showed a positive correlation to titanium blood plasma values. The present study is one of the first studies to analyze retrieved MCGRs using high-precision metrological techniques and compare these results with ICP-MS analyses determining blood titanium values. more...

Reduktion und Retention des Krummungswinkels bei Kindern mit neuromuskularen Erkrankungen und progredienten Wirbelsaulendeformitaten. Progressive neuromyopathische Skoliose, welche konservativ nicht beherrscht werden kann (v. a. beim nicht gehfahigen Kind), und/oder Entwicklung eines Thoraxinsuffizienzsyndroms (TIS). Ungenugende Weichteildeckung, Korpergewicht 25 oder >50 kg, fehlende knocherne Verankerungsstrukturen (Rippen), ausgewachsenes Skelett (normalerweise Alter 50 % erzielt und eine Progression uber Jahre effektiv verhindert werden. Bei 495 ambulanten Verlangerungsprozeduren betrug die Rate an operationspflichtigen implantatassoziierten Komplikationen 3,7 %. Im Verlauf erhielten 29 % der 45 Kinder eine operative Revision. Mit der vorgestellten operativen „No-touch“-Technik zur Skoliosekorrektur kindlicher neuromyopathischer Deformitaten kann eine effektive Reduktion des Skoliosekrummungswinkels erreicht und gehalten werden. Vorteile der Methode sind ein teilweiser Erhalt der Wirbelsaulenflexibilitat und eine Reduktion wirbelsaulennaher Ossifikationen, was die definitive dorsale Spondylodese als Endpunkt der Behandlung erleichtert. more...

Purpose 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. more...

Congenital pseudarthrosis of the tibia (CPT) is a rare disease that has challenged trauma and orthopedic surgeons for a long time. Clinically CPT mostly presents with progressive varus and antecurvation malformation of the tibia in infancy and childhood. In many cases CPT is associated with neurofibromatosis or fibrous dysplasia. Without knowledge of the clinical picture a discontinuity of the tibia and/or fibula visible on an X‑ray can be misinterpreted as a simple fracture. Histopathological investigations have revealed that a pathological alteration of the periosteum in the region of the pseudarthrosis, a hamartoma, may be essentially responsible for this disorder. Consequently, to treat the CPT a resection of the hamartoma must also be carried out. Multimodal treatment approaches combining pharmacological and surgical treatment, such as the cross-union technique of Paley, seem to have improved the prognosis of CPT.Die kongenitale Pseudarthrose der Tibia (CPT) ist eine seltene Erkrankung, die Unfallchirurgen und Orthopäden seit langer Zeit herausfordert. Klinisch imponieren eine meist zunehmende Antekurvations- und Varusfehlstellung der Tibia im Säuglings- und im Kleinkindalter. Oft ist die CPT mit einer Neurofibromatose oder fibrösen Dysplasie vergesellschaftet. Eine Diskontunität der Tibia und/oder Fibula kann bei Unkenntnis des Krankheitsbildes als einfache Fraktur fehlgedeutet werden. Histopathologische Untersuchungen lassen vermuten, dass eine pathologische Veränderung des Periosts im Bereich der Pseudarthrose, das Hamartom, im Wesentlichen für dieses Krankheitsbild verantwortlich zu sein scheint. Somit muss zur Heilung der CPT auch eine Resektion des Hamartoms erfolgen. Multimodale Therapieansätze mit Vereinigung medikamentöser und operativer Ansätze wie z. B. die X‑Union-Technik von Paley scheinen im Verlauf eine günstigere Prognose aufzuzeigen. more...

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. more...

Purpose In young children, growth-friendly spinal implants with bilateral rib to pelvis fixation are used to control progressive spinal deformity. Whereas curve progression, complications and side-effects have been extensively studied in this patient population, no data are available on gait pattern changes and postural body adjustments. Our study evaluates whether gait pattern changed for ambulatory children treated with bilateral rib to pelvis implants compared to age-matched healthy children. Methods In this small cohort study, gait analysis was performed using spatiotemporal and kinematic parameters of four ambulatory children with severe scoliosis and growth-friendly spinal implants using the bilateral rib to pelvis fixation. Data were statistically analyzed and compared to seven healthy age-matched children. Results Between both groups, no differences were seen in walking speed, cadence and stride length. The treated patients showed a lower range of motion of the pelvic obliquity and of the trunk obliquity and rotation, but a higher knee flexion. Conclusions Growth-friendly spinal implants with bilateral rib to pelvis fixation are commonly used in wheelchair children and rarely indicated in ambulatory patients. The presented data show reduced trunk and pelvis motion using this implant construct. These findings help to understand body postural adjustments and add valuable information for families and care providers when considering this surgery. Level of evidence Therapeutic level IV. more...

Flexible flatfoot is among the most common skeletal disorders in childhood. This study describes the dynamic and static correction effects of subtalar arthroereisis in adolescents with flexible symptomatic flatfeet in comparison to normal subjects as well as to results before and after removal of metal. Eighteen adolescents with 25 symptomatic flexible flatfeet were treated surgically with a subtalar arthroereisis at a mean of 12.5 (10-16) years. At follow-up (mean 3.9 years, range 0.4-8), patients filled out the American Orthopaedic Foot and Ankle Society questionnaire, received radiographs and were examined using dynamic and static pedobarography as well as static hindfoot axis examination. Results were compared to healthy controls (n = 13; 26 feet). Surgically treated feet (n = 25) had better questionnaire results after surgery than before, but lower scores than healthy feet. Radiological parameters improved significantly after surgery. Removal of metal did not influence post-surgical results (follow-up 2.8 years). Surgically treated feet had larger contact areas than normal feet with predominance to the midfoot region. The relative maximum force, relative peak pressure and contact time were higher in the midfoot of treated feet compared to controls. When comparing pedobarography data of treated versus untreated feet of the same patients (subgroup n = 11 feet), there were no differences. Subtalar arthroereisis was able to effectively treat symptomatic flexible flatfeet in this population. Results improved significantly evaluating a questionnaire, radiographs, dynamic and static weight distribution, but were still worse than results of healthy feet. There was no relapse after removal of metal. more...

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&deg, (range 21&ndash, 116&deg, ), pathological thoracic kyphosis of 66&deg, (range 50&ndash, 130&deg, ), and lordosis of 61&deg, (range 41&ndash, 97&deg, ). 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. more...

Purpose Growth-friendly spinal implants (GFSI) were established for scoliotic children as an interim solution until definite spinal fusion could be performed during puberty. While deformity control was clearly proven, the effects on vertebral shape and morphology are still unclear. Our prospective study assesses the effect of GFSI with continuous distraction on vertebral body shape and volume in SMA children in comparison with previously untreated age-matched SMA patients. Methods Cohort I (n = 19, age 13.2 years) were SMA patients without prior surgical scoliosis treatment. Cohort II (n = 24, age 12.4 years) were children, who had continuous spinal distraction with GFSI for 4.5 years. Radiographic measurements and computed tomography (CT) 3D volume rendering were performed before definite spinal fusion. For cohort II, additional radiographs were analyzed before the first surgical implantation of GFSI, after surgery and every year thereafter. Results Our analysis revealed decreased depth and volume in scoliotic patients with prior GFSI compared to scoliotic patients without prior implants. This difference was significant for the lower thoracic and entire lumbar spine. Vertebral body height and pedicle size were unchanged between the two cohorts. Conclusion CT data showed volume reduction in the vertebral body in scoliotic children after GFSI treatment. This effect was more severe in the lumbar and lower thoracic area. While vertebral height was identical in both groups, vertebral depth was reduced in the GFSI-treated group. Reduced vertebral depth and altered vertebral morphology should be considered before instrumenting the spine in previously treated scoliotic SMA children. Level of evidence III Diagnostic: individual cross-sectional studies with consistently applied reference standard and blinding. more...

Wearing a school backpack every day may cause postural problems and affect the gait pattern of children and adolescents. The aim of the present study was to analyze the influence of a 4 kg backpack load on the gait pattern and postural sway.The aim of the present study was to analyze the influence of a backpack load of 4 kg on the gait and postural sway of elementary school children.In this prospective study, a group of 12 elementary school children aged between 7 and 10 years without neurological or orthopedic problems participated. The measurements included a clinical examination, three-dimensional gait analysis with electromyographic recordings and measurement of postural sway on a force plate.The backpack load, on average 15% of the body weight, led to a slower walking speed, shorter step length and increased double-support phase. Increased anterior pelvic and trunk tilt, as well as hip flexion were also observed. Furthermore, the muscle activity and postural sway of the children were affected by the increased load.School backpacks weighing 4 kg caused changes in gait, muscle activity, posture and stability in elementary school children. Due to the weight of the backpack, the centre of mass shifted backwards and the children became less stable. During gait, this was compensated by increased anterior pelvic and trunk tilt and increased hip flexion. The activity of the paraspinal muscles was decreased and indicates that the backpack is carried passively. This may cause a negative long-term effect.HINTERGRUND: Das tägliche Tragen eines schweren Schulrucksacks kann möglicherweise Haltungsstörungen hervorrufen, die sich auf das Gangbild und die Standstabilität der Kinder und Jugendlichen auswirken.Ziel der vorliegenden Studie war es, den Einfluss einer Rucksacklast von 4 kg auf das Gangbild und die Standstabilität bei Grundschulkindern zu analysieren.In der prospektiven Arbeit wurde ein Kollektiv von zwölf Grundschulkindern zwischen 7 und 10 Jahren ohne neurologische oder orthopädische Probleme untersucht. Die Messungen beinhalteten eine klinische Untersuchung, eine dreidimensionale Ganganalyse mit elektromyografischen Aufzeichnungen und die Prüfung des ruhigen Stehens auf einer Kraftmessplatte.Die Rucksacklast von durchschnittlich 15 % des Körpergewichts führte zu reduzierter Ganggeschwindigkeit, verkürzter Schrittlänge und verlängerter Doppelunterstützungsphase. Zudem kam es zu einer erhöhten Oberkörpervorneigung, Beckenkippung und Hüftbeugung. Auch die Muskelaktivität und Stabilität der Kinder wurde durch die erhöhte Traglast beeinflusst.Schulrucksäcke mit einem Gewicht von 4 kg führten bei Grundschulkindern zu Änderungen von Gang, Muskelaktivität, Haltung und Standstabilität. Das Gewicht des Rucksacks verlagert den Körperschwerpunkt nach hinten und führt zur Instabilität. Dies wird beim Gehen durch eine vermehrte Oberkörpervorneigung, Beckenkippung nach vorne und vermehrte Hüftbeugung kompensiert. Die verminderte Paraspinalmuskelaktivität deutet darauf hin, dass der Rucksack passiv getragen wird. Dies könnte sich im Langzeitverlauf negativ auswirken. more...

ZusammenfassungDer kindliche Knick-Senk-Fuß ist eine 3-dimensionale Fußfehlstellung mit vermehrter Valgusstellung der Ferse, Abflachung der medialen Längswölbung und Abduktion des Vorfußes. Man unterscheidet die flexible von der kontrakten (rigiden) Form. Die Diagnostik gliedert sich in Anamnese, klinische Untersuchung, apparative Diagnostik wie Pedobarografie und die Ganganalyse sowie bildgebende Verfahren. Wichtig ist die Einbeziehung in ein Therapieschema von eventuell vorliegenden pädiatrischen oder neuropädiatrischen Erkrankungen (z. B. Muskeldystrophien, Ehlers-Danlos- oder Marfan-Syndrom u. Ä.). Es zeigt sich, dass bei Kindern ≤ 6 Jahre der flexible Knick-Senk-Fuß fast ausschließlich physiologisch vorkommt (bei 97% aller 19 Monate alten Kinder). Bis zum Alter von 10 Jahren bildet sich die mediale Fußlängswölbung zunehmend aus. Bei einem kleinen Anteil (10-jährige Kinder 4%) persistiert der Knick-Senk-Fuß oder wird progredient. Oberhalb von 10 Jahren besteht die Gefahr der Dekompensation mit zunehmender Rigidität. Nur die Minderheit der Kinder entwickelt eine Schmerzsymptomatik ( more...

Background Context In several studies, vertical expandable prosthetic titanium rib (VEPTR) implants have shown good scoliosis control in children with the longest reported follow-up of 3.6 years. For growing rods, recent studies suggest a decreased efficiency of correction starting just after that time. To our knowledge, no long-term results of children with VEPTR treatment are available. Purpose This study aimed to evaluate spinal deformity in scoliotic children and to investigate correction potential of VEPTR implants at several time points of treatment, particularly after long-term follow-up. Study Design/Setting We performed a retrospective case series of 32 children with spinal deformity and VEPTR treatment with analysis of clinical and radiological data pre- and post-VEPTR implantation and every 2 years during the follow-up period. Patient Sample Thirty-two patients with spinal deformity and VEPTR treatment comprised the patient sample. Outcome Measures Patients had a primary VEPTR implantation due to spinal deformity and thoracic insufficiency syndrome and repeated lengthening procedures every 6 months. Clinical data were assessed and radiological parameters were analyzed. The main thoracic scoliotic curve and associated curves as well as kyphosis, lordosis, pelvic obliquity, and spinal length were measured in all radiographs until the end of VEPTR treatment or the last available examination. Methods Development of the different parameters during follow-up was evaluated and statistical analysis was performed with Statistica version 13.0. No funding was obtained for this study. The authors have no conflicts of interest to declare. Results Directly after VEPTR implantation, thoracic and lumbar curves corrected significantly, were stable at 2.8-year follow-up, and increased at 5.5-year follow-up, whereas cervical scoliosis was not affected by the treatment. The sagittal profile was initially improved both in kyphosis and lordosis. However, at 5.5-year follow-up, hyperkyphosis had deteriorated beyond the initial deformity. Pelvic obliquity was significantly restored especially in neuromuscular patients, and increasing spinal length was achieved within the 5.5-year follow-up. Conclusion In children with spinal deformity, implantation of the VEPTR device sufficiently corrected the deformity in all planes. During long-term follow-up, scoliosis increased slightly and was rather well controlled, whereas the implant system was not able to prevent deterioration of hyperkyphosis. Pelvic obliquity was well balanced and spinal lengthening was achieved during long-term follow-up. more...

BACKGROUND Almost all children with spinal muscular atrophy (SMA) develop a scoliosis during childhood and adolescence. In the last decades, growth-friendly spinal implants have been established as an interim solution for these patients until definite spinal fusion can be performed. The effect of those implants on the final outcome has yet to be described. OBJECTIVE To assess the effect of prior growth-friendly spinal surgical treatment on the outcome after spinal fusion in SMA children in comparison to untreated SMA patients through the prospective study. METHODS A total of 28 SMA patients with (n = 14) and without (n = 14) prior surgical treatment with growth-friendly implants were included. Average surgical treatment prior to definite spinal fusion was 4.9 yr. Scoliotic curve angle, pelvic obliquity, spinal length, kyphosis, and lordosis were evaluated for children with prior treatment and before and after dorsal spondylodesis for all children. RESULTS The curve angle before definite spinal fusion averaged at 104° for SMA patients without prior treatment and 71° for patients with prior treatment. Spondylodesis reduced the scoliotic curve to 50° and 33°, respectively, which equals a correction of 52% vs 54%. Pelvic obliquity could be improved by spinal fusion in all patients with better results in the pretreated group. Results for spinal length, kyphosis, and lordosis were similar in both groups. CONCLUSION These data show the positive effect of prior growth-friendly surgical treatment on radiographic results of spinal fusion in children with SMA. Both scoliotic curve angles and pelvic obliquity showed significantly better values when patients had growth-friendly implants before definite spinal fusion. more...

Cohen syndrome (CS) is a rare autosomal recessive disorder associated with mutations in the vacuolar protein sorting 13 homolog B (VPS13B; formerly COH1) gene. The core clinical phenotype comprises a characteristic facial gestalt, marked developmental delay, and myopia. Additional, nonobligatory features include obesity, microcephaly, short stature, muscular hypotonia, scoliosis, narrow hands and feet, progressive retinopathy, as well as neutropenia. Here we report a novel homozygous nonsense mutation in the VPS13B gene and previously undescribed clinical features in a 19-year-old woman with developmental delay, intellectual disability, and a particular facial appearance. The patient showed several features consistent with CS. In addition, the parents observed congenital alacrima and anhidrosis persisting until onset of puberty. The diagnosis was not established based on the clinical phenotype. We performed whole-genome sequencing and identified a novel homozygous nonsense mutation c.62T>G (NM_152564.4), p.(Leu21*) in the VPS13B gene. Our findings extended the previously reported phenotype of CS. We conclude that transient, prepubertal alacrima and anhidrosis are part of the phenotypic spectrum of CS associated with a novel homozygous nonsense mutation in the VPS13B gene. peerReviewed more...

ZusammenfassungAngeborene vaskuläre Malformationen sind häufig mit Problemen des muskuloskeletalen Systems vergesellschaftet. Stimulatives Wachstum führt zu Hypertrophien und Mehrwachstum, wie dies beispielsweise beim Klippel-Trenaunay-Syndrom mit einer zunehmenden Beinlängendifferenz vorkommt. Eine frühzeitige engmaschige klinische und radiologische kinderorthopädische Betreuung ist nötig, um rechtzeitig konservative oder operative Maßnahmen durchzuführen. Gravierende Beinlängendifferenzen im Wachstumsalter können in der Folge zu Skoliosen, aber auch rigiden Kontrakturen führen. Nach entsprechender Beobachtung und Planung sind wachstumshemmende operative Maßnahmen wie beispielsweise eine temporäre Epiphyseodese aufwändigeren verlängernden Verfahren oder Operationen nach Wachstumsabschluss vorzuziehen. Im Vorfeld einer Operation müssen zur Vermeidung von Komplikationen sorgfältige interdisziplinäre Abklärungen und Interventionen erfolgen beispielsweise zur Behandlung von Koagulopathien oder vorbereitenden Embolisationen im Operationsgebiet. more...

The objective of this study was to evaluate the presence of Aspergillus section Flavi and A. section Nigri in cashew nuts harvested in the Northern Guinea (NG) and Southern Sudanian (SS) agro-ecological zones of Benin. Also, the presence of aflatoxins was investigated. For detection of fungal contamination, a total of 100 kernels/sample (with disinfection) and 40 kernels/sample (without disinfection) were plated. Seventy samples from fourteen villages were used. Aflatoxins occurrence was analysed on 84 samples by ultraehigh performance liquid chromatography tandem mass spectrometry (UHPLC-MS/MS). The average water content and the cashew nuts count were respectively 8.6% and 172 nuts/kg in NG and 8.7% and 174 nuts/kg in SS. Significant differences between villages in both zones were found for both water content and nuts count. In disinfected samples, strains of Aspergillus section Nigri were predominant, in NG and SS zones (90.2% and 87.2%) respectively. When non disinfected kernels were plated, A. section Nigri was predominant in both NG and SS zones, with percentages of 89.7% and 93.4%, respectively. None of the 84 nuts samples were positive for natural occurrence of aflatoxins with a detection limit of 0.05 e0.2 mg/kg. more...

It is understood that an effective brace therapy requires a primary curve angle reduction of 50% after administering the first orthotic brace.The aim of the study was to determine the efficacy of conservative brace therapy for scoliosis with a curve angle above 20° and to determine possible influencing factors.The current study included a cohort of 110 scoliosis patients with conservative brace therapy. The development of the scoliotic curve during brace therapy was documented for an average of 40 months. Influencing factors such as the initial Risser sign, age at the start of treatment, gender, curve patterns and body mass index were analyzed.The collective consisted of 88 patients with idiopathic and 22 with neuromuscular spinal deformities. At the beginning of the brace therapy, the average age was 12.2 ± 2.8 years with a mean scoliosis curve angle of 30.4° ± 12.5°. The primary brace reduced the scoliotic curve by 31% to 20.9°. In children and adolescents with lower maturity status, the success of the brace therapy was greater than in patients with a higher Risser sign. In addition, children with obesity had less success during brace therapy than normal- or underweight children.The initial curvature correction of 50% required for effective brace therapy could only be achieved in one third of the patients. On average, the correction was 31%. more...

Eighty and sixty maize fields were sampled in 1994 and 1995, respectively, to monitor Aspergillus infection and aflatoxin contamination of preharvest maize in Benin. Three Aspergillus species were isolated from different agroecological zones, with A. flavus being the most prevalent. The countrywide mean percentage of kernel infection was about 20% in both years. Aflatoxin was extracted from maize in at least 30% of the fields sampled. Toxin concentrations exhibited a distinct zonal variation, with relatively high levels in the Guinea Savanna. There was a trend toward higher rate of aflatoxin accumulation per percentage A. flavus infection from the south to the north. Damage by the ear borer, Mussidia nigrivenella, increased aflatoxin accumulation in maize. Hence, the geographic pattern observed in the occurrence of A. flavus and aflatoxin may be related to the incidence of M. nigrivenella. more...

OBJECTIVEDuring childhood, early-onset scoliosis (EOS) may show severe progressive deformity, which consequently leads to aggressive treatment strategies, such as serial casting, long-term bracing, or surgical interventions. The latter usually includes repeated surgeries for implant lengthening every 6 months in order to allow sufficient growth of the thorax and spine. In 2011, the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24) was introduced to measure health-related quality of life for this patient group and their families. Since then, cross-culturally adapted versions of the EOSQ-24 have been published in Spanish, Turkish, traditional Chinese, and Norwegian. The purpose of the study was to transculturally adapt the original English version of the EOSQ-24 into the German language and evaluate the reliability of the German version.METHODSAfter adaptation and forward/backward translation, the German version of the EOSQ-24 was given to the parents or caregivers of 67 EOS patients (33 male, 34 female) Data quality was evaluated by mean, standard deviation, percentage of data missing, and extent of ceiling and floor effects. Reliability was estimated by internal consistency using Cronbach α and item-total correlations.RESULTSIn the study group (n = 67), 12 children were either observed (n = 7) or treated with a brace (n = 5). The other 55 patients were treated surgically with growth-friendly implants. The item response to the German EOSQ-24 was high with a minimum of missing data (1.7%). All items showed very good to excellent internal consistencies (0.879–0.903). Floor effects for the 24 items were between 0% and 31% and ceiling effects between 9% and 78%. The calculated Cronbach α for the 24-item scale was 0.9003, indicating excellent reliability.CONCLUSIONSThe German adaptation of the EOSQ-24 shows excellent reliability and therefore is a valid tool to measure objective health-related quality of life in children with EOS. more...

Reduction and retention of the scoliotic curve in children with progressive spinal deformities.Progressive neuromyopathic scoliosis which cannot be controlled conservatively (especially by walking disability), and/or development of a thorax insufficiency syndrome (TIS).Insufficient soft tissue coverage; body weight 11.4 kg; body mass index (BMI) 25 or50 kg; missing osseous anchoring structures (ribs); adult skeleton (usually age 12 years at surgery); severe spasticity.Indirect correction and distraction of the spinal deformity by two extendable, paravertebral telescopic implants, anchored to the cranial ribs and the iliac crest; the spine is not compromised surgically.Early functional therapy, no brace; multiple surgical (VEPTR®-system) or externally (magnetically controlled rods) controlled extensions per year.The surgical paravertebral "no-touch" technique for spine correction is particularly suitable for children with neuromyopathic scoliosis with a body weight 11.4 kg. Our prospective group of children (n = 45), was treated with a combination of the classic vertical expandable prosthetic titanium rib (VEPTR®) anchored to the ribs and iliac crest combined with a magnetically controlled telescopic implant (MAGEC®). The primary correction of50% was achieved, while progression was effectively prevented over years. In 495 outpatient lengthening procedures, the rate of implant-associated complications requiring surgery was 3.7%. Of the 45 children, 13 (29%) underwent surgical revision. With the proposed surgical "no-touch" technique for scoliosis correction of pediatric neuromyopathic deformities, an effective reduction of the scoliotic curve can be achieved and maintained. Advantages of the method are a partial retention of spinal flexibility and a reduction of spinal ossifications, which facilitates dorsal spondylodesis as the final treatment. more...

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.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.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 by50 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.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.Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence. more...

Background The impact of backpack carrying on body postural changes in schoolchildren has been discussed intensively. However, findings are inconsistent mainly due to the lack of long-term results. Objective The purpose of this investigation was to study the influence of backpack weight on body posture and prevalence of back pain. Methods In our prospective, comparative study, we investigated the influence of backpack weight on 77 schoolchildren (average age 10.8 ± 0.7 years) over a 1-year period. Participants were evaluated at the beginning of the study by clinical examination and formal static posture analysis. Forty-nine students comprised the control group while 28 students in the intervention cohort had the weight of their backpacks reduced by 2 kg. All students were reevaluated after 6 and 12 months by formal postural examination. Results Children with reduced backpack weights had a significantly improved bending posture when wearing their backpacks when compared to the participants of the control group, whereas the analysis of the children while they did not wear their backpacks revealed no difference between the groups. Within the follow-up period all postural changes were reversible once the backpack weight was removed. Conclusions Schoolchildren with heavy backpack loads show postural changes while carrying their backpack but this impact was reversible when backpacks were removed. Continuous reduction of backpack loads minimizes prevalence of back pain. more...

In pediatric flat foot a differentiation has to be made between the flexible and the rigid form. The diagnosis is based on the history, clinical examination as well as pedobarography, gait analysis and imaging techniques. It is important to rule out neuropediatric conditions such as muscular dystrophies, Ehlers-Danlos- or Marfan syndrome. In children six years of age and younger a flexible flat foot is nearly always physiological (97% of all 19 months old children). Up to the age of ten years the medial column of the foot is developing. Only a minority of children (4% in ten year olds) has a persistent or progressive deformity. Beyond to age of ten there is a danger of deformity decompensation as well as an increased rigidity. Only a minority of children develops some pain ( 2%). A clear risk factor for persistent pediatric flat foot is obesity (62% of six year old children with flat foot are obese). Pathogenetic factors include muscular, bony or soft tissue conditions. However, there specific rule is still unclear. Prevention consists in a thorough parent information about the normal development as well as encouragement of regular sportive activities. Soft and large enough shoes should be carried as a protection. Barfoot walking has to be encouraged on uneven grounds. If physiotherapy is needed different methods can be applied. Orthosis treatment should include a proprioceptive approach. Surgical interventions in children are rare. If surgical treatment is planned a detailed algorhythm should be used before utilizing one of the many different surgical methods.Der kindliche Knick-Senk-Fuß ist eine 3-dimensionale Fußfehlstellung mit vermehrter Valgusstellung der Ferse, Abflachung der medialen Längswölbung und Abduktion des Vorfußes. Man unterscheidet die flexible von der kontrakten (rigiden) Form. Die Diagnostik gliedert sich in Anamnese, klinische Untersuchung, apparative Diagnostik wie Pedobarografie und die Ganganalyse sowie bildgebende Verfahren. Wichtig ist die Einbeziehung in ein Therapieschema von eventuell vorliegenden pädiatrischen oder neuropädiatrischen Erkrankungen (z. B. Muskeldystrophien, Ehlers-Danlos- oder Marfan-Syndrom u. Ä.). Es zeigt sich, dass bei Kindern ≤ 6 Jahre der flexible Knick-Senk-Fuß fast ausschließlich physiologisch vorkommt (bei 97% aller 19 Monate alten Kinder). Bis zum Alter von 10 Jahren bildet sich die mediale Fußlängswölbung zunehmend aus. Bei einem kleinen Anteil (10-jährige Kinder 4%) persistiert der Knick-Senk-Fuß oder wird progredient. Oberhalb von 10 Jahren besteht die Gefahr der Dekompensation mit zunehmender Rigidität. Nur die Minderheit der Kinder entwickelt eine Schmerzsymptomatik ( 2%). Ein Risikofaktor zur Persistenz des Knick-Senk-Fußes stellt das Übergewicht im Kindesalter dar (62% der 6-Jährigen mit Knick-Senk-Fuß sind übergewichtig). Pathogenetische Faktoren können muskulär, knöchern und bindegewebig sind, wobei hier der Einfluss der unterschiedlichen Faktoren kontrovers diskutiert wird. Die Hauptprävention besteht in einer umfassenden Aufklärung über eine normale kindliche motorische Entwicklung. Daraus resultiert die Eigenförderung der physiologischen Entwicklung durch tägliche Bewegung, das Anstreben eines normalen Gewichtes, das Tragen von weichen Schuhen mit ausreichender Länge und Weite zum Schutz und das Barfußlaufen auf wechselnden Untergründen. Im Falle einer nötigen Intervention kann Physiotherapie inklusive manueller Techniken nötig werden. Eine Einlagen- oder Orthesenversorgung sollte bevorzugt mit einer sensomotorischen Bettung erfolgen. Operative Eingriffe sind selten. Im Falle einer geplanten operativen Intervention muss ein differenziertes Indikationsschema angewandt werden. Verschiedenste operative knöcherne und weichteilige Methoden stehen zur Verfügung. more...

Patients with psychiatric disorders of significant neurodevelopmental origin, such as schizophrenia and autism frequently experience genuine motor abnormalities, such as neurological soft signs (NSS). Previous MRI studies in patients with schizophrenia have shown that NSS are associated with abnormal cortical, thalamic and cerebellar structure and function. So far, however, no neuroimaging study focused on the role of the local gyrification index (LGI) in the pathophysiology of NSS. This study sought to explore the relationship between NSS and folding patterns of the cerebral cortex that are thought to be established during early brain development. In this study, whole brain high-resolution magnetic resonance imaging (MRI) at 3 Tesla was used to investigate the LGI in 33 patients with recent-onset schizophrenia. Cortical reconstruction was performed with the Freesurfer image analysis suite. NSS were examined on the Heidelberg Scale and related to LGI. Age, gender, years of education and medication were considered as potential confounding variables. In summary, higher NSS scores were positively associated with morphological changes of LGI predominantly in parietal and occipital areas. Our results confirm the hypothesis of a significant relationship between LGI changes and the NSS expression in schizophrenia. Investigation of LGI may help to explain subtle motor symptoms such as NSS in schizophrenia. more...

Spine-based fixation of magnetically controlled lengthening devices has been successfully performed for children with early-onset scoliosis. However, spinal manipulation may lead to ossifications, stiffness, and autofusion as previously described. To avoid these problems, a surgical technique combining bilateral externally controlled magnetic device implantation with a rib cradle and pelvic hook fixation was introduced by us in 2011. By using a bilateral single-rib or double-rib cradle fixation and a pelvic hook, the magnetic device is percutaneously inserted. The spine corrects indirectly without further manipulation. In small rib diameter or severe osteoporosis, double-rib cradles are used. Our introduced technique enables modification of the rib vertebral angle, which may be beneficial in children with spinal muscular atrophy and rib-cage deformity. This nonrandomized prospective study describes 18 children with neuromuscular scoliosis treated first by this method. All patients followed the same protocol, with expansion procedures being performed 5 months after surgery and every 3 months thereafter. Clinical, radiologic, and complication data were analyzed, showing a significant reduction in scoliosis and pelvic obliquity and an increase in spinal length, which could be maintained over a follow-up period of 1.2 years. The overall complication rate was lower than previously described, with 8%, 2 of them requiring surgery. more...

Bacterial colonization of spinal implants may cause severe complications in patients with early-onset scoliosis. Correct diagnosis and detection of microbiologic formation is crucial to prevent delayed infections caused by bacterial colonization. The purposes of this study were to estimate the rate and risk factors of colonization of vertical expandable prosthetic titanium rib (VEPTR) implants in children and to compare the different methods for detecting microbiologic formation on the spinal implants.We evaluated prospectively a group of 42 children with spinal deformities with an overall of 95 lengthening surgeries and applied different methods to detect potential bacterial colonization of VEPTR implants: swab of the implant, swab with culture of tissue, analysis of the removed lock, polymerase chain reaction (PCR), and confocal microscopy. Potential risk factors were evaluated.Of 42 patients, 17 (40%) were rated positive for bacterial colonization with Propionibacterium acnes and coagulase-negative staphylococci being the most commonly found bacteria. Risk factors for colonization were increasing age, body height, and weight. The swab with culture of removed tissue yielded most positive results, whereas direct microscopy and PCR were the least sensitive detection methods. Furthermore, commonly used infectious blood parameters were inconclusive.Although the impact of bacterial colonized implants on the health of the patients is not fully elucidated, clinicians aim for prevention of microbiologic formation on implanted devices. Therefore, reliable, inexpensive, and easy to apply diagnostic tools are indispensable to detect colonization. Based on our data, the swab together with tissue culture has the potential to become the method of choice for future diagnosis. more...