Low dose pediatric chest computed tomography on a photon counting detector system - initial clinical experience.

BACKGROUND: With the clinical release of a photon counting detector-based computed tomography (CT) system, the potential benefits of this new technology need to be evaluated clinically. Literature concerning this new generation of detector is sparse, especially in the field of pediatric radiology. Therefore, this study outlines our initial experience with ultra-low dose chest CT imaging on the new photon counting CT system. MATERIALS AND METHODS: A pediatric phantom (1-year old, CIRS ATOM phantom, model 704 [CIRS-computerized imaging reference system, Norfolk, VA]) was scanned at different dose levels and different image quality levels to define a protocol for clinical examinations. Next, 20 consecutive pediatric non-contrast ultra-low dose chest CT examinations were evaluated for radiation dose and diagnostic image quality using a 4-point Likert-scale-1 = excellent, 4 = bad image quality-by two radiologists in a consensus reading. This retrospective analysis was approved by the local research ethics committee. RESULTS: Chest CT examinations performed at ultra-low radiation dose (effective dose 0.19 ± 0.07 mSv; size-specific dose estimate 0.45 ± 0.14 mGy) in pediatric patients ages (2.6 ± 1.8 years) show good to excellent image quality for lung structures (1.4 ± 0.4) and moderate image quality for soft tissue structures (2.8 ± 0.2). CONCLUSION: Pediatric ultra-low dose chest CT examinations are feasible with the new generation photon counting detector-based CT system. The benefits of this technology must be evaluated for pediatric patients from the outset.

Interventional closure of a bronchopleural fistula in a 2 year old child with detachable coils.

BACKGROUND: Bronchopleural fistula (BPF) is a severe complication following pneumonia or pulmonary surgery, resulting in persistent air leakage (PAL) and pneumothorax. Surgical options include resection, coverage of the fistula by video-assisted thoracoscopic surgery (VATS), or pleurodesis. Interventional bronchoscopy is preferred in complex cases and involves the use of sclerosants, sealants and occlusive valve devices. CASE PRESENTATION: A 2.5-year-old girl was admitted to our hospital with persistent fever, cough and dyspnoea. Clinical and radiological examination revealed right-sided pneumonia and pleural effusion. The child was started on antibiotics, and the effusion was drained by pleural drainage. Following removal of the chest tube, the child developed tension pneumothorax. Despite insertion of a new drain, the air leak persisted. Thoracoscopic debridement with placement of another new drain was performed after 4 weeks, without abolishment of the air leak. Bronchoscopy with bronchography revealed a BPF in right lung segment 3 (right upper-lobe anterior bronchus). We opted for an interventional approach that was performed under general anaesthesia during repeat bronchoscopy. Following bronchographic visualisation of the fistula, a 2.7 French microcatheter was placed in right lung segment 3 (upper lobe), allowing occlusion of the fistula by successive implantation of 4 detachable high-density packing volume coils, which were placed into the fistula. Subsequent bronchography revealed no evidence of residual leakage, and the chest tube was removed 2 days later. The chest X-ray findings normalized, and follow-up over 4 years was uneventful. CONCLUSIONS: Bronchoscopic superselective occlusion of BPF using detachable high-density packing large-volume coils was a successful minimally invasive therapeutic intervention performed with minimal trauma in this child and has not been reported thus far. In our small patient, the short interventional time, localized intervention and minimal damage in the lung seemed superior to the corresponding outcomes of surgical lobectomy or pleurodesis in a young growing lung, enabling normal development of the surrounding tissue. Follow-up over 4 years did not show any side effects and was uneventful, with normal lung-function test results to date.

Surgical Therapy After Failed Feminizing Genitoplasty in Young Adults With Disorders of Sex Development: Retrospective Analysis and Review of the Literature.

BACKGROUND: Secondary vaginal stenosis may occur after reconstruction of genital malformations in childhood or after failed vaginal aplasia repair in adults. AIM: This study focusses on the results of the surgical treatment of these patients in our multidisciplinary transitional disorders/differences of sex development team of pediatric surgeons and gynecologists. METHODS: A retrospective analysis was carried out on adult and female identified disorders/differences of sex development patients with vaginal stenoses treated between 2015 and 2018 in a single center with revision vaginoplasty. The underlying type of malformation, the number and surgical techniques of vaginoplasties in infancy, techniques of revision of the stenotic vagina, vaginal length and caliber, possibility of sexual intercourse, and temporary vaginal dilatation. A review of literature with regard to recommended surgical techniques of revision vaginoplasties was accomplished. OUTCOMES: To describe the surgical technique, the main outcome measures of this study are vaginal calipers after revision vaginoplasty as well as ability for sexual intercourse. RESULTS: Thirteen patients presented with vaginal stenosis with a median age of 19 years (range 16-31). All patients had one or more different types of vaginoplasties in their medical history, with a median age at first vaginoplasty of 15 months (0-233). Underlying anatomical conditions were urogenital sinus (n = 8), vaginal agenesis (n = 2), persistent cloacae (n = 2), and cloacal exstrophy (n = 1). The main symptoms were disability of sexual intercourse in 13 patients due to stenotic vaginal tissue. The most frequently performed surgical technique was partial urogenital mobilization with a perineal or lateral flaps (n = 10), followed by bowel vaginoplasty (n = 2), in 1 patient a revision vaginoplasty failed due to special anatomical conditions. In a median follow-up of 11 months, all but one patient presented with physiological vaginal length and width, and normal sexual intercourse in those with a partnership. CLINICAL IMPLICATIONS: Perineal flap with partial urogenital mobilization should be considered as a treatment of choice in severe cases of distal vaginal stenosis and after multiple failed former vaginoplasties, while bowel vaginoplasty should be reserved only for cases of complete cicatrization or high located stenosis of the vagina. STRENGTHS & LIMITATIONS: The strength of this study is the detailed description of several cases while the retrospective character is a limitation. CONCLUSION: In patients after feminizing genital repair, perineal flap with partial urogenital mobilization provides a normal anatomical outcome and allows unproblematic sexual intercourse. Ellerkamp V, Rall KK, Schaefer J, et al. Surgical Therapy After Failed Feminizing Genitoplasty in Young Adults With Disorders of Sex Development: Retrospective Analysis and Review of the Literature. J Sex Med 2021;18:1797-1806.

A pilot study of patient satisfaction with a self-completed tablet-based digital questionnaire for collecting the patient's medical history in an emergency department.

BACKGROUND: The increasing popularity and availability of tablet computers raises questions regarding clinical scenarios. This pilot study examined the patient's satisfaction when using a tablet-based digital questionnaire as a tool for obtaining medical history in an emergency department and to what extent gender, age, technical competence and mother tongue influence the user satisfaction. Patients were asked to complete three consecutive questionnaires: The first questionnaire collected basic epidemiological data to measure past digital usage behaviour, the second questionnaire collected the patient's medical history, and the third questionnaire assessed the overall perceived user satisfaction when using the tablet-based survey application for medical anamnesis. RESULTS: Of 111 consenting patients, 86 completed all three questionnaires. In summary, the user evaluation was positive with 97.7% (n = 84) of the patients stating that they had no major difficulties using the digital questionnaire. Only 8.1% (n = 7) of patients reported a preference to fill out a paper-and-pen version on the next visit instead, while 98.8% (n = 85) stated that they would feel confident filling out a digital questionnaire on the next visit. The variables gender, age, mother tongue and/or technical competence did not exert a statistically significant influence towards the defined scales usability, content and overall impression. CONCLUSION: In conclusion, self-administered tablet-based questionnaires are widely accepted tools for collecting medical information in the emergency room across all ages and genders, regardless of technical competence.

123Iodine-metaiodobenzylguanidine scintigraphy versus whole-body magnetic resonance imaging with diffusion-weighted imaging in children with high-risk neuroblastoma - pilot study.

BACKGROUND: The prognostic value of the International Society of Paediatric Oncology European Neuroblastoma Research Network (SIOPEN) skeletal score using 123iodine-metaiodobenzylguanidine (MIBG) has been confirmed for people with high-risk neuroblastoma. Whole-body MRI with diffusion-weighted imaging is used increasingly. OBJECTIVE: To compare the original SIOPEN score and its adaption by diffusion-weighted imaging in high-risk stage 4 neuroblastoma and to evaluate any consequences of score differences on overall survival. MATERIALS AND METHODS: This retrospective observational study included pediatric patients who underwent MIBG scintigraphy and whole-body MRI, including diffusion-weighted imaging, between 2010 and 2015. Semi-quantitative skeletal scores for each exam were calculated independently. A difference of two or more points was defined as clinically relevant and counted as M+ (more in diffusion-weighted imaging) or S+ (more in MIBG). In cases of a negative result in one of the studies, residual disease of 1 point was also rated as relevant. We tested correlation and differences on an exam basis and also grouped by different therapeutic conditions. Overall survival was used to evaluate prognostic relevance. RESULTS: Seventeen children with 25 paired examinations were evaluated. Median MIBG scintigraphy score was 0 (interquartile range [IQR] 0-4, range 0-25) vs. a median whole-body MRI score of 1 (IQR 0-5.5, range 0-35) (P=0.018). A relevant difference between whole-body MRI and MIBG scintigraphy was noted in 14 of the 25 paired examinations (M+: n=9; S+: n=5). After treatment, the median survival of cases with M+ was 14 months (IQR 4-59, range 1-74 months), while S+ cases showed a median survival of 49 months (IQR 36-52, range 36-52 months) (P=0.413). CONCLUSION: The SIOPEN scoring system is feasible for whole-body MRI but might result in slightly higher scores, probably because of MRI's superior spatial resolution. Further studies are necessary to validate any impact on prognosis.

Reduction of radiation exposure in scoliosis monitoring using flat detector and pulsed fluoroscopy technology.

A new flat detector and pulsed fluoroscopy technology is available to further reduce radiation exposure in radiological monitoring during scoliosis treatment in children and adolescents. The aim of this study is to compare different settings of the system (opening area(OA) and image quality settings (IQS)) in order to find the optimal parameters with high image quality and the lowest possible radiation exposure. Therefore, we examined four cadaver spines (T1 to sacrum) with the flat detector technique using digital pulsed fluoroscopy and simulated the abdominal soft tissues. The images were merged and evaluated by three different investigators using an established scoring system. For comparison, we used digital radiography images of the cadaver spines. The values for the DAP increased from the small OA (33% ; 0.56 µGy·m²) to the maximum OA (100% ; 0.82 µGy·m²) by 45% (p = .003) and from the low image quality setting (0.57 µGy·m²) to the high setting (0.84 µGy·m²) by 48% (p = .028). Despite the low DAP, the setting 33% OA achieved the best point values for image quality, therefore this setting is clearly preferred. Using a digital fluoroscopy system allows a significant reduction of radiation exposure by a factor of 7.5 (3.88µGy·m² to 0.5µGy·m²) compared to slot- scanning x-ray (EOS). Due to this success, the flat detector and pulsed fluoroscopy technology can be an alternative to established methods such as X-ray and EOS in clinical use.

The Role of Reconstructive Surgery and Brachytherapy in Pediatric Bladder/Prostate Rhabdomyosarcoma.

PURPOSE: The surgical approach to localized bladder/prostate rhabdomyosarcoma in children may change due to a new radiotherapeutic modality. We assessed the impact of brachytherapy following surgery for local tumor control, and report surgical techniques and outcomes. MATERIALS AND METHODS: We retrospectively analyzed the records of all children who underwent surgery for bladder/prostate rhabdomyosarcoma, including tumor relapse, at our institution from 2009 onward. RESULTS: A total of 38 patients with a median age of 29 months (range 10 to 134) met inclusion criteria. Five-year overall survival was 92.8% (95% CI 72.9 to 98.1), and event-free survival at a median followup of 12 months (range 3 to 111) was 73.7% (95% CI 53.4 to 86.2). Three treatment groups were defined, ie bladder preserving surgery combined with brachytherapy, bladder preserving surgery alone and cystectomy. Five-year event-free survival rates for the 3 groups were 85.6% (95% CI 61.2 to 95.2), 66.7% (95% CI 27.2 to 88.2) and 50% (95% CI 5.8 to 84.5), respectively. Bladder preserving surgery was performed in 33 patients (87%), of whom 23 (70%) also underwent brachytherapy, while cystectomy was performed in 5 (13%). Reconstructive procedures varied depending on tumor location and spread. CONCLUSIONS: Combining brachytherapy with surgery results in a high bladder preservation rate and improves event-free survival compared to surgery alone in children with bladder/prostate rhabdomyosarcoma. The combination is also effective in treating local tumor relapse, and is associated with less extensive reconstructive procedures due to exclusion of tumors of unfavorable size and location for brachytherapy.

Forensic age estimation in living adolescents with CT imaging of the clavicula-impact of low-dose scanning on readers' confidence.

OBJECTIVES: Computed tomography (CT) imaging of the clavicula displays the reference standard for forensic bone age diagnostics in adolescents and young adults. Consequently, highest efforts on radiation reduction are warranted. Therefore, the aim of this study was to investigate the feasibility of low-dose (LD) CT imaging of the clavicula for age estimation in living adolescents. METHODS: A total of 207 non-contrast chest CT of 144 patients born between 1988 and 2012, performed in 2018 due to various clinical indications, were included in this retrospective study. The mean patient age was 16.9 ± 6.6 years. Patients were divided into a LD (n = 146) and standard-dose (SD; n = 61) group. Image quality, confidence levels, and ossification stages (using the 5-stage classification including the subgroups 2a-3c) were assessed by two radiologists independently. Radiation dose was determined via dosimetry software. RESULTS: Dose simulation with z-axis reduction to depict the clavicula only resulted in a median exposure of 0.1 mSv (IQR: 0.0) in LD compared with 0.9 mSv (IQR: 0.6) in SD (p < 0.001). The median image quality was rated by both readers significantly worse in LD compared with SD on a Likert scale ranging from 1 to 4 with a median of 3 (IQR: 1) versus 4 (IQR: 0; p < 0.001 for both readers). There was an almost perfect agreement for the ossification stages between both readers with a Cohen's kappa of 0.83 (p < 0.001). Median confidence levels of both readers were not significantly different between LD and SD in the decisive subgroups 2a-3c. CONCLUSIONS: Low-dose CT imaging of the clavicula for age estimation in living adolescents is possible without loss of readers' confidence. KEY POINTS: • Radiological bone age diagnostics in young delinquents with unknown exact chronological age is important as the judicial systems differentiate between youths and adults. • Low-dose computed tomography scanning of the medial clavicular joint for forensic age estimation is feasible in living adolescents without loss of readers' confidence. • Sufficient image quality of the medial clavicular joint for forensic bone age diagnostics in living adolescents is achievable using a median dose of 0.1 mSv.

New severity assessment in cystic fibrosis: signal intensity and lung volume compared to LCI and FEV1: preliminary results.

OBJECTIVES: Magnetic resonance imaging (MRI) aids diagnosis in cystic fibrosis (CF) but its use in quantitative severity assessment is under research. This study aims to assess changes in signal intensity (SI) and lung volumes (Vol) during functional MRI and their use as a severity assessment tool in CF patients. METHODS: The CF intra-hospital standard chest 1.5 T MRI protocol comprises of very short echo-time sequences in submaximal in- and expiration for functional information. Quantitative measurements (Vol/SI at in- and expiration, relative differences (Vol_delta/SI_delta), and cumulative histograms for normalized SI values across the expiratory lung volume) were assessed for correlation to pulmonary function: lung clearance index (LCI) and forced expiratory volume in 1 s (FEV1). RESULTS: In 49 patients (26 male, mean age 17 ± 7 years) significant correlation of Vol_delta and SI_delta (R = 0.86; p < 0.0001) during respiration was observed. Individual cumulated histograms enabled severity disease differentiation (mild, severe) to be visualized (defined by functional parameter: LCI > 10). The expiratory volume at a relative SI of 100% correlated significantly to LCI (R = 0.676 and 0.627; p < 0.0001) and FEV1 (R = - 0.847 and - 0.807; p < 0.0001). Clustering patients according to Vol_SI_100 showed that an amount of ≤ 4% was related to normal, while an amount of > 4% was associated with pathological pulmonary function values. CONCLUSION: Functional pulmonary MRI provides a radiation-free severity assessment tool and can contribute to early detection of lung impairment in CF. Lung volume with SI below 100% of the inspiratory volume represents overinflated tissue; an amount of 4% of the expiratory lung volume was a relevant turning point. KEY POINTS: • Signal intensity and lung volumes are used as potential metric parameters for lung impairment. • Quantification of trapped air impacts on therapy management. • Functional pulmonary MRI can contribute to early detection of lung impairment.

Retrospective accuracy analysis of MRI based lesion size measurement in neuroblastic tumors: which sequence should we choose?

BACKGROUND: MR imaging of neuroblastic tumors is widely used for assessing the effect of chemotherapy on tumor size. However, there are some concerns that MRI might falsely estimate lesion diameters due to calcification and fibrosis. Therefore, the aim of our study was to compare neuroblastic tumor size based on MRI measurements to histopathology measurements of the resected specimens as standard of reference. METHODS: Inclusion criteria were diagnosis of a neuroblastic tumor, MR imaging within 100 days to surgery and gross total resection without fragmentation of the tumor between 2008 and 2019. Lesion diameters were measured by two radiologists according to RECIST 1.1 in axial plane in T2w turbo spin echo (TSE), diffusion-weighted imaging (DWI), and in T1w pre- and postcontrast sequences. Furthermore, the largest lesion size in three-dimensions was noted. The largest diameter of histopathology measurements of each specimen was used for comparison with MRI. RESULTS: Thirty-seven patients (mean age: 5 ± 4 years) with 38 lesions (neuroblastoma: n = 17; ganglioneuroblastoma: n = 11; ganglioneuroma: n = 10) were included in this retrospective study. There was excellent intra-class correlation coefficient between both readers for all sequences (> 0.9) Tumor dimensions of reader 1 based on axial MRI measurements were significantly smaller with the following median differences (cm): T1w precontrast - 1.4 (interquartile range (IQR): 1.8), T1w postcontrast - 1.0 (IQR: 1.9), T2w TSE: -1.0 (IQR: 1.6), and DWI -1.3 (IQR: 2.2) (p < 0.001 for all sequences). However, the evaluation revealed no significant differences between the three-dimensional measurements and histopathology measurements of the resected specimens regardless of the applied MRI sequence. CONCLUSIONS: Axial MRI based lesion size measurements are significantly smaller than histopathological measurements. However, there was no significant difference between three-dimensional measurements and histopathology measurements of the resected specimens. T2w TSE and T1w postcontrast images provided the lowest deviation and might consequently be preferred for measurements.

Allogeneic hematopoietic stem cell transplantation in two brothers with DNA ligase IV deficiency: a case report and review of the literature.

BACKGROUND: DNA ligase IV deficiency is a rare autosomal recessive disorder caused by hypomorphic mutations in the DNA ligase IV (LIG4) gene. DNA ligase IV is an essential protein for the development of a healthy immune system as well as for the protection of genomic integrity. Apart from typical stigmata, patients with DNA ligase IV deficiency are characterized by progressive bone marrow failure and a predisposition to malignancy. To our knowledge this reported case is the first description of two brothers with ligase IV deficiency who are treated with different hematopoietic stem cell transplantation (HSCT) regimens resulting in vastly divergent outcomes. CASE PRESENTATION: The cases of two brothers suffering from severe recurrent infections and growth retardation are described. The laboratory findings showed pancytopenia with significant lymphopenia. The two boys were diagnosed with DNA ligase IV deficiency, associated with severe combined immunodeficiency (SCID). Both patients received HSCT from two different matched unrelated donors (MUD) at the age of 33 and 18 months. The older brother succumbed post-transplant due to fatal side-effects 143 days after allogeneic HSCT. The younger brother - conditioned with a different regimen - received a T cell depleted graft 4 months later. No severe side-effects occurred, neither post-transplant nor in the following years. Ten years after HSCT the patient is well off, living a normal life and attending a regular high school. His immune system is fully reconstituted, resulting in a maximum of T cell receptor (TCR) diversity, which is a prerequisite for immune competence. However, he still suffers from microcephaly, dwarfism and dystrophy. CONCLUSIONS: This case report gives an example of a successful HSCT as a treatment option in a genetic disorder such as ligase IV deficiency, using a rather mild conditioning regimen. Further studies are required to determine the viability and efficacy of this treatment option.

Correction to: Allogeneic hematopoietic stem cell transplantation in two brothers with DNA ligase IV deficiency: a case report and review of the literature.

After publication of the original article (1), it was brought to our attention that references 24 and 31 are inappropriately cited in the article.

A semiquantitative MRI-Score can predict loss of lung function in patients with cystic fibrosis: Preliminary results.

OBJECTIVES: To evaluate the applicability of a semiquantitative MRI scoring system (MR-CF-S) as a prognostic marker for clinical course of cystic fibrosis (CF) lung disease. METHODS: This observational study of a single-centre CF cohort included a group of 61 patients (mean age 12.9 ± 4.7 years) receiving morphological and functional pulmonary MRI, pulmonary function testing (PFT) and follow-up of 2 years. MRI was analysed by three raters using MR-CF-S. The inter-rater agreement, correlation of score categories with forced expiratory volume in 1 s (FEV1) at baseline, and the predictive value of clinical parameters, and score categories was assessed for the whole cohort and a subgroup of 40 patients with moderately impaired lung function. RESULTS: The inter-rater agreement of MR-CF-S was sufficient (mean intraclass correlation coefficient 0.92). MR-CF-S (-0.62; p < 0.05) and most of the categories significantly correlated with FEV1. Differences between patients with relevant loss of FEV1 (>3%/year) and normal course were only significant for MR-CF-S (p < 0.05) but not for clinical parameters. Centrilobular opacity (CO) was the most promising score category for prediction of a decline of FEV1 (area under curve: whole cohort 0.69; subgroup 0.86). CONCLUSIONS: MR-CF-S is promising to predict a loss of lung function. CO seems to be a particular finding in CF patients with an abnormal course. KEY POINTS: • Lung imaging is essential in the diagnostic work-up of CF patients • MRI serves as a powerful, radiation-free modality in paediatric CF patients • Observational single-centre study showed significant correlation of MR-CF score and FEV 1 • MR-CF score is promising in predicting a loss of lung function.

Multimodal lipid-lowering treatment in pediatric patients with homozygous familial hypercholesterolemia-target attainment requires further increase of intensity.

BACKGROUND: Familial hypercholesterolemia (FH) causes premature cardiovascular disease (CVD). Lipoprotein apheresis (LA) is recommended as first-line lipid-lowering treatment (LLT) for homozygous (ho) FH. METHODS: Efficacy of multimodal LLT including lifestyle counseling, drug treatment, and LA was analyzed in 17 pediatric hoFH or compound heterozygous (c-het) FH patients, who commenced chronic LA in Germany before the age of 18. RESULTS: At time of diagnosis, mean low-density lipoprotein cholesterol (LDL-C) concentration was 19.6 mmol/l (756 mg/dl). Multimodal LLT resulted in 73% reduction of mean LDL-C concentration including a 62% contribution of LA. Only three children (18%) achieved mean LDL-C concentrations below the recommended pediatric target of 3.5 mmol/l (135 mg/dl). In 13 patients (76%) during chronic LA, neither cardiovascular events occurred nor was CVD progression detected clinically or by routine imaging techniques. In four patients (24%), cardiovascular events documented progression of CVD despite weekly LA, including one death due to coronary and cerebrovascular CVD which was not stabilized after commencing LA. Based on the mutational status, only 6 out of the 17 children were candidates for proprotein convertase subtilisin-kexin type 9 (PCSK9) inhibition. Two already responded with further LDL-C decrease by 40%. CONCLUSIONS: Next to drug therapy, regular LA is an essential component of LLT for approaching LDL-C targets in children with hoFH or c-hetFH, which was successful only in a minority of children. Progression of CVD morbidity and resulting mortality remain unresolved issues. Early and intensified multimodal LLT guided by risk factors beyond LDL-C concentration is needed to improve outcome.

Fibrolipomatous hamartomas of the median nerve in infancy and early childhood-imaging hallmarks, symptomatology, and treatment.

A fibrolipomatous hamartoma (FLH) is a rare lesion leading to an enlargement of the affected nerve and commonly manifests at the median nerve. Symptomatic patients are mostly adolescents or adults. In children below 10 years, this entity is rather unknown and likely to be misdiagnosed. We report three children with FLH, two severely and one mildly symptomatic, all below 4 years of age at the time of first presentation. Two of three children were initially misdiagnosed. We provide a review of the pertinent clinical and radiological findings of the entity. Two patients had a characteristic macrodactyly. The two symptomatic children underwent surgical carpal tunnel decompression. The intervention relived their symptoms with a long-lasting effect. Surgical reduction of the hamartoma mass is not indicated and medical treatment non-existent. CONCLUSION: A symptomatic FLH of the median nerve is rare in children below the age of 5 years but has to be kept in mind as differential diagnosis in case of wrist and/or palm swelling, macrodactyly, and pain in hand or forearm. MRI is diagnostic, with very characteristic features, which can also be identified in high-resolution nerve ultrasound. This article aims to increase the knowledge about the entity including the diagnostic features and the management options. What is Known: • Fibrolipomatous hamartomas (FLHs) of the median nerve are rare, possibly associated with macrodactyly and tissue growth at the wrist and thenar side of the palm. • An associated carpal tunnel syndrome typically occurs, if at all, in adulthood. What is New: • We describe two children below 4 years with symptomatic carpal tunnel syndrome, experiencing a long-lasting favorable outcome after carpal tunnel decompression. In this age group, only one other child undergoing surgery has been published so far. • MRI and high-resolution ultrasound demonstrate the characteristic features of FLHs and are the diagnostic modalities of choice. Biopsy is not recommended.

Comparison between high-resolution CT and MRI using a very short echo time in patients with cystic fibrosis with extra focus on mosaic attenuation.

BACKGROUND: It would be beneficial to establish pulmonary MRI as a complementary approach to CT for direct visualization of mosaic perfusion, bullae, and emphysema in patients with cystic fibrosis. OBJECTIVES: The purpose of this study was to compare both modalities, CT and MRI, using the Helbich-Bhalla score with a special focus on reliable detection of a mosaic pattern. METHODS: Out of 51 patients examined by MRI on a 1.5-Tesla system during a period of 2 years, 19 patients were scheduled for additional low-dose CT in a clinical context. The MRI protocol comprised a gradient echo (GRE) sequence with a very short echo time (TE = 0.8 ms) in inspiration and expiration, a 3-D GRE sequence in breath hold, and a fast spin echo sequence with respiration and ECG triggering. MDCT was carried out in inspiration and adapted to body weight using 100 or 120 kV, 30-60 mA, 1- and 3-mm slice thicknesses, as well as low and high kernels. Additionally incremental slices in 3 positions were recorded in expiration for distinct detection of air trapping. CT and MRI analyses were performed by two radiologic readers in consensus unaware of the clinical parameters. The Helbich-Bhalla score of both examinations was correlated. Mean difference and accordance were assessed in each category. RESULTS: There was a strong correlation between CT and MRI (R = 0.87, p < 0.01). The mean Helbich-Bhalla score for CT was 12.2 (range 1-18) and for MRI it was 11.7 (range 2-19). The mean difference was 0.5 points. Besides this strong correlation for findings (bronchiectasis, mucus plugging, peribronchial thickening, and consolidation) with a prolonged T2 TE in MRI, we could also state a qualitative agreement of 95-100% in the categories with short T2 and low signal intensity in MRI as emphysema, bullae, and mosaic perfusion. CONCLUSIONS: These results suggest that in our patient group none of the relevant findings were missed by MR imaging and reading.

Teaching medicine web-based with the help of interactive audience response systems.

The COVID-19 pandemic confronted the medical community worldwide with numerous challenges, not only with respect to medical care, but also for teaching the next generation of physicians. To minimize the risk of infections patient-unrelated classes can be held digitally. Here we present a student initiated, web-based teaching approach, called "From symptom to diagnosis". In this seminar case reports of rare diseases were presented to the audience in a symptom-focused manner. The patients´ most significant symptoms were presented, followed by an in-depth discussion about differential diagnosis. First glance diagnosis pictures were shown to improve students´ ability to identify important clinical scenarios. We used chat functions as well as an audience response system to make the seminar more interactive. By this we attracted between 71 and 147 participants per session. The online seminar was very well perceived and 97% of the students saw an improvement of their diagnostic skills. In summary, we successfully established an interactive, web-based teaching format for medical students.