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Purpose To investigate the potential effects of volleyball as a competitive sport in adolescence on the cartilage of knee joints using T2 mapping in MRI and identification of preclinical cartilage changes. Volleyball as an impact sport often leads to damage of the knee joint cartilage in adulthood. As T2 mapping is widely available and highly capable of detecting cartilage changes prior to conventional MRI sequences, such a detection may allow adolescent volleyball players to change their training regime before structural damage can occur to the cartilage and pose the risk of osteoarthritis. Materials and Methods Comparative study of the patellar, femoral, and tibial cartilage of 60 knee joints using T2 mapping on 3 T MRI. In each case, both knees of 15 adolescent competitive volleyball athletes were compared with 15 controls. Results In the group of competitive athletes, more focal cartilage changes were detected in the medial facet of the patellofemoral cartilage and in the medial femoral condyle of the knee joint cartilage (p = .01 and p Conclusion In adolescent volleyball players in competitive sports, T2 mapping demonstrates early cartilage changes in both the patellofemoral and medial femoral cartilages. The distribution of lesions depends on the player’s position. Since the cascade from T2 relaxation time increase to conspicuous cartilage damage is well established, early counter-regulation (e. g., adapted training profile, targeted physiotherapy, and appropriate muscle building training) has the potential to prevent later damage. Key Points: Citation Format

Objectives Depositions of linear gadolinium-based MRI contrast agents are readily visible in T1-weighted MRIs of certain brain regions in both adults and children. Macrocyclic contrast agents such as gadobutrol have so far escaped detection by qualitative MRI in children. This study aimed to assess whether there is evidence for deposition of gadobutrol in children using quantitative T1 mapping. Methods This retrospective study included patients, naive to other gadolinium-based contrast agents than gadobutrol, who had received gadobutrol as part of a clinically indicated MRI. For each patient, T1 relaxation times at 3 T were measured using single-shot T1 mapping at two time points. In each of six brain regions, age-adjusted T1 relaxation times were correlated with a number of previous gadobutrol administrations. To combine interindividual, cross-sectional effects with intraindividual, longitudinal effects, both linear mixed model and generalized additive mixed model were applied. Results One hundred four examinations of 52 children (age median 11.4, IQR 6.3–15, 26 female) with a median of 7 doses of gadobutrol in the history of their neurological or neurooncological disease were included. After correction for age and indeterminate disease-related effects to T1 time, a negative correlation of T1 time with the number of gadobutrol doses administered was observed in both mixed models in the putamen (beta − 1.65, p = .03) and globus pallidus (beta − 1.98, p = .012) Conclusions The results indicate that in children, gadobutrol is deposited in the globus pallidus and putamen. Key Points • Previous gadobutrol administration correlates with reduced T1 relaxation times in the globus pallidus and putamen in children. • This decreased T1 might be caused by gadobutrol retention within these gray-matter nuclei.

OBJECTIVES Magnetic resonance imaging (MRI) of the lungs is challenging for several reasons, mainly due to the respiratory motion, low proton density, and rapid T2* decay. Recent MR sequences with ultrashort TE (UTE) coupled with respiratory compensation promise to overcome these obstacles. So far, there are very few studies on the relevance of these sequences in children. The aim of the study was to compare the diagnostic value of a respiratory-self-gated three-dimensional UTE sequence versus a conventional respiratory-triggered T2-weighted turbo spin echo (T2-TSE) sequence in a pediatric collective. STUDY DESIGN Seventy-one patients between 0 and 18 years of age, who were scheduled for a thoracic MRI based on diverse clinical indications, were examined on a 3T MRI system. The UTE and T2-TSE sequences were evaluated by two readers regarding quality features and visualization of eight common pathology patterns. RESULTS The image quality of both sequences was equally high, with UTE depicting pleural and central bronchi more clearly. In pathologies, UTE was superior to T2-TSE for so-called "MR-negative pathologies", significant for air trapping, and in tendency for bullae and cysts. In all remaining pathologies, T2-TSE proved to be at least equivalent to UTE. CONCLUSIONS At present, UTE cannot serve as a universal replacement for conventional T2-TSE for all pathologies. It yields, however, a substantial benefit in the context of hyperinflation, emphysema, cysts, or pathologies of the bronchial system.

Lymphomerkrankungen sind im Kindesalter die dritthaufigste Neoplasie. Die Erkennung, das exakte Staging und Restaging sind wichtig fur alle Radiologen, die mit der Diagnostik von Kindern befasst sind. Magnetresonanztomographie (MRT), Positronen-Emissions-Tomographie/Computertomographie (PET/CT), CT, Ultraschall, Rontgen. Die Ganzkorperbildgebung (MRT und PET/MRT bzw. PET/CT) spielt in der Diagnostik und fur die Therapieauswahl eine zentrale Rolle beim Hodgkin-Lymphom. Insbesondere die Hybridbildgebung mittels 18F‑FDG-PET erweist sich dabei als leistungsfahige Methode im Staging und Restaging. Die Standardisierung der Bildgebung und der Einschluss in Therapieoptimierungsstudien (z. B. im Rahmen der EuroNet-PHL-C2-Studie) verbessert die Diagnostik und vermindert gleichzeitig die therapiebedingten Nebenwirkungen. Beim Hodgkin-Lymphom (HD) sind Abweichungen vom vorgegebenen Diagnostikprozedere zu vermeiden. Beim klinisch sehr heterogenen Non-Hodgkin-Lymphom (NHL) sollte dagegen die Diagnostik dem aktuellen klinischen Zustand des Kindes angepasst werden. Die Rolle der Interims-PET beim NHL ist derzeit noch Gegenstand klinischer Diskussionen.

ZUSAMMENFASSUNGDie Trichterbrust ist die häufigste Form der Brustwanddeformitäten. Die Therapieentscheidung erfolgt stets gemeinsam zwischen Patient, den Eltern und dem Chirurgen in wiederholten Gesprächen. Die nicht-invasive Saugglockentherapie stellt für Kinder, welche die Kriterien für eine operative Korrektur nicht erfüllen oder eine chirurgische Therapie ablehnen, eine gute Alternative dar und birgt keine Gefahren. Seit Einführung der minimalinvasiven Trichterbrustkorrektur (MIRPE) hat die Anzahl von Patienten, die eine operative Korrektur Ihrer PE wünschen, deutlich zugenommen. Die Ergebnisse der operativen Korrektur zeigen neben einer hohen Patientenzufriedenheit mit dem kosmetischen Ergebnis auch eine verbesserte Herzleistung und Sauerstoffaufnahmekapazität. Obwohl die MIRPE in erfahrenen Händen sicher und effektiv ist, sind ernsthafte lebensbedrohliche Komplikationen möglich.Zur Behandlung der Kielbrust ist die nicht-invasive Kompressionstherapie Therapie der Wahl. Dies gilt besonders für die dynamische Kompressionstherapie, welche eine objektive Messung und Einstellung der notwendigen Therapiedrücke, ein laterales Ausweichen der Thoraxwand und individuelle Anpassung ermöglicht. Der Therapieerfolg der Kompressionstherapie liegt „in der Hand des Patienten“. Eine operative Korrektur sollte nur nach nicht erfolgreicher Kompressionstherapie oder bei unbedingtem Patientenwunsch erfolgen.

ZUSAMMENFASSUNGZu den angeborenen Lungenfehlbildungen gehören neben den häufigen kongenitalen pulmonalen Atemwegsmalformationen (CPAM), bronchopulmonale Sequester (BPS), kongenitale lobäre Emphyseme (CLE) und bronchogene Zysten. Meist liegen isolierte Befunde vor. Mehr als 75 % der Kinder werden pränatal diagnostiziert. Anhand von Lokalisation und Gefäßversorgung in der Bildgebung sowie des prä- bzw. postnatalen Verlaufs werden die Eltern beraten. Gemeinsam ist den Lungenfehlbildungen eine Begünstigung bronchopulmonaler Infekte. Auch maligne Entartungen sind beschrieben, ein Kausalzusammenhang ist bisher nicht hinreichend belegt. Selten zeigen die Kinder schwere prä- bzw. postnatale Verläufe, die einer akuten Therapie bedürfen. Ansonsten erfolgt um den 3. Lebensmonat bzw. nach Diagnosestellung eine Schnittbildgebung. Hier gewinnt das Thorax-MRT zunehmend an Bedeutung. Spontanregressionen sind selten (< 10 %). Symptomatische Läsionen werden thorakoskopisch oder offen chirurgisch entfernt. Bei asymptomatischen Läsionen müssen die Risiken rezidivierender Infektionen sowie einer malignen Entartung gegen Komplikationen einer OP abgewogen werden. Die Langzeitprognose ist bei adäquater Behandlung gut, die Lungenfunktion in der Mehrzahl der Fälle unbeeinträchtigt.

Detection of cochlear nerve deficiency (CND) is usually straightforward using magnetic resonance imaging (MRI). In patients in whom MRI cannot be performed or imaging provides equivocal findings, computed tomography (CT) of the temporal bone might offer indirect evidence of CND. Our study aimed to derive a cut-off value for the diameter of the cochlear nerve canal (CNC) and internal auditory canal (IAC) in temporal bone CT to predict CND.This retrospective study included 70 children with sensorineural hearing loss (32 with CND and 38 control patients). The height, width, and cross-sectional area of the IAC and diameter of the CNCs were determined using temporal bone CT. Receiver operating characteristic (ROC) and Student's t-tests were performed for each parameter.The mean diameter of the CNCs was significantly smaller in children with CND than in the control group (1.2 mm versus 2.4 mm, p .001). The optimal threshold for CNC for separation of the two groups was 1.9 mm, resulting in a sensitivity of 98.7 % and specificity of 89.2 %. The IAC dimensions could not distinguish between children with CND and controls.A CNC diameter of less than 1.9 mm is a reliable predictor of CND in children with sensorineural hearing loss.· A small cochlear nerve canal predicts cochlear nerve deficiency (CND). · The size of the internal auditory canal cannot predict CND. · Whenever MRI is impossible or ambigous, CT can rule out CND.· Sorge M, Sorge I, Pirlich M et al. Diameter of the Cochlear Nerve Canal predicts Cochlear Nerve Deficiency in Children with Sensorineural Hearing Loss. Fortschr Röntgenstr 2022; 194: 1132 - 1139.ZIEL: Eine Nervus-Cochlearis-Defizienz (CND) kann mittels Magnetresonanztomografie (MRT) in der Regel einfach diagnostiziert werden. Bei Patienten, bei denen eine MRT kontraindiziert ist oder keine eindeutigen Ergebnisse erbringt, kann die Computertomografie (CT) des Schläfenbeins einen indirekten Nachweis der CND liefern. Ziel unserer Studie war es, einen Schwellenwert für den Durchmesser des Canalis nervi cochlearis (CNC) und des inneren Gehörgangs (IAC) im Schläfenbein-CT für die Vorhersage einer CND zu ermitteln. An der retrospektiven Studie nahmen 70 Kinder mit Innenohrschwerhörigkeit teil (32 mit CND und 38 Kontrollpatienten). Die Höhe, Breite und Querschnittsfläche der IAC und der Durchmesser der CNC wurden mittels Schläfenbein-CT bestimmt. Für jeden Parameter wurde eine Receiver Operating Characteristic (ROC) und ein Studentʼs t-Tests durchgeführt. Der mittlere Durchmesser der CNCs war bei Kindern mit CND deutlich kleiner als in der Kontrollgruppe (1,2 mm gegenüber 2,4 mm, p .001). Der optimale Schwellenwert zur Unterscheidung der beiden Gruppen lag für den CNC bei 1,9 mm mit einer Sensitivität von 98,7 % und einer Spezifität von 89,2 %. Die Dimensionen des IAC waren für die Unterscheidung zwischen Kindern mit und ohne CND nicht geeignet. Ein CNC-Durchmesser unter 1,9 mm ist ein zuverlässiger Prädiktor für CND bei Kindern mit sensorineuralem Hörverlust. · Ein kleiner Canalis nervi cochlearis ist ein exzellenter Prädiktor einer Nervus-Cochlearis-Defizienz (CND). · Die Größe des inneren Gehörgangs hingegen ist kein guter Prädiktor. · Wenn eine MRT kontraindiziert ist, kann die CT eine CND ausschließen.

Neurofibromatosis type 1 (NF1) is a tumor predisposition syndrome and is one of the most common genetic diseases. It is therefore a condition encountered by radiologists in clinical routine. Since the variability of the clinical expression is very high and several organ systems are affected, we present a standardized diagnostic approach in this article.Evaluation of the literature on neurofibromatosis type 1 in the context of radiological examination methods.In addition to the frequently known changes in the central and peripheral nervous system such as optic gliomas and plexiform neurofibromas, lesions from the orthopedic spectrum and vascular changes must also be included in the radiological diagnosis.Due to the diversity of the clinical picture of NF1, it is reasonable to define an examination strategy which takes into account the needs of radiological routine and also reliably detects the most frequent and prognostically significant pathologies accompanying this disease. In this article, the current recommendations for diagnosis of neurofibromatosis-associated tumors and skeletal changes are summarized, and examination protocols and time intervals are suggested.HINTERGRUND: Die Neurofibromatose Typ 1 (NF1) ist ein Tumorprädispositionssyndrom und die häufigste autosomal dominant vererbte Erkrankung. Daher ist sie ein im klinischen Alltag des Radiologen anzutreffendes Krankheitsbild. Da die Variabilität der klinischen Ausprägung sehr groß ist und mehrere Organsysteme betroffen sind, soll in diesem Artikel eine standardisierte diagnostische Herangehensweise präsentiert werden.Auswertung der Literatur zur Neurofibromatose Typ 1 im Kontext mit den radiologischen Untersuchungsmethoden.Neben den häufig bekannten Veränderungen im zentralen und peripheren Nervensystem wie dem Optikusgliom und den plexiformen Neurofibromen müssen auch Läsionen aus dem orthopädischen Spektrum und Gefäßveränderungen in die radiologische Ausbreitungsdiagnostik einbezogen werden.Aufgrund der Diversität des Krankheitsbilds der NF1 ist es sinnvoll, eine Untersuchungsstrategie zu formulieren, die einerseits den Bedürfnissen der radiologischen Routine Rechnung trägt und andererseits die häufigsten und prognostisch bedeutsamsten Pathologien dieser Erkrankung sicher erfasst. In der vorliegenden Arbeit werden die aktuellen Empfehlungen zur Diagnostik der Neurofibromatose-assoziierten Tumoren wie auch der orthopädisch skeletalen Veränderungen zusammengefasst und Vorschläge zu den Untersuchungsprotokollen sowie den Zeitintervallen unterbreitet.

Cystic fibrosis (CF) is one of the most common inherited and life-shortening pulmonary diseases in the Caucasian population. With the widespread introduction of newborn screening and the development of modulator therapy, tremendous advances have been made in recent years both in diagnosis and therapy. Since paediatric CF patients tend to be younger and have lower morbidity, the type of imaging modality that should be used to monitor the disease is often debated. Computed tomography (CT) is sensitive to many pulmonary pathologies, but radiation exposure limits its use, especially in children and adolescents. Conventional pulmonary magnetic resonance imaging (MRI) is a valid alternative to CT and, in most cases, provides sufficient information to guide treatment. Given the expected widespread availability of sequences with ultra-short echo times, there will be even fewer reasons to perform CT for follow-up of patients with CF. This review aims to provide an overview of the process and results of monitoring CF with MRI, particularly for centres not specialising in the disease.

Background Quantitative mapping of MRI relaxation times is expected to uncover pathological processes in the brain more subtly than standard MRI techniques with weighted contrasts. So far, however, most mapping techniques suffer from a long measuring time, low spatial resolution or even sensitivity to magnetic field inhomogeneity. Objective To obtain T1 relaxation times of the normal brain from early infancy to adulthood using a novel technique for fast and accurate T1 mapping at high spatial resolution. Materials and methods We performed whole-brain T1 mapping within less than 3 min in 100 patients between 2 months and 18 years of age with normal brain at a field strength of 3 T. We analyzed T1 relaxation times in several gray-matter nuclei and white matter. Subsequently, we derived regression equations for mean value and confidence interval. Results T1 relaxation times of the pediatric brain rapidly decrease in all regions within the first 3 years of age, followed by a significantly weaker decrease until adulthood. These characteristics are more pronounced in white matter than in deep gray matter. Conclusion Regardless of age, quantitative T1 mapping of the pediatric brain is feasible in clinical practice. Normal age-dependent values should contribute to improved discrimination of subtle intracerebral alterations.

Background Conventional MRI sequences are often affected in neuropediatric imaging by unavoidable movements. Therefore, children younger than 6 years usually have to be examined under sedation/anesthesia. A new real-time MRI technique with automatic slice advancement allows for motion-robust T2-weighted volume coverage of the whole brain within a few seconds in adults. Objective To evaluate to which extent the new volume coverage method can be used to visualize cerebrospinal fluid and reduce the need for anesthesia in children. Materials and methods We assessed 30 children ages 6 years and younger with suspected or proven hydrocephalus, hygroma or macrocephalus using volume coverage sequences with 20 slices per second in three planes. If necessary, a parent was placed in the bore together with the child for calming and gentle immobilization. We compared visualization of cerebrospinal fluid spaces and course of the shunt catheter in volume coverage sequences vs. fast spin-echo sequences. Results The clinical issue could be sufficiently assessed in all children with use of volume coverage sequences, whereas conventional fast spin-echo sequences performed moderately to poorly. Visualization of the tip of a shunt failed in 16% of volume coverage scans and 27% of turbo spin-echo scans. A subsequent examination under anesthesia was never necessary. None of the examinations had to be stopped prematurely. Conclusion The motion-robust volume coverage sequences with T2-type contrast can be used to avoid sedation of children in the evaluation of cerebrospinal fluid spaces, even in the presence of vigorous motion. For other indications and contrasts, the technique must still be evaluated.

ZusammenfassungDie Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

Lung MRI makes it possible to replace up to 90% of CT examinations with radiation-free magnetic resonance diagnostics of the lungs without suffering any diagnostic loss. The individual radiation exposure can thus be relevantly reduced. This applies in particular to children who repeatedly require sectional imaging of the lung, e.g., in tumor surveillance or in chronic lung diseases such as cystic fibrosis. In this paper we discuss various factors that favor the establishment of lung MRI in the clinical setting. Among the many sequences proposed for lung imaging, respiration-triggered T2-W turbo spin-echo (TSE) sequences have been established as a good standard for children. Additional sequences are mostly dispensable. The most important pulmonary findings are demonstrated here in the form of a detailed pictorial essay. T1-weighted gradient echo sequences with ultrashort echo time are a new option. These sequences anticipate signal loss in the lung and deliver CT-like images with high spatial resolution. When using self-gated T1-W ultrashort echo time 3-D sequences that acquire iso-voxel geometry in the sub-millimeter range, secondary reconstructions are possible. Electronic supplementary material The online version of this article (10.1007/s00247-019-04594-z) contains supplementary material, which is available to authorized users.

Zystische Raumforderungen im Abdomen stellen einen haufigen Haupt- oder Zufallsbefund im radiologischen Alltag dar, wobei die Differenzierung nicht immer trivial ist. Im Kindesalter stellen zystische abdominale Raumforderungen eine Besonderheit im Vergleich zu Erwachsenen dar, da hier das Spektrum der angeborenen Lasionen mitbedacht werden muss. Der Artikel gibt einen strukturierten Uberblick uber die haufigsten Entitaten. Die Standardmethoden in der Bildgebung des Abdomens sind in der Kinderradiologie der Ultraschall und die MRT. Durch Sichtung der Literatur wurden die wichtigsten Differentialdiagnosen mit ihren Charakteristika im Ultraschall und MRT zusammengestellt. Mit der anatomischen Zuordnung, dem Vorhandensein bzw. Fehlen von soliden Komponenten sowie dem Kontrastmittelverhalten in der MRT konnen die zystischen Raumforderungen gut differenziert und in drei Gruppen eingeteilt werden: angeborene und erworbene Zysten sowie Neoplasien.

Rapid volume coverage sequences based on real-time MRI allow for scanning of the entire brain within a few seconds. Movements of children become almost irrelevant due to the ultra-fast acquisition of 30 ms per slice. The adoption of these sequences in a real-time cranial MRI protocol (RT-cMRI) is expected to reduce the frequency of examinations requiring anesthesia in infants and toddlers. The aim of the study was to quantify the reduction in the number of anesthesia examinations in young children after the implementation of the new RT-cMRI protocol.All cMRI studies of children up to 6 years in the first 12 months after the establishment of the RT-cMRI 2019/2020 were retrospectively compared to a matched group of the same period in 2017/2018. The frequency of examinations under anesthesia vs. non-sedation examinations was analyzed. In addition, the number of follow-up examinations and the effectiveness of RT-cMRI was determined.The launch of RT-cMRI led to a significant decrease in the proportion of cMRI under anesthesia from 92 % to 55 %. Only 2 % of the RT-cMRI failed and required conventional MRI under sedation in the follow-up. The speed and ease of use of RT-cMRI increased the number of follow-up examinations from 1.3 to 1.4 examinations per child.This innovative real-time MRI examination allows a drastic reduction in the number of studies under anesthesia for suitable cranial pathologies in children under 6 years. However, cautious selection of indications as well as adjustments to the workflow in the radiological department are required.· Real-time MRI sequences are almost unaffected by patient movement. · The application of real-time cranial MRI can spare children from sedation. · Low-threshold access results in more frequent follow-up examinations.· Sorge I, Hirsch FW, Voit D et al. Decreased Need for Anesthesia during Ultra-Fast Cranial MRI in Young Children: One-Year Summary. Fortschr Röntgenstr 2022; 194: 192 - 198.ZIEL: Durch schnelle, der Echtzeit-MRT entlehnten Volume-Coverage-Sequenzen kann das gesamte Gehirn innerhalb weniger Sekunden abgebildet werden. Durch die ultraschnelle Akquisition von 30 ms pro Schicht führen selbst Makrobewegungen der Kinder nicht zu bewegungsbedingten Bildartefakten. Es ist erwartbar, dass sich mit der Implementierung dieser Sequenzen in ein Echtzeit-MRT-Protokoll (RT-cMRT) der Sedierungsbedarf bei Kleinkindern und Säuglingen verringert. Das Ziel dieser Studie war, das Ausmaß des Rückgangs an Sedierungsuntersuchungen nach Einführung eines neuen RT-cMRT-Protokolls zu quantifizieren.Es wurden retrospektiv alle cMRT-Untersuchungen eines Jahres bei Kindern unter 6 Jahren nach Einführung der RT-cMRT (2019/2020) mit einer gleichen Zeitspanne vor der Umstellung (2017/2018) verglichen. Es wurde die Häufigkeit von Untersuchungen mit oder ohne Sedierung erfasst und die Zahl an Wiederholungsuntersuchungen sowie die Effektivität der RT-cMRT bestimmt.Die Einführung der RT-cMRT ging mit einem deutlichen Rückgang des Anteils an Sedierungsuntersuchungen von 92 % auf 55 % einher. Nur 2 % der RT-cMRT-Untersuchungen waren insuffizient und benötigten im Anschluss noch eine konventionelle MRT. Durch die leichte Verfügbarkeit der RT-cMRT wurden 1,4 statt bisher nur 1,3 Untersuchungen pro Kind und somit mehr Follow-up-Untersuchungen durchgeführt.Mit innovativer Echtzeit-MRT ist es möglich, bei Säuglingen und Kleinkindern die Zahl an Sedierungen für kraniale MRT – bei geeigneten Erkrankungen – drastisch zu reduzieren. Jedoch sind hierfür neben der Auswahl geeigneter Indikationen auch Anpassungen der Arbeitsabläufe in der radiologischen Abteilung vonnöten.· Echtzeit-MRT-Sequenzen sind kaum anfällig für Patientenbewegungen.. · Der Einsatz von kranialer Echtzeit-MRT kann Kindern Sedierungen ersparen.. · Durch den niedrigschwelligen Zugang kommt es zu häufigeren Follow-up-Untersuchungen..

Aim In 2015, the revised International Pediatric Non-Hodgkin Lymphoma Staging System was published. It mentions [18F]-FDG-PET/MRI as the latest method to perform whole-body imaging. However, supporting data are pending. Our aim was to investigate the performance of whole-body [18F]-FDG-PET/MRI in pediatric non-Hodgkin lymphoma patients by using a limited number of MRI sequences. Materials and methods Ten pediatric patients with histologically proven non-Hodgkin lymphoma underwent whole-body [18F]-FDG-PET/MRI at staging. The retrospective analysis included three steps: First, [18F]-FDG-PET and MR scans were evaluated separately by a nuclear medicine physician and a pediatric radiologist. Nineteen nodal and two extranodal regions as well as six organs were checked for involvement. Second, discrepant findings were reviewed together in order to reach consensus. Third, [18F]-FDG-PET/MRI findings were correlated with the results of other clinical investigations. Results Of the 190 lymph node regions evaluated, four were rated controversial. Consensus was reached by considering metabolic, functional and morphologic information combined. Concordantly, [18F]-FDG-PET and MRI detected Waldeyer’s ring involvement in two patients whose Waldeyer’s ring was negative on clinical assessment. In four patients MRI showed pleural effusion. However, in only two of them an increased glucose metabolism as a reliable sign of pleural involvement was detectable. In six patients [18F]-FDG-PET and MRI detected skeletal lesions although bone marrow biopsy was positive in only one of them. Conclusion Despite the small number of cases evaluated, whole-body [18F]-FDG-PET turned out to be a valuable tool for staging of pediatric non-Hodgkin lymphoma.

Lymphoma is the third most common neoplasm in children. Detection, accurate staging, and restaging are important for all radiologists involved in the diagnosis of children.Magnetic resonance imaging (MRI), positron emission tomography/computed tomography (PET/CT), CT, ultrasound, X‑ray.Whole-body imaging (MRI and PET-MRI or PET-CT) play a key role in diagnostics and for therapy selection in Hodgkin lymphoma.In particular, hybrid imaging usingStandardization of imaging and inclusion in therapy studies (e.g. within the framework of the EuroNet-PHL-C2 study) improves diagnostics and simultaneously reduces therapy-related side effects.In Hodgkin lymphoma, deviations from the prescribed diagnostic procedure should be avoided. In clinically very heterogeneous non-Hodgkin lymphoma (NHL), on the other hand, the diagnostic procedure should be adapted to the actual clinical condition of the child. The role of interim PET in NHL is currently still the subject of clinical discussion.KLINISCHES/METHODISCHES PROBLEM: Lymphomerkrankungen sind im Kindesalter die dritthäufigste Neoplasie. Die Erkennung, das exakte Staging und Restaging sind wichtig für alle Radiologen, die mit der Diagnostik von Kindern befasst sind.Magnetresonanztomographie (MRT), Positronen-Emissions-Tomographie/Computertomographie (PET/CT), CT, Ultraschall, Röntgen.Die Ganzkörperbildgebung (MRT und PET/MRT bzw. PET/CT) spielt in der Diagnostik und für die Therapieauswahl eine zentrale Rolle beim Hodgkin-Lymphom. LEISTUNGSFäHIGKEIT: Insbesondere die Hybridbildgebung mittels Die Standardisierung der Bildgebung und der Einschluss in Therapieoptimierungsstudien (z. B. im Rahmen der EuroNet-PHL-C2-Studie) verbessert die Diagnostik und vermindert gleichzeitig die therapiebedingten Nebenwirkungen. EMPFEHLUNG FüR DIE PRAXIS: Beim Hodgkin-Lymphom (HD) sind Abweichungen vom vorgegebenen Diagnostikprozedere zu vermeiden. Beim klinisch sehr heterogenen Non-Hodgkin-Lymphom (NHL) sollte dagegen die Diagnostik dem aktuellen klinischen Zustand des Kindes angepasst werden. Die Rolle der Interims-PET beim NHL ist derzeit noch Gegenstand klinischer Diskussionen.

Die Kinderradiologie steht heute vor weitreichenden Veränderungen. Basismodalitäten wie Röntgen und Ultraschall werden zunehmend durch neuere, moderne Techniken ergänzt. Dieser Übersichtsartikel stellt Fortschritte in der Kinderradiologie sowie technische Innovationen vor, welche in Zukunft noch größere Bedeutung erlangen könnten. Hierzu werden CT-Dosisreduktionstechniken inklusive der Anwendung künstlicher Intelligenz sowie Fortschritte in den Gebieten der Magnetresonanztomografie und molekularen Bildgebung dargestellt. Kernaussagen

The recent development of highly undersampled radial gradient echo sequences in combination with nonlinear inverse image reconstruction now allows for MRI examinations in real time. Image acquisition times as short as 20 ms yield MRI videos with rates of up to 50 frames per second with spin density, T1- and T2-type contrast. The addition of an initial 180° inversion pulse achieves accurate T1 mapping within only 4 s. These technical advances promise specific advantages for studies of infants and young children by eliminating the need for sedation or anesthesia. Our preliminary data demonstrate new diagnostic opportunities ranging from dynamic studies of speech and swallowing processes and body movements to a rapid volumetric assessment of brain cerebrospinal fluid spaces in only few seconds. Real-time MRI of the heart and blood flow can be performed without electrocardiogram gating and under free breathing. The present findings support the idea that real-time MRI will complement existing methods by providing long-awaited diagnostic options for patients in early childhood. Major advantages are the avoidance of sedation or anesthesia and the yet unexplored potential to gain insights into arbitrary body functions.

During the outbreak of the COVID-19 pandemic, guidelines have been issued by international, national and local authorities to address management and the need for preparedness. Children with COVID-19 differ from adults in that they are less often and less severely affected. Additional precautions required in the management of children address their increased radiosensitivity, need for accompanying carers, and methods for dealing with children in a mixed adult-paediatric institution. In this guidance document, our aim is to define a pragmatic strategy for imaging children with an emphasis on proven or suspected COVID-19 cases. Children suspected of COVID-19 should not be imaged routinely. Imaging should be performed only when expected to alter patient management, depending on symptoms, preexisting conditions and clinical evolution. In order to prevent disease transmission, it is important to manage the inpatient caseload effectively by triaging children and carers outside the hospital, re-scheduling nonurgent elective procedures and managing symptomatic children and carers as COVID-19 positive until proven otherwise. Within the imaging department one should consider conducting portable examinations with COVID-19 machines or arranging dedicated COVID-19 paediatric imaging sessions and performing routine nasopharyngeal swab testing before imaging under general anaesthesia. Finally, regular personal hygiene, appropriate usage of personal protective equipment, awareness of which procedures are considered aerosol generating and information on how to best disinfect imaging machinery after examinations should be highlighted to all staff members. Electronic supplementary material The online version of this article (10.1007/s00247-020-04749-3) contains supplementary material, which is available to authorized users.

Esophageal atresia (EA) is a congenital anomaly that entails an interrupted esophagus with or without tracheoesophageal fistula (TEF). Depending on the distance of the two esophageal pouches a "short-gap" is distinguished from a "long-gap" variant. Up to 50% of newborns have additional anomalies. EA is prenatally diagnosed in 32-63% of cases. Recently, the interdisciplinary care in these children underwent substantial changes. Therefore, we summarize the current guideline of the German society of pediatric surgery for the treatment of patients with EA and distal TEF (Gross Type C). Controversies regarding the perioperative management include surgical-technical aspects, such as the thoracoscopic approach to EA, as well as general anesthesia (preoperative tracheobronchoscopy, intraoperative hypercapnia and acidosis). Moreover, postoperative complications and their management like anastomotic stricture are outlined. Despite significant improvements in the treatment of EA, there is still a relevant amount of long-term morbidity after surgical correction. This includes dysmotility of the esophagus, gastroesophageal reflux disease, recurrent respiratory infections, tracheomalacia, failure to thrive, and orthopedic complications following thoracotomy in the neonatal age. Therefore, close follow-up is mandatory to attain optimal quality of life.Die Ösophagusatresie (ÖA) ist eine angeborene Kontinuitätsunterbrechung der Speiseröhre mit oder ohne Fistel zur Trachea (Tracheoösophageale Fistel, TÖF). Je nach Abstand zwischen den beiden Ösophagusenden unterscheidet man eine „kurzstreckige“ von einer „langstreckigen“ Form. Bis zu 50% der Neugeborenen weisen weitere Anomalien auf. Eine pränatale Diagnose der ÖA gelingt in 32–63% der Fälle. Die interdisziplinäre Betreuung dieser Kinder hat sich in den letzten Jahren gewandelt. In der folgenden Arbeit wird die aktuelle S2K-Leitlinie der Deutschen Gesellschaft für Kinderchirurgie zur Behandlung der ÖA mit unterer TÖF, die etwa 90% aller Fälle ausmacht, zusammengefasst. Hierzu gehören das präoperative Management sowie operative und anästhesiologische Aspekte (u. a. Thorakoskopie vs. Thorakotomie, präoperative Tracheobronchoskopie, intraoperative Hyperkapnie und Azidose). Ferner wird das postoperative Management insbesondere von relevanten Komplikationen wie der Anastomosenstenose beschrieben. Trotz Fortschritten in der Behandlung der ÖA ist die Langzeitmorbidität mit Motilitätsstörungen des Ösophagus, gastroösophagealer Refluxkrankheit, rezidivierenden Infektionen der oberen und unteren Atemwege, Tracheomalazie, mangelhaftem Gedeihen sowie orthopädischen Problemen nach Thorakotomie weiterhin hoch. Zum Erreichen einer guten Lebensqualität ist daher eine gute interdisziplinäre Nachsorge wichtig.

Background COVID-19 is a novel coronavirus infection that can cause a severe respiratory illness and has been declared a pandemic by the World Health Organization (WHO). Because children appear to be less severely affected than adults, their imaging appearances have not been extensively reported. Objective To systematically review available literature regarding imaging findings in paediatric cases of COVID-19. Materials and methods We searched four databases (Medline, Embase, Cochrane, Google Scholar) for articles describing imaging findings in children with COVID-19. We included all modalities, age Results Twenty-two articles were included, reporting chest imaging findings in 431 children, of whom 421 (97.7%) underwent CT. Criteria for imaging were lacking. At diagnosis, 143/421 (34.0%) had a normal CT. Abnormalities were more common in the lower lobes and were predominantly unilateral. The most common imaging pattern was ground-glass opacification (159/255, 62.4%). None of the studies described lymphadenopathy, while pleural effusions were rare (three cases). Improvement at follow-up CT imaging (3–15 days later) was seen in 29/100 (29%), remained normal in 25/100 (25%) and progressed in 9/100 (9%). Conclusion CT chest findings in children with COVID-19 are frequently normal or mild. Lower lobes are predominantly affected by patchy ground-glass opacification. Appearances at follow-up remain normal or improve in the majority of children. Chest CT imaging adds little to the further management of the patient and should be reserved for severe cases or for identifying alternative diagnoses.

Zu klassischen Notfallen der Kindertraumatologie gehoren das Schadel-Hirn-Trauma, das stumpfe Bauchtrauma sowie die Frakturen der oberen und unteren Extremitat, die Fremdkorperingestion und -aspiration. Auch Bissverletzungen und Pfahlungsverletzungen sowie thermische Verletzungen sind haufig. Viele der Kinder werden initial beim Kinder- und Jugendarzt vorgestellt. Dieses Kapitel ermoglicht einen pragnanten Uberblick der Blickdiagnosen, stellt die wichtigsten diagnostischen Schritte sowie chirurgischen Behandlungen vor und bietet so eine fundierte Grundlage fur die Anschlussbetreuung der Patienten.

Neonatal hydrocephalus can arise from a multitude of disturbances, among them congenital aqueductal stenosis, myelomeningocele or posthemorrhagic complications in preterm infants. Diagnostic work-up comprises transfontanellar ultrasonography, T2 weighted MRI and clinical assessment for rare inherited syndromes. Classification of hydrocephalus and treatment guidelines is based on detailed consensus statements. The recent evidence favors catheter-based cerebrospinal fluid diversion in children below 6 months, but emerging techniques such as neuroendoscopic lavage carry the potential to lower shunt insertion rates. More long-term study results will be needed to allow for individualized, multidisciplinary decision making. This article gives an overview regarding contemporary pathophysiological concepts, the latest consensus statements and most recent technical developments.

Acrodysostosis is a very rare congenital multisystem condition characterized by skeletal dysplasia with severe brachydactyly, midfacial hypoplasia, and short stature, varying degrees of intellectual disability, and possible resistance to multiple G protein-coupled receptor signalling hormones. Two distinct subtypes are differentiated: acrodysostosis type 1 resulting from defects in protein kinase type 1-α regulatory subunit and acrodysostosis type 2 caused by mutations in phosphodiesterase 4D (PDE4D). Most cases are sporadic. We report on a rare multigenerational familial case of acrodysostosis type 2 due to a novel autosomal dominantly inherited PDE4D mutation. A 3.5-year-old boy presented with short stature, midfacial hypoplasia, severe brachydactyly, developmental delay, and behavioural problems. Laboratory investigations revealed mild thyrotropin resistance. His mother shared some characteristic features, such as midfacial hypoplasia and severe brachydactyly, but did not show short stature, intellectual disability or hormonal resistance. Genetic analysis identified the identical, novel heterozygous missense mutation of the PDE4D gene c.569C>T (p.Ser190Phe) in both patients. This case illustrates the significant phenotypic variability of acrodysostosis even within one family with identical mutations. Hence, a specific clinical diagnosis of acrodysostosis remains challenging because of great interindividual variability and a substantial overlap of the two subtypes as well as with other related Gsα-cAMP-signalling-linked disorders.

International audience; In two independent consanguineous families each with two children affected by mild intellectual disability and microcephaly, we identified two homozygous missense variants (c.119T>A [p.Met40Lys] and c.92T>A [p.Leu31His]) in TATA-box-binding-protein-associated factor 13 (TAF13). Molecular modeling suggested a pathogenic effect of both variants through disruption of the interaction between TAF13 and TAF11. These two proteins form a histone-like heterodimer that is essential for their recruitment into the general RNA polymerase II transcription factor IID (TFIID) complex. Co-immunoprecipitation in HeLa cells transfected with plasmids encoding TAF11 and TAF13 revealed that both variants indeed impaired formation of the TAF13-TAF11 heterodimer, thus confirming the protein modeling analysis. To further understand the functional role of TAF13, we performed RNA sequencing of neuroblastoma cell lines upon TAF13 knockdown. The transcriptional profile showed significant deregulation of gene expression patterns with an emphasis on genes related to neuronal and skeletal functions and those containing E-box motives in their promoters. Here, we expand the spectrum of TAF-associated phenotypes and highlight the importance of TAF13 in neuronal functions.

Significant changes can be expected in modern pediatric radiology. New imaging techniques are progressively added to basic modalities like Xrays and ultrasound. This essay summarizes recent advances and technical innovations in pediatric radiology, which are supposed to gain further importance in the future. Thus, CT dose reduction techniques including artificial intelligence as well as advances in the fields of magnetic resonance and molecular imaging are presented. KEY POINTS: · Technical innovations will lead to significant changes in pediatric radiology.. · CT dose reduction is crucial for pediatric patient collectives.. · New MR-techniques will lower the need for sedation and contrast media application.. · Functional MR-imaging might gain further importance in patients with chronic lung disease.. · Molecular imaging enables detection, characterization and quantification of molecular processes in tumors.. CITATION FORMAT: · Staatz G, Daldrup-Link HE, Herrmann J et al. From Xrays to PET/MR, and then? - Future imaging in pediatric radiology. Fortschr Röntgenstr 2019; 191: 357 - 366.Die Kinderradiologie steht heute vor weitreichenden Veränderungen. Basismodalitäten wie Röntgen und Ultraschall werden zunehmend durch neuere, moderne Techniken ergänzt. Dieser Übersichtsartikel stellt Fortschritte in der Kinderradiologie sowie technische Innovationen vor, welche in Zukunft noch größere Bedeutung erlangen könnten. Hierzu werden CT-Dosisreduktionstechniken inklusive der Anwendung künstlicher Intelligenz sowie Fortschritte in den Gebieten der Magnetresonanztomografie und molekularen Bildgebung dargestellt. KERNAUSSAGEN: · Technische Innovationen werden die Kinderradiologie signifikant verändern.. · CT-Dosisreduktion ist insbesondere in pädiatrischen Patientenkollektiven bedeutsam.. · Neue MR-Techniken werden die Notwendigkeit von Sedierung und Kontrastmittelapplikation reduzieren.. · Die funktionelle MR-Bildgebung kann bei Patienten mit chronischen Lungenerkrankungen an Bedeutung gewinnen.. · Die molekulare Bildgebung ermöglicht die Detektion, Charakterisierung und Quantifizierung molekularer Prozesse in Tumoren..

Es gibt mindestens 3 Voraussetzungen fur eine erfolgreiche Einfuhrung der Lungen-MRT in einer kinderradiologischen Einrichtung: indikationsgerechte Lungen-MRT-Untersuchungsangebote offerieren, bei misslingendem Lungen-MRT auch die CT-Option zeitnah zur Verfugung stellen und standardisierte, artefaktarme Basisprotokolle einhalten. Der Beitrag empfiehlt MRT-Protokolle und gibt praktische Tipps, um Lungen-MRT auch im klinischen Routinekontext zu etablieren. Bei indikationsadaquatem Einsatz der Lungen MRT und qualitativ tadelloser Untersuchung lassen sich weit mehr als 50 % der bisherigen Lungen-MRT-Untersuchungen - bei vergleichbarer diagnostischer Aussagekraft - ersetzen. Geeignet fur eine Lungen-MRT-Untersuchung sind Erkrankungen, die ein uberwiegend alveolares Befallsmuster aufweisen und Rundherde, sobald sie die Schichtbreite der Untersuchung uberschreiten (>4mm). Ungeeignet fur Lungen MRT sind bisher interstitielle Lungenprozesse und zystische Lungenlasionen.

Mutations in SCN2A have been associated with benign familial neonatal-infantile seizures (BFNIS) as well as infantile-onset epileptic encephalopathy, such as Ohtahara syndrome (OS). We describe a family with 3 affected individuals carrying the novel SCN2A missense variant c.1147C>G, p.Q383E affecting a residue proximal to the highly conserved selectivity filter in the P-loop of the voltage-gated sodium channel (Nav1.2). All 3 individuals presented with seizures in early infancy. However, there were striking differences in the spectrum of clinical presentations, ranging from BFNIS to OS. A change of ion selectivity of Nav1.2 is considered to be the potential pathomechanism underlying this Nav1.2 channel dysfunction. The observation of benign and severe phenotypes due to an identical mutation within one family contradicts the hypothesis of different modes of inheritance as a mandatory feature discriminating BFNIS from SCN2A encephalopathy.

Medulloblastomas are the most common central nervous system tumors in childhood. Treatment and prognosis strongly depend on histology and transcriptomic profiling. However, the proliferative potential also has prognostical value. Our study aimed to investigate correlations between histogram profiling of diffusion-weighted images and further microarchitectural features. Seven patients (age median 14.6 years, minimum 2 years, maximum 20 years; 5 male, 2 female) were included in this retrospective study. Using a Matlab-based analysis tool, histogram analysis of whole apparent diffusion coefficient (ADC) volumes was performed. ADC entropy revealed a strong inverse correlation with the expression of the proliferation marker Ki67 (r = − 0.962, p = 0.009) and with total nuclear area (r = − 0.888, p = 0.044). Furthermore, ADC percentiles, most of all ADCp90, showed significant correlations with Ki67 expression (r = 0.902, p = 0.036). Diffusion histogram profiling of medulloblastomas provides valuable in vivo information which potentially can be used for risk stratification and prognostication. First of all, entropy revealed to be the most promising imaging biomarker. However, further studies are warranted.

Attainment of local control is a cornerstone in the management of brain tumors in children. Treatment of progressive low-grade and all high-grade gliomas traditionally includes resection followed by fractionated radiotherapy (FRT) or FRT alone in neoplasms not amenable to surgical removal. While in younger children chemotherapy is typically used as a first-line non-surgical management option, FRT in such patients remains the standard of salvage therapy. Recent improvements in techniques for radiation treatment planning and delivery allow conformal and selective coverage of the target volume with a prescribed dose, sparing adjacent normal tissues to assure maximal tumor control and minimal toxicity. Implementation of novel functional and metabolic neuroimaging allows better delineation of the lesion margins and organs at risk, which also reduces the incidence of adverse effects. Further improvements of outcome after radiation treatment of pediatric gliomas may be expected with wider application of intensity-modulated radiation therapy (IMRT), proton therapy, and stereotactic techniques, including brachytherapy.

We report the case of a 3-year-old boy who presented with an upper respiratory tract infection and severe dyspnea. A chest X-ray revealed a left-sided tension pneumothorax with mediastinal shift and suspected enterothorax. After thoracic computed tomography (CT) scan, a chest tube was inserted, which drained fluid which had the same consistency and color as the one derived from the nasogastric (NG) tube. The boy underwent diagnostic laparoscopy for suspected bowel perforation, which confirmed a left-sided Bochdalek hernia with herniation of the viscera into the chest. After repositioning of the herniated organs into the abdomen, a gastric perforation was identified and repaired. This case demonstrates that the cause of a tension pneumothorax in an infant may be a rare combination of congenital diaphragmatic hernia (CDH) and perforation of a visceral hollow organ.

Attainment of local control is a cornerstone in the management of brain tumors in children. Treatment of progressive low-grade and all high-grade gliomas traditionally includes resection followed by fractionated radiotherapy (FRT) or FRT alone in neoplasms not amenable to surgical removal. While in younger children chemotherapy is typically used as a first-line non-surgical management option, FRT in such patients remains the standard of salvage therapy. Recent improvements in techniques for radiation treatment planning and delivery allow conformal and selective coverage of the target volume with a prescribed dose, sparing adjacent normal tissues to assure maximal tumor control and minimal toxicity. Implementation of novel functional and metabolic neuroimaging allows better delineation of the lesion margins and organs at risk, which also reduces the incidence of adverse effects. Further improvements of outcome after radiation treatment of pediatric gliomas may be expected with wider application of intensity-modulated radiation therapy (IMRT), proton therapy, and stereotactic techniques, including brachytherapy.

Purpose: A highly striated contrast pattern of the kidneys occasionally appears in abdominal MRI examinations of children following the administration of gadolinium. As this phenomenon is well known but has not yet been explicitly described in literature, we investigated how frequently and in which clinical context this occurred. Material and Methods: 855 abdominal MRI examinations with contrast media of 362 children between 2006 and 2014 were analysed retrospectively. Results: A striated renal parenchyma was found in a total of nine children and eleven examinations (1.3 % of examinations) and did only occur at a field strength of 3 Tesla. Of these children, seven had previously had tumors and chemotherapy. In two children there was no evidence of a previously serious condition with medications or a kidney disease. All of them had a normal renal function. Conclusion: A noticeably striated nephrogram in the later phase of an MRI examination following administration of gadolinium may appear as an incidental finding in examinations at 3 Tesla without pathological relevance. Key points: • striated nephrograms may appear at a field strength of 3 Tesla. • incidental finding without pathological relevance. Citation Format: • Strocka S, Sorge I, Ritter L et al. Striated nephrogram as an incidental finding in MRI examination of children. Fortschr Rontgenstr 2016; 188: 69 – 72

Pediatric tumors of the central nervous system composed of oligoid tumor cells showing diffuse leptomeningeal spread without a primary mass lesion seem to represent a novel tumor entity. The terms “diffuse leptomeningeal glioneural tumor” or—preferably—“disseminated oligodendroglial-like leptomeningeal tumor of childhood” (DOGLT) were proposed. Four patients were identified with clinico-neuropathologic findings compatible with DOGLT and a mean follow-up time of 54 months was determined. Seven different biopsies obtained from the four patients were histologically evaluated. Clinical course, diagnostic measures, histopathologic and radiologic features and treatment suggestions were recorded, on the basis of which diagnostic and therapeutic algorithm was proposed. Patients with DOGLT presented with hydrocephalus as first symptom, requiring neurosurgical therapy. Open arachnoid biopsy was necessary to confirm diagnosis. The oligoid cells in a desmoplastic or focally myxoid matrix showed OLIG2-, MAP2-, S-100 and rare HuC/HuD protein-immunopositivity. IDH1 (R132H)- and CD99-immunohistochemistry was negative in all patients. None of the evaluable biopsies of three patients showed chromosome 1p/19q deletion, neither as isolated nor combined allelic loss. Chemotherapy according to the SIOP-LGG 2004 standard induction and consolidation protocol resulted in complete response and partial response, respectively, in 50 % of the patients. However, after discontinuation of chemotherapy, two patients experienced tumor progression and one of them succumbed to the disease after 19 months. Radiological criteria as well as preliminary treatment results are presented after observation of four clinical cases. Prognosis and long-term clinical courses remain to be observed.