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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.

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.

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.

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.

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.

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.

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.

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.

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.

Bilateral vertical expandable prosthetic titanium rib (VEPTR) treatment using rib-to-pelvis constructs without touching the spine is a safe surgical technique to correct scoliosis while still allowing further MRI or neurosurgical interventions. In this retrospective cohort study, 4 paraplegic children with spinal deformity after intraspinal tumors and 4 children with neuromuscular diseases were compared. VEPTR treatment was able to considerably reduce the main scoliotic curve in both patient groups (41 vs. 40%). However, the tumor group constantly showed more severe curve progression over time and less favorable pelvic obliquity control. In conclusion, bilateral VEPTR can be expected to be less satisfying in children with tumors.

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.

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.

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.

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.

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.

Ellipse MAGEC rod implantation in combination with a bilateral VEPTR fixation from rib to pelvis is a sufficient method in treating flexible scoliosis in children with SMA without instrumentation of the spine.