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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...

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...

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...

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...

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. 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...

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...

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...

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...

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...

Study Design. Retrospective analysis of 1328 spinal radiographs of 57 patients after vertical expandable prosthetic titanium rib (VEPTR) implantation with an average follow-up of 30 months. Objective. To evaluate the incidence, time of onset, as well as the underlying factors influencing the occurrence of ossifications in children treated with VEPTR. Summary of Background Data. Spontaneous spinal fusions and ossifications are well known in children treated with spinal implants. In theory, children with spinal deformity and VEPTR implantation are less likely to develop these complications because of either little or no implant contact to the spine. Methods. Fifty-seven patients had a primary VEPTR implantation due to spinal deformity and thoracic insufficiency syndrome and repeated lengthening procedures. The mean age at the time of primary surgery was 7.7 years, the mean duration of follow-up was 29.8 months, and the mean number of operations was 5.9. A total of 1328 spinal radiographs were analyzed with respect to the occurrence and growth of ossifications, implant migration, and other complications. Results. Overall, 24% of the patients (n = 13) had ossifications, which affected in 92% the main load sharing VEPTR implant. The fusions involved in 54% of cases the lumbar spine and in each 23% the ribs and the iliac crest. Ossifications developed in 11% of children within the first year of treatment and increased by about the same rate per annum. After 53 months, 48% of the radiographs showed some ossifications. There was a significant correlation to the stiffness of the deformity and the number of surgical procedures. Conclusion. Contrary to previous assumptions, it was shown that in children treated with the VEPTR system, 48% of children showed ossifications after 53 months. Ossifications were observed significantly more often in children with stiff deformities and after multiple surgical procedures. It is a much more common problem than previously thought. Conclusion. Level of Evidence: 3 more...

Study design Case Series. Objective To describe the post-VEPTR (vertical expandable prosthetic titanium rib) treatment changes in early-onset spinal deformity (EOSD), which may influence the final correction spondylodesis. Summary of background data The VEPTR device, originally developed for the treatment of congenital rib cage malformation, is nowadays more widely used in the treatment of EOSD. At present, only a few reports describe the possible complications that may occur with repeated lengthening procedures of the VEPTR, thereby making the final spondylodesis more complicated and less satisfactory. Methods X-rays of 5 children treated for EOSD with 2 unilateral VEPTR (each rib to rib and rib to lumbar lamina) were analyzed for curve patterns and Cobb angles before, during, and at the end of VEPTR treatment, and after the final spondylodesis. Intraoperative observations during the spondylodesis, which influenced the possibilities of the curve correction, were documented. Results All patients showed a marked decompensation of the frontal balance and a high degree of rigidity of the main curve and the compensatory curves after treatment with the VEPTR device. Because of this spontaneous autofusion of spinal segments, migration of the rib cradles and/or the laminar hook, and a change in the curve patterns, the final fusion had to be longer in all patients than the primary deformity would have intended. Conclusions If an EOSD is treated with VEPTR, the curve progression and, in particular, the development of a high thoracic hyperkyphosis or rotation of the main curve should be critically observed. Autofusion of ribs and vertebral bodies may make the final correction spondylodesis even more challenging and risky for the patient and the end result less satisfactory. more...

Mycotoxins contamination in some agricultural food commodities seriously impact human and animal health and reduce the commercial value of crops. Mycotoxins are toxic secondary metabolites produced by fungi that contaminate agricultural commodities pre- or postharvest. Africa is one of the continents where environmental, agricultural and storage conditions of food commodities are conducive of Aspergillus fungi infection and aflatoxin biosynthesis. This paper reviews the commodity-wise aetiology and contamination process of aflatoxins and evaluates the potential risk of exposure from common African foods. Possible ways of reducing risk for fungal infection and aflatoxin development that are relevant to the African context. The presented database would be useful as benchmark information for development and prioritization of future research. There is need for more investigations on food quality and safety by making available advanced advanced equipments and analytical methods as well as surveillance and awareness creation in the region. more...

Fungal infection and aflatoxin contamination was evaluated on 180 samples of dried vegetables such as okra, hot chilli, tomato, melon seeds, onion and baobab leaves from Benin, Togo and Mali collected in September to October 2006. These products are dried to preserve them for lean periods and decrease their perishability. Fungal contamination was evaluated after plating on selective media with a total of 561 fungal isolates identified, ranging from 18 in tomato and 218 in baobab leaves. Baobab leaves, followed by hot chilli and okra showed high incidence of fungal contamination compared to the other dried vegetables, while shelled melon seeds, onion leaves and dried tomato had lower levels of fungal contamination. Species of Aspergillus were dominant on all marketed dried vegetables irrespective of country. Mycotoxin assessment by Reversed-Phase High Performance Liquid Chromatography showed that only okra and hot chilli were naturally contaminated with aflatoxin B(1) and aflatoxin B(2), at concentrations of 6.0 microg/kg on okra and 3.2 microg/kg on hot pepper. This is the first time that mycotoxigenic fungi and resultant toxins were found on dried vegetable products sampled from African markets. Previous reports have mostly highlighted the risk of mycotoxin exposure from staple crops in Africa, but such risks now need to be evaluated for other products such as dried vegetables. more...

The presence of fungi, aflatoxins and fumonisin B1 in cassava and yam chips (during 28 processing and storage) were evaluated during two consecutive seasons in two agroecological zones of Benin (Northern Guinea Savannah, NGS and Sudan Savannah, SS). The Benin samples were assessed for moisture content, fungal infestation and total aflatoxin and fumonisin B1 contamination. During the two seasons, samples collected from the NGS, had moisture contents ranging from 10.0 to 14.7% in cassava chips and from 11.4 to 15.3% in yam chips. In samples from the SS, moisture content ranged from 10.1 to 14.5% and 11.1 to 14.5% in cassava and yam chips, respectively. A. flavus was the predominant fungal species. The maximum cfu/g in cassava and yam chips was 8950 and 6030, respectively. Other fungal species isolated included P. chrysogenum, M. piriformis, Phoma sorghina, F. verticillioides, R. oryzae and Nigrospora oryzae. High performance liquid chromatography analysis of both cassava and yam chips showed no contamination by either aflatoxins or fumonisin B1. more...

Consecutive outbreaks of acute aflatoxicosis in Kenya in 2004 and 2005 caused > 150 deaths. In response, the Centers for Disease Control and Prevention and the World Health Organization convened a workgroup of international experts and health officials in Geneva, Switzerland, in July 2005. After discussions concerning what is known about aflatoxins, the workgroup identified gaps in current knowledge about acute and chronic human health effects of aflatoxins, surveillance and food monitoring, analytic methods, and the efficacy of intervention strategies. The workgroup also identified public health strategies that could be integrated with current agricultural approaches to resolve gaps in current knowledge and ultimately reduce morbidity and mortality associated with the consumption of aflatoxin-contaminated food in the developing world. Four issues that warrant immediate attention were identified: a) quantify the human health impacts and the burden of disease due to aflatoxin exposure; b) compile an inventory, evaluate the efficacy, and disseminate results of ongoing intervention strategies; c) develop and augment the disease surveillance, food monitoring, laboratory, and public health response capacity of affected regions; and d) develop a response protocol that can be used in the event of an outbreak of acute aflatoxicosis. This report expands on the workgroup’s discussions concerning aflatoxin in developing countries and summarizes the findings. more...

The natural occurrence of Fusarium and fumonisin contamination was evaluated from 1999 to 2003 in both preharvest and stored maize produced by small-scale farmers in four agroecological zones of Benin. Mycological analyses revealed a predominance of both Fusarium and Aspergillus in maize samples compared to other genera. The two Fusarium species most commonly isolated from maize were Fusarium verticillioides (68%) and Fusarium proliferatum (31%). Atypical isolates of F. verticillioides with some characteristics of Fusarium andiyazi but apparently closer to F. verticillioides, because the isolates were all high fumonisin producers, were also found only on preharvest maize. Study of F. verticillioides strains showed the presence of extremely high fumonisin producers in Benin with total fumonisin levels ranging from 8240 to 16,690 mg/kg. Apart from 2002-2003, Fusarium occurrence was not significantly different from one zone to another, although a slight decrease was observed from south, humid, to north, drier. Fusarium occurrence varied somewhat from one season to another. It significantly decreased over the 6 months of storage. Widespread fumonisin occurrence in maize was observed. Most of the maize samples collected were found positive for fumonisin with levels ranging from not detected to 12 mg/kg in 1999-2000, 6.7 mg/kg in 2000-2001 and 6.1 mg/kg in 2002-2003. Fumonisin levels in maize were found to be significantly higher in the two southern zones during all the surveys. The highest mean total fumonisin level was detected in 1999-2000 in maize samples from the southern Guinea Savannah (SGS) (12 mg/kg), whereas in both 2000-2001 and 2002-2003, it was in samples from the forest mosaic savannah (FMS) (6.7 and 6.1 mg/kg, respectively). Fumonisin levels varied from one season to another and, throughout the storage time, showing a decreasing trend in each zone. However, this decrease was not significant every season. An increasing trend was observed during some seasons in the SGS and northern Guinea Savannah (NGS) zones. The results of this study emphasise that farmers and consumers, not only in Benin but also in other West African countries, should be alerted to the danger of fumonisin contamination in maize. more...

Smac/Diablo and HtrA2/Omi promote apoptosis by binding to and antagonizing IAP proteins, including the 'X chromosome-linked inhibitor of apoptosis' (XIAP). Here we show that caspase-mediated proteolysis of a limited subset of cell death substrates exposes functional Smac/Diablo-like N-termini after cleavage, which are able to bind to and antagonize XIAP. We propose that this mechanism may establish a feedforward sensitization of the apoptotic pathway and contribute to the functional redundancy of IAP antagonism. In addition, this may be particularly relevant in Alzheimer's disease since the caspase-generated C31 peptide, an established cytotoxin, acquires Smac/Diablo-like properties after apoptotic processing. more...

Titanium (Ti) surfaces with rough microtopographies enhance osteogenic differentiation, local factor production, and response to osteogenic agents in vitro and increase pullout strength of dental implants in vivo. Estrogens regulate bone formation, resorption, and remodeling in females and may be important in implant success. Here, we tested the hypothesis that estrogen modulates osteoblast response to implant surface morphology. Primary female human osteoblasts were cultured to confluence on three Ti surfaces (pretreatment, PT - R(a) 0.60 microm; sandblasted and acid-etched, SLA - R(a) 3.97 microm; and Ti plasma-sprayed, TPS - R(a) 5.21 microm) and treated for 24 h with 10(-7) or 10(-8) M 17beta-estradiol (E(2)). Cell number decreased with increasing surface roughness, but was not sensitive to E(2). Alkaline phosphatase specific activity of isolated cells and cell layer lysates was lower on rough surfaces. E(2) increased both parameters on smooth surfaces, whereas on rough surfaces, the stimulatory effect of E(2) on alkaline phosphatase was evident only when measuring cell layer lysates. Osteocalcin levels were higher in the conditioned media of cells grown on rough surfaces; E(2) had no effect in cultures on the plastic surfaces, but increased osteocalcin production on all Ti surfaces. TGF-beta1 and PGE(2) production was increased on rough surfaces, and E(2) augmented this effect in a synergistic manner; on smooth surfaces, there was no change in production with E(2). The response of osteoblasts to surface topography was modulated by E(2). On smooth surfaces, E(2) affected only alkaline phosphatase, but on rough surfaces, E(2) increased levels of osteocalcin, TGF-beta1, and PGE(2). These results show that normal adult human female osteoblasts are sensitive to surface microtopography and that E(2) can alter this response. more...

Epstein-Barr virus is associated with several human malignancies including Burkitt’s lymphoma, nasopharyngeal carcinoma, and Hodgkin’s disease (HD). To examine the effect of Epstein-Barr virus nuclear antigen 1 (EBNA-1) in the pathogenesis of HD, we transfected the gene into the HD cell line L428. EBNA-1 expression was associated with significantly enhanced CD25 expression (interleukin 2 [IL-2]-receptor α chain) in transient and stably transfected L428 cells but did not affect the expression of IL-2 receptor β and γ chains. There was no up-regulation of the B-cell activation molecules CD23, CD30, CD39, CD40, CD44, CD71, and CD54 (intercellular adhesion molecule 1) or enhanced production of IL-6, IL-10, lymphotoxin alpha, and the soluble form of CD25. Stable EBNA-1-expressing L428 cells were nontumorigenic in SCID mice but showed enhanced lymphoma development in nonobese diabetic-SCID mice compared to mock-transfected cells. more...

Erectile dysfunction (ED) is often associated with increased cardiovascular risk. There is increasing evidence suggesting that dysfunction of the vascular endothelium with reduced bioavailability of nitric oxide (NO) may be the pathogenetic link between ED and cardiovascular disease. The crucial importance of the NO-guanylatecyclase-cGMP-phosphodiesterase pathway for penile erection is mirrored by the efficacy of phosphodiesterase-5 (PDE5) inhibitors in the treatment of ED. In contrast to other currently available PDE5 inhibitors with a half-life time of about 4 h Tadalafil has a half-life time of about 17.5 h resulting in erectile responsiveness for up to 36 h after 1 single dose. Most clinical experience has been reported with on-demand use of PDE-5 inhibitors, but meanwhile several studies were able to demonstrate that Tadalafil given daily in low (2.5 and 5 mg) doses is both highly effective and well-tolerated. In three randomized, double-blind, placebo-controlled multi-center trials, various validated measures of erectile function indicated that once daily Tadalafil at doses of 2.5, 5, and 10 mg was significantly superior to placebo.In another mono-center trial, once daily Tadalafil has shown significant efficacy even after failure of on-demand treatment. In a controlled cross-over study of on-demand versus daily Tadalafil treatment, 72% of the patients preferred once daily administration, mainly because of superior and longer efficacy allowing a more spontaneous sexual life. Interestingly in a pilot study of on-demand versus chronic administration of Tadalafil for 4 weeks, only regular dosing improved several markers of endothelial function. more...

Torsional deformity in the lower extremities is well known in children and adolescents with hemiplegic cerebral palsy, however, little or nothing is known about possible differences in torsional behavior of the upper extremities. Alterations of torsion in the lower extremities influence the gait, alterations in the rotation behavior of the upper extremities, for example after fractures, lead to limited movement of the arms or to an altered movement radius. The aim of this study was to investigate whether there is a difference in humeral torsion between affected and non-affected arms in children and adolescents with hemiplegic cerebral palsy and which factors play a decisive role.A total of 33 children and adolescents with hemiplegic cerebral palsy (mean age 9.7 years, range 3-16 years) answered a questionnaire and were examined clinically, photographically and with a new ultrasound method. Severity of cerebral palsy was classified into three levels. Ultrasound measurements of humeral torsion were compared with the results from a normal collective (n=149).In approximately half (n=16, 48.5%) of the 33 children investigated, humeral torsion was directed more internally, in 17 (51.5%) children humeral torsion was directed more externally compared to the healthy side. The difference in humeral torsion between the affected and non-affected sides was significant (p0.05) in both groups. Differences in humeral torsion between the affected and non-affected sides in children with hemiplegic cerebral palsy were significantly increased (p0.00001) compared to differences in humeral torsion between left and right arms in healthy children. A correlation with the level of severity of hemiplegic cerebral palsy was observed but there was no correlation with age. Children and adolescents with external torsional deformity had a smaller range of rotation, a larger muscle force deficit of the affected upper extremity and did less sport using the affected arm than children and adolescents with internal torsional deformity.Humeral torsion deformity in children and adolescents with hemiplegic cerebral palsy was proven to exist in addition to the already well known difference in torsion of the lower extremities. Analogous to increased activity of external or internal rotator muscles codetermined by the palsy, increased external or internal humeral torsion occurs. External torsion deformity was associated with a smaller range of motion and seemed to have a greater importance for routine daily activities (e.g. muscle force, sports ability) than internal torsion deformity. more...

Inter- and intraobserver reliability and learning curve using Graf's ultrasonographic hip examination were assessed.189 participants of the basic, advanced and final courses on hip ultrasound using the Graf method (DEGUM) were asked to answer a questionnaire and to measure 34 normal and pathological sonographic hip examinations. Measurement differences and the learning curve during the different courses were evaluated.There was a significant (p = 0.001) improvement of the average angle between the basic (6.74 degrees [+/- 1.46 degrees ]) and the advanced course (6.22 degrees [+/- 1.17 degrees ]). However, there were no better results in the final course and no additional improvement. Participants had higher variations when measuring the beta-angle than the alpha-angle. A significant improvement was seen if the participants performed a large number of hip ultrasounds between the courses. Higher measurement differences were seen in pathological hip ultrasounds and in ultrasonographic examinations of poorer quality.During the educational courses on hip ultrasound using the Graf method, a high intra- and interobserver variability was seen. These findings were most prominent in measurement of the beta-angle, in pathological hip ultrasounds and in ultrasonographic hip examinations of poorer quality. The best results were achieved by participants who had performed a large number of examinations between courses. For the improvement of ultrasonographic measurements, a large number of self-performed examinations and a training in potential mistakes seem to be of great importance. more...

Rotary International with the International Institute of Tropical Agriculture (IITA) conducted an information campaign from 2000 to 2004 to increase public awareness of aflatoxin in Benin, Ghana and Togo. Key informant interviews with 2416 respondents showed poor baseline knowledge of aflatoxin and its health risks. The campaign included monitoring of aflatoxin contamination in maize grains from market stores in 38 cities and towns. Aflatoxin concentration in contaminated samples ranged from 24 to 117.5 ng g(-1) in Benin, from 0.4 to 490.6 ng g(-1) in Ghana, and from 0.7 to 108.8 ng g(-1) in Togo. The campaign significantly increased public awareness that populations were exposed to high levels of aflatoxin. The number of maize traders who were informed about the toxin increased 10.3 and 3.2 times in Togo and Benin, respectively; at least 33% more traders believed the information in each of Benin and Togo; 11.4 and 28.4% more consumers sorted out and discarded bad grains in Benin and Ghana, respectively. This paper concludes that sustained public education can help reduce aflatoxin contamination. more...

Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will inevitably develop thoracic insufficiency syndrome and curve progression with hemithorax compression without treatment. It is assumed that the concave side of such curves and their unilateral unsegmented bars do not grow. In the past early spinal fusion was performed with consecutive short thoracic spines and loss of lung volume. Little attention has been paid to lung function. These patients often suffered from lung failure and early death due to a small thorax.A new surgical technique is based on an indirect deformity correction and enlargement of the thorax due to a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The spine is not fused, thus promoting growth of the spine, the thorax and the lungs. Elongation of the implant is done every six months. Since 2002 this method has been performed on fifteen children in Basel as the first European center.Patients (mean age 6 years; 11 months to 12 years) were suffering from thoracic insufficiency syndrome due to unilateral unsegmented bars with fused ribs (n = 4), absent ribs (n = 2), bilaterally fused ribs (n = 2), hemivertebrae (n = 3) or neuromuscular scoliosis (n = 6). Doing fifteen primarily implantations and thirteen elongations there were three complications (two hook dislocations, one skin breakage). All patients improved cosmetically, functionally and radiologically which was shown on X-rays as a reduction of the Cobb angle from an average of 76 degrees (40-110 degrees ) to 55 degrees (30-67 degrees ).Expansion thoracoplasty and VEPTR implantation is a new treatment concept for children with thoracic insufficiency syndrome due to spinal deformities, which is based on distraction and expansion of the thorax thus allowing growth of the spine, the thorax and probably lungs. Presently it seems to be superior to any other method for the treatment of small children with progressive scoliosis and thoracic insufficiency syndrome. more...

Expansion thoracoplasty and vertical expandable prosthetic titanium rib (VEPTR; Synthes Spine Co., West Chester, Pennsylvania, USA) implantation is a new method for the treatment of thoracic insufficiency syndrome and congenital spinal deformity in children. The longitudinal rib implant expands the thorax and indirectly corrects spinal deformity, thus allowing spinal, thoracic and probably lung growth. VEPTR has been used since 1989 in San Antonio, USA, and was introduced to Europe in 2002. This paper describes the preliminary experience with the European patients. Fifteen children with progressive scoliosis had a VEPTR implantation at a mean age of 6 years (11 months to 12 years). Nine children had thoracic insufficiency syndrome due to unilateral unsegmented bars (n = 4), absent ribs (n = 1), hemivertebrae (n = 2) or bilateral fused ribs (n = 2). Six children had severe thoracolumbar scoliosis and pelvic obliquity due to neuromuscular scoliosis. After VEPTR implantation, families and patients reported improvement of the thoracic insufficiency syndrome and better sitting abilities in the neuromuscular patients, as well as radical cosmetic improvement. There were three complications (skin breakage, lumbar hook displacement, rib fracture) after performing fifteen primary VEPTR implantations and 13 expansion surgeries in eight patients. Our experience suggests that expansion thoracoplasty and VEPTR implantation is a safe and efficient method for the treatment of thoracic insufficiency syndrome in young children with severe scoliosis. more...

The slipped capital femoral epiphysis (SCFE) is defined as a nontraumatic epiphyseal separation and slipping of the proximal femoral epiphysis, which usually occurs during the adolescent growth spurt. Slipping of the upper femoral epiphysis may be classified as acute, chronic, and acute on chronic. The etiology of the disease is still not fully understood but seems to be multifactorial. The typical SCFE during puberty has to be differentiated from the atypical form, which may be associated with an endocrinological disorder or with its therapy. The typical SCFE may be found in male patients, with increased height and weight. It is likely that the growth rate is slightly accelerated before slippage. Obesity is often associated with a decreased femoral anteversion accounting for abnormal mechanical shear forces at the growth plate. SCFE is treated surgically. Surgical methods are administered according to the degree of disease. Because of possible alterations of blood supply to the femoral head, acute SCFE is an emergency. Following SCFE, complications such as chondrolysis and avascular necrosis are feared. more...

We compared the electromyographic (EMG) signals of lower extremity muscle groups in 10 children with hemiplegic cerebral palsy (CP) while walking barefoot and in a hinged ankle-foot orthosis (HAFO). All children had excessive plantarflexion and initial toe-contact on the affected side when walking barefoot, a typical gait pattern for hemiplegic patients. The patients walked with a physiological heel-toe gait pattern when wearing the HAFO. The peak activity of the tibialis anterior muscle was reduced by 36.1% at initial contact and loading response phase and by 57.3% just after toe-off when using a HAFO. The decrease in activity was thought to result from the change in gait pattern from a toe-gait to a heel-toe gait as well as the use of a HAFO. The HAFO also slightly decreased muscle activity in the proximal leg muscles mainly during swing phase, improved stride length, decreased cadence, improved walking speed, increased peak hip flexion, improved kinematics in loading response phase at the knee, and reduced the excessive ankle plantarflexion. more...

We reviewed 15 patients, nine girls and six boys, with chronic anterior dislocation of the radial head which was treated by ulnar osteotomy, external fixation and open reconstruction of the elbow joint but without repair of the annular ligament. Their mean age was 9.5 years (5 to 15) and the mean interval between the injury and reconstruction was 22 months (2 months to 7 years). All radial heads remained reduced at a mean follow-up of 20 months (6 months to 5 years). Normal ranges of movement for flexion, extension, pronation and supination were unchanged in 96.1% (49/51) and worse in 3.9% (2/51). Limited ranges of movement were improved in 77.8% (7/9), unchanged in 11% (1/9) and further decreased in 11% (1/9).There were two superficial pin-track infections and two cases of delayed union but with no serious complications. Reconstruction of the radiocapitellar joint is easier using external fixation since accurate correction of the ulna can be determined empirically and active functional exercises started immediately. Only patients with a radial head of normal shape were selected for treatment by this method. more...

Aflatoxins are a family of fungal toxins that are carcinogenic to man and cause immunosuppression, cancer and growth reduction in animals. We conducted a cross-sectional study among 480 children (age 9 months to 5 years) across 4 agro-ecological zones (SS, NGS, SGS and CS) in Benin and Togo to identify the effect of aflatoxin exposure on child growth and assess the pattern of exposure. Prior reports on this study [Gong, Y.Y.,Cardwell, K., Hounsa, A., Egal, S., Turner, Hall, A.J., Wild, C.P., 2002. Dietary aflatoxin exposure and impaired growth in young children from Benin and Togo: cross sectional study. British Medical Journal 325, 20-21, Gong, Y.Y., Egal, S., Hounsa, A., Turner, P.C., Hall, A.J., Cardwell, K., Wild, C.P., 2003. Determinants of aflatoxin exposure in young children from Benin and Togo, West Africa: the critical role of weaning and weaning foods. International Journal of Epidemiology, 32, 556-562] showed that aflatoxin exposure among these children is widespread (99%) and that growth faltering is associated with high blood aflatoxin-albumin adducts (AF-alb adducts), a measure of recent past exposure. The present report demonstrates that consumption of maize is an important source of aflatoxin exposure for the survey population. Higher AF-alb adducts were correlated with higher A. flavus (CFU) infestation of maize (p=0.006), higher aflatoxin contamination (ppb) of maize (p more...

The purpose of the study was to evaluate the prevalence of foot pain and foot deformities in adolescents. It was aimed to obtain information on the association between foot pain and foot deformities.A total of 2 368 adolescents (age 14.5 +/- 0.7 years) were evaluated. The frequency of foot pain was probed by using a self-reporting questionnaire. The foot deformities were evaluated during clinical examinations by school doctors.The prevalence of foot pain was 14.0 % and the prevalence of foot deformities was 13.7 %. The prevalence of pain was significantly higher in adolescents with foot deformity (17.8 %) than in persons without deformity (13.4 %), p0.05. The prevalence of a flexible flat foot was 6.2 % and the prevalence of a rigid flat foot was 0.5 %. Other deformities registered were splay foot (2.3 %) and flexible splay-flat foot (2.0). The prevalence of hallux-valgus deformity was 3.5 %. A total of 3.5 % patients were suffering from a plantar hyperkeratosis. This was significantly correlated to a high pain prevalence (the hyperkeratosis was significantly associated with a high prevalence of pain). Significant factors which were significantly associated with foot pain were foot deformity (1.4 fold) and hyperkeratosis (75 fold). Foot pain was 1.4 fold higher in children with foot deformity and 75 fold higher in feet with hyperkeratosis.The prevalence of foot pain and foot deformity in adolescent is high. Mild deformities (flexible flat foot and splay foot) are physiological variations without any association to foot pain. Pathological conditions that are associated with foot pain are the rigid flat foot, the hallux valgus and the cavus deformity. Plantar hyperkeratosis is an indicator of foot pathology. more...

Children with congenital thoracic scoliosis associated with fused ribs and unilateral unsegmented bars adjacent to convex hemivertebrae will invariably have curve progression without treatment. It is assumed that the unilateral unsegmented bars do not grow and therefore early spinal fusion has been performed in the past with consecutive short thoracic spines and loss of lung volume. A new surgical technique is based on an indirect deformity correction and enlargement of the thorax via a longitudinal implant, the vertical expandable prosthetic titanium rib (VEPTR). The length of the thoracic spine after VEPTR implantation was analyzed in 21 children with an average follow-up of 4.2 years. Eighteen children did not have any prior surgery while three had already undergone spinal fusion. Using a 3-D analysis of computed tomography, a significant growth of the concave (7.9 mm/7.1% increase per year) and convex (8.3 mm/6.4%) side of the thoracic spine was found with no significant difference between sides. Unilateral unsegmented bars showed significant growth as well. Contrary to common knowledge, we were able to demonstrate the growth of the concave side of the thoracic spine and of the unilateral unsegmented bars after expansion thoracoplasty and VEPTR implantation. more...

Children with congenital thoracic scoliosis associated with fused ribs with a unilateral unsegmented bar adjacent to convex hemivertebrae will invariably have curve progression without treatment. Surgery has been thought to have a negligible growth-inhibition effect on the thoracic spine in such patients because it has been assumed that the concave side of the curve and the unilateral unsegmented bar do not grow, but we are unaware of any conclusive studies regarding this assumption.The changes in the length of the concave and convex sides, anterior and posterior vertebral edges, posterior arch, and unilateral unsegmented bars of the thoracic spine were measured in the twenty-one children with congenital scoliosis and fused ribs after expansion thoracoplasty had been carried out with use of a vertical, expandable titanium prosthetic rib. Three of these children had undergone posterior spinal fusion previously. Measurements were made with use of a three-dimensional software program that analyzed baseline and follow-up computed tomography scans. The technique was validated through measurement of the thorax of a small female adult cadaver.The patients without spine fusion had an average age of 3.3 years at the time of the baseline computed tomography scan, and the average duration of follow-up was 4.2 years. On the average, these patients showed significant growth (p0.0001) of the concave side of the thoracic spine (an increase in length of 7.9 mm/yr, or 7.1%/yr) and the convex side (8.3 mm/yr, or 6.4%/yr) compared with the baseline lengths. There was no significant difference in the increases in length (p = 0.38) between the concave and convex sides. Eleven patients with an unsegmented bar had an average 7.3% increase in the length of the bar (p0.0001). In the three children with prior spinal fusion, the increase in length averaged only 4.6 mm/yr (3%/yr) on the concave side of the thoracic spine and 3.7 mm/yr (2.2%/yr) on the convex side; both increases were significant (p0.0001).Longitudinal growth of the thoracic spine in a normal child has been estimated to be 0.6 cm/yr between the ages of five and nine years. After expansion thoracoplasty, growth of the thoracic spine was approximately 8 mm/yr in our series of children with congenital scoliosis and fused ribs. After expansion thoracoplasty, both the concave and the convex side of the thoracic spine and unilateral unsegmented bars appeared to grow in these patients. When a thorax is already foreshortened by congenital scoliosis, control of spine deformity with expansion thoracoplasty allows growth of the thoracic spine, and it is likely that the longer thorax provides additional volume for growth of the underlying lungs with probable clinical benefit. more...

The activation of caspase-3 represents a critical step in the pathways leading to the biochemical and morphological changes that underlie apoptosis. Upon induction of apoptosis, the large (p17) and small (p12) subunits, comprising active caspase-3, are generated via proteolytic processing of a latent proenzyme dimer. Two copies of each individual subunit are generated to form an active heterotetramer. The tetrameric form of caspase-3 cleaves specific protein substrates within the cell, thereby producing the apoptotic phenotype. In contrast to the proenzyme, once activated in HeLa cells, caspase-3 is difficult to detect due to its rapid degradation. Interestingly, however, enzyme stability and therefore detection of active caspase-3 by immunoblot analysis can be restored by treatment of cells with a peptide-based caspase-3 selective inhibitor, suggesting that the active form can be stabilized through protein-inhibitor interaction. The heteromeric active enzyme complex is necessary for its stabilization by inhibitors, as expression of the large subunit alone is not stabilized by the presence of inhibitors. Our results show for the first time, that synthetic caspase inhibitors not only block caspase activity, but may also increase the stability of otherwise rapidly degraded mature caspase complexes. Consistent with these findings, experiments with a catalytically inactive mutant of caspase-3 show that rapid turnover is dependent on the activity of the mature enzyme. Furthermore, turnover of otherwise stable active site mutants of capase-3 is rescued by the presence of the active enzyme suggesting that turnover can be mediated in trans. more...

Ultrasound investigation of the hip according to Graf is performed, whenever possible, as a routine screening test for hip dysplasia and dislocation in neonates. However, in spite of screening, hip dysplasia and/or dislocation is identified in a number of children after the third month of life only. The present study presents an analysis of reasons and causes. Between August 1999 and July 2001 children aged between six months and five years were documented, in whom the diagnosis of hip dysplasia or dislocation was made, despite normal ultrasound findings at primary investigation and who required non operative or operative treatment by a specialist surgeon working in Switzerland in paediatric orthopaedics. The study included 26 children (17 girls / 9 boys). Hip dysplasia and/or dislocation was diagnosed between the age of 6 days and 41 months. Twelve children had to be excluded from the sample (n = 26), four were infants who had basic neurological disorders or multiple anomalies and there were eight children from abroad whose original documentation could not be obtained. Analysis of the remaining 14 children showed that 43% were misdiagnosed (n = 6) and a treatment error occurred in 36% (n = 5) of cases. One child presented with a teratogenic hip dislocation. In two other infants with normal primary ultrasound findings, hip dysplasia was identified radiographically once the child started to walk. Delayed diagnosis can be the result of technical errors or misinterpretation. The six patients meeting the criteria of misinterpretation can be expressed as a rate of 0.04% of all births. However, even if the disorder is correctly diagnosed, its identification and treatment may be inadequate or fail to produce the desired results. This was the case for five of the children, that is, for a rate of 0.03% of births. more...

Background: Children with congenital thoracic scoliosis associated with fused ribs with a unilateral unsegmented bar adjacent to convex hemivertebrae will invariably have curve progression without treatment. Surgery has been thought to have a negligible growth-inhibition effect on the thoracic spine in such patients because it has been assumed that the concave side of the curve and the unilateral unsegmented bar do not grow, but we are unaware of any conclusive studies regarding this assumption. Methods: The changes in the length of the concave and convex sides, anterior and posterior vertebral edges, posterior arch, and unilateral unsegmented bars of the thoracic spine were measured in the twenty-one children with congenital scoliosis and fused ribs after expansion thoracoplasty had been carried out with use of a vertical, expandable titanium prosthetic rib. Three of these children had undergone posterior spinal fusion previously. Measurements were made with use of a three-dimensional software program that analyzed baseline and follow-up computed tomography scans. The technique was validated through measurement of the thorax of a small female adult cadaver. Results: The patients without spine fusion had an average age of 3.3 years at the time of the baseline computed tomography scan, and the average duration of follow-up was 4.2 years. On the average, these patients showed significant growth (p < 0.0001) of the concave side of the thoracic spine (an increase in length of 7.9 mm/yr, or 7.1%/yr) and the convex side (8.3 mm/yr, or 6.4%/yr) compared with the baseline lengths. There was no significant difference in the increases in length (p = 0.38) between the concave and convex sides. Eleven patients with an unsegmented bar had an average 7.3% increase in the length of the bar (p < 0.0001). In the three children with prior spinal fusion, the increase in length averaged only 4.6 mm/yr (3%/yr) on the concave side of the thoracic spine and 3.7 mm/yr (2.2%/yr) on the convex side; both increases were significant (p < 0.0001). Conclusions: Longitudinal growth of the thoracic spine in a normal child has been estimated to be 0.6 cm/yr between the ages of five and nine years. After expansion thoracoplasty, growth of the thoracic spine was approximately 8 mm/yr in our series of children with congenital scoliosis and fused ribs. After expansion thoracoplasty, both the concave and the convex side of the thoracic spine and unilateral unsegmented bars appeared to grow in these patients. When a thorax is already foreshortened by congenital scoliosis, control of spine deformity with expansion thoracoplasty allows growth of the thoracic spine, and it is likely that the longer thorax provides additional volume for growth of the underlying lungs with probable clinical benefit. Level of Evidence: Therapeutic study, Level IV (case series [no, or historical, control group]). See Instructions to Authors for a complete description of levels of evidence. more...

Non-randomized prospective multicenter studies involving a large number of patients are often capable to prove effectiveness of perioperative antimicrobial prophylaxis in situations where it is not feasible to carry out highly sophisticated clinical trials due to limitations in logistical or financial resources. Furthermore, these studies tend to give a true picture of the daily work and procedures in hospitals, contrary to controlled trials in clinical research which might suffer of multitudes of restrictions and exclusions. Some non-randomized studies each involving more than 1,000 patients on antibiotic prophylaxis in colonic and biliary surgery as well as appendectomy are discussed and its usefulness demonstrated. more...

A subcutaneous, T-phenotypic anaplastic large cell lymphoma (CD30/Ki1-positive, EBV positive) was diagnosed in a HIV-infected bisexual man. Without chemotherapy the patient had a sustained long-term remission of this tumor (more than three years) after the initiation of highly active antiretroviral therapy. By PCR analysis of T-cell receptor beta gene rearrangements the tumor was found to be oligoclonal. Improvement of cellular immune function by antiretroviral therapy is the only recognizable factor which may have led to tumor remission. This hypothesis is supported by parallels to EBV associated polyclonal lymphoproliferation in allogeneic transplantat recipients where regression of lymphoma can be induced by reducing immunosuppressive therapy. more...

The anti-CD25 immunotoxin RFT5.dgA was constructed by coupling the monoclonal antibody RFT5 via a sterically hindered disulfide linker to deglycosylated ricin A-chain and was administered to patients with relapsed Hodgkin's lymphoma in four bolus infusions over 7 days (day 1, 3, 5 and 7). The maximum tolerated dose in these patients as defined in a previous phase I study was 15 mg/m2. Subsequently, further patients were enrolled at the maximum tolerated dose and a total of 18 patients were treated at this level. All patients had signs of progressive disease and were heavily pretreated. Side-effects in this trial were moderate and related to vascular leak syndrome. Five of 18 patients experienced NCI grade III toxicities including weakness, edema, dyspnea, and myalgia. Eleven of 16 (69%) patients receiving two or more cycles produced human anti-ricin antibodies and human anti-mouse antibodies (/=1.0 microg/ml). Seventeen of 18 patients were evaluable for clinical response. These included two partial remissions. One patient demonstrated minor response and five patients stable diseases. We conclude that RFT5.dgA is of moderate clinical efficacy in this group of heavily pretreated refractory patients. Leukemia (2000) 14, 129-135. more...