Computed tomography imaging phenotypes of hepatoblastoma identified from radiomics signatures are associated with the efficacy of neoadjuvant chemotherapy.

BACKGROUND: Though neoadjuvant chemotherapy has been widely used in the treatment of hepatoblastoma, there still lacks an effective way to predict its effect. OBJECTIVE: To characterize hepatoblastoma based on radiomics image features and identify radiomics-based lesion phenotypes by unsupervised machine learning, intended to build a classifier to predict the response to neoadjuvant chemotherapy. MATERIALS AND METHODS: In this retrospective study, we segmented the arterial phase images of 137 cases of pediatric hepatoblastoma and extracted the radiomics features using PyRadiomics. Then unsupervised k-means clustering was applied to cluster the tumors, whose result was verified by t-distributed stochastic neighbor embedding (t-SNE). The least absolute shrinkage and selection operator (LASSO) regression was used for feature selection, and the clusters were visually analyzed by radiologists. The correlations between the clusters, clinical and pathological parameters, and qualitative radiological features were analyzed. RESULTS: Hepatoblastoma was clustered into three phenotypes (homogenous type, heterogenous type, and nodulated type) based on radiomics features. The clustering results had a high correlation with response to neoadjuvant chemotherapy (P=0.02). The epithelial ratio and cystic components in radiological features were also associated with the clusters (P=0.029 and 0.008, respectively). CONCLUSIONS: This radiomics-based cluster system may have the potential to facilitate the precise treatment of hepatoblastoma. In addition, this study further demonstrated the feasibility of using unsupervised machine learning in a disease without a proper imaging classification system.

Timing of magnetic resonance imaging in pregnancy for outcome prediction in congenital diaphragmatic hernia.

PURPOSE: To evaluate the impact of the timing of MRI on the prediction of survival and morbidity in patients with CDH, and whether serial measurements have a beneficial value. METHODS: This retrospective cohort study was conducted in two perinatal centers, in Germany and Italy. It included 354 patients with isolated CDH having at least one fetal MRI. The severity was assessed with the observed-to-expected total fetal lung volume (o/e TFLV) measured by two experienced double-blinded operators. The cohort was divided into three groups according to the gestational age (GA) at which the MRI was performed (< 27, 27-32, and > 32 weeks' gestation [WG]). The accuracy for the prediction of survival at discharge and morbidity was analyzed with receiver operating characteristic (ROC) curves. Multiple logistic regression analyses and propensity score matching examined the population for balance. The effect of repeated MRI was evaluated in ninety-seven cases. RESULTS: There were no significant differences in the prediction of survival when the o/e TFLV was measured before 27, between 27 and 32, and after 32 WG (area under the curve [AUC]: 0.77, 0.79, and 0.77, respectively). After adjustment for confounding factors, it was seen, that GA at MRI was not associated with survival at discharge, but the risk of mortality was higher with an intrathoracic liver position (adjusted odds ratio [aOR]: 0.30, 95% confidence interval [95%CI] 0.12-0.78), lower GA at birth (aOR 1.48, 95%CI 1.24-1.78) and lower o/e TFLV (aOR 1.13, 95%CI 1.06-1.20). ROC curves showed comparable prediction accuracy for the different timepoints in pregnancy for pulmonary hypertension, the need of extracorporeal membrane oxygenation, and feeding aids. Serial measurements revealed no difference in change rate of the o/e TFLV according to survival. CONCLUSION: The timing of MRI does not affect the prediction of survival rate or morbidity as the o/e TFLV does not change during pregnancy. Clinicians could choose any gestational age starting mid second trimester for the assessment of severity and counseling.

Functional lung imaging of 2-year-old children after congenital diaphragmatic hernia repair using dynamic mode decomposition MRI.

OBJECTIVES: To investigate the feasibility of non-contrast-enhanced functional lung imaging in 2-year-old children after congenital diaphragmatic hernia (CDH) repair. METHODS: Fifteen patients after CDH repair were examined using non-contrast-enhanced dynamic magnetic resonance imaging (MRI). For imaging two protocols were used during free-breathing: Protocol A with high temporal resolution and Protocol B with high spatial resolution. The dynamic images were then analysed through a recently developed post-processing method called dynamic mode decomposition (DMD) to obtain ventilation and perfusion maps. The ventilation ratios (VRatio) and perfusion ratios (QRatio) of ipsilateral to contralateral lung were compared to evaluate functional differences. Lastly, DMD MRI-based perfusion results were compared with perfusion parameters obtained using dynamic contrast-enhanced (DCE) MRI to assess agreement between methods. RESULTS: Both imaging protocols successfully generated pulmonary ventilation (V) and perfusion (Q) maps in all patients. Overall, the VRatio and QRatio values were 0.84 ± 0.19 and 0.70 ± 0.24 for Protocol A, and 0.88 ± 0.18 and 0.72 ± 0.23 for Protocol B, indicating reduced ventilation ([Formula: see text]) and perfusion ([Formula: see text]) on the ipsilateral side. Moreover, there is a very strong positive correlation ([Formula: see text]) and close agreement between DMD MRI-based perfusion values and DCE MRI-based perfusion parameters. CONCLUSIONS: DMD MRI can obtain pulmonary functional information in 2-year-old CDH patients. The results obtained with DMD MRI correlate with DCE MRI, without the need for ionising radiation or exposure to contrast agents. While further studies with larger cohorts are warranted, DMD MRI is a promising option for functional lung imaging in CDH patients. CLINICAL RELEVANCE STATEMENT: We demonstrate that pulmonary ventilation and perfusion information can be obtained in 2-year-old patients after CDH repair, without the need for ionising radiation or contrast agents by utilising non-contrast-enhanced MRI acquisitions together with dynamic mode decomposition analysis. KEY POINTS: • Non-contrast-enhanced functional MR imaging is a promising option for functional lung imaging in 2-year-old children after congenital diaphragmatic hernia. • DMD MRI can generate pulmonary ventilation and perfusion maps from free-breathing dynamic acquisitions without the need for ionising radiation or contrast agents. • Lung perfusion parameters obtained with DMD MRI correlate with perfusion parameters obtained using dynamic contrast-enhanced MRI.

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.

Phase-cycled balanced SSFP imaging for non-contrast-enhanced functional lung imaging.

PURPOSE: To introduce phase-cycled balanced SSFP (bSSFP) acquisition as an alternative in Fourier decomposition MRI for improved robustness against field inhomogeneities. METHODS: Series 2D dynamic lung images were acquired in 5 healthy volunteers at 1.5 T and 3 T using bSSFP sequence with multiple RF phase increments and compared with conventional single RF phase increment acquisitions. The approach was evaluated based on functional map homogeneity analysis, while ensuring image and functional map quality by means of SNR and contrast-to-noise ratio analyses. RESULTS: At both field strengths, functional maps obtained with phase-cycled acquisitions displayed improved robustness against local signal losses compared with single-phase acquisitions. The coefficient of variation (mean ± SD, across volunteers) measured in the ventilation maps resulted in 29.7 ± 2.6 at 1.5 T and 37.5 ± 3.1 at 3 T for phase-cycled acquisitions, compared with 39.9 ± 5.2 at 1.5 T and 49.5 ± 3.7 at 3 T for single-phase acquisitions, indicating a significant improvement ( p<0.05$$ p<0.05 $$ ) in ventilation map homogeneity. CONCLUSIONS: Phase-cycled bSSFP acquisitions improve robustness against field inhomogeneity artifacts and significantly improve ventilation map homogeneity at both field strengths. As such, phase-cycled bSSFP may serve as a robust alternative in lung function assessments.

MR lung perfusion measurements in adolescents after congenital diaphragmatic hernia: correlation with spirometric lung function tests.

OBJECTIVES: To evaluate whether lung perfusion continues to be reduced in 10-year-old children after congenital diaphragmatic hernia (CDH) and whether lung perfusion values correlate with spirometric lung function measurements. METHODS: Fifty-four patients after CDH repair received dynamic contrast-enhanced (DCE) magnetic resonance imaging (MRI)-based lung perfusion measurements at the age of 10 years (10.2 ± 1.0 years). Additionally, a control group of 10 children has been examined according to the same protocol. Lung spirometry was additionally available in 43 patients of the CDH group. A comparison of ipsilateral and contralateral parameters was performed. RESULTS: Pulmonary blood flow (PBF) was reduced on the ipsilateral side in CDH patients (60.4 ± 23.8 vs. 93.3 ± 16.09 mL/100 mL/min; p < 0.0001). In comparison to the control group, especially the ratio of ipsilateral to contralateral, PBF was reduced in CDH patients (0.669 ± 0.152 vs. 0.975 ± 0.091; p < 0.0001). There is a positive correlation between ipsilateral pulmonary blood flow, and spirometric forced 1-s volume (r = 0.45; p = 0.0024). CONCLUSIONS: Pulmonary blood flow impairment persists during childhood and correlates with spirometric measurements. Without the need for ionizing radiation, MRI measurements seem promising as follow-up parameters after CDH. KEY POINTS: • Ten-year-old children after congenital diaphragmatic hernia continue to show reduced perfusion of ipsilateral lung. • Lung perfusion values correlate with lung function tests after congenital diaphragmatic hernia.

Radiation dose of chaperones during common pediatric computed tomography examinations.

BACKGROUND: One main challenge in pediatric imaging is to reduce motion artifacts by calming young patients. To that end, the Radiological Society of North America (RSNA) as early as 1997 stated the necessity of adults accompanying their child during the child's examination. Nonetheless, current research lacks data regarding radiation dose to these chaperones. OBJECTIVE: The aim of this study was to measure the radiation dose of accompanying adults during state-of-the-art pediatric CT protocols. MATERIALS AND METHODS: In addition to a 100-kV non-contrast-enhanced chest CT (Protocol 1), we performed a 70-kV contrast-enhanced chest protocol (Protocol 2) using a third-generation dual-source CT. We acquired data on the radiation dose around the scanner using digital dosimetry placed right at the gantry, 1 m away, as well as beside the gantry. We acquired the CT-surrounding radiation dose during scanning of a pediatric phantom as well as 12 pediatric patients. RESULTS: After conducting 10 consecutive phantom scans using Protocol 1, we found the location with the highest cumulative dose acquired was right next to the gantry opening, at 3 µSv. Protocol 2 showed highest cumulative dose of 2 µSv at the same location. For Protocol 1, the location with the highest radiation doses during pediatric scans was right next to the gantry opening, with doses of 0.75±0.70 µSv. For Protocol 2, the highest radiation was measured 1 m away at 0.50±0.60 µSv. No radiation dose was measured at any time beside the gantry. CONCLUSION: Our results provide proof that chaperones receive low radiation doses during state-of-the-art CT examinations. Given knowledge of these values as well as the optimal spots with the lowest radiation doses, parents as well as patients might be more relaxed during the examination.

[MRI-Based Ratio of Fetal Lung to Body Volume as New Prognostic Marker for Chronic Lung Disease in Patients with Congenital Diaphragmatic Hernia]. / MRT-basierter Quotient aus fetalem Lungen- und Ganzkörpervolumen als Prognosemarker für chronische Lungenerkrankung bei kongenitaler Zwerchfellhernie.

Background Our aim was to evaluate the prognostic value of magnetic resonance imaging (MRI)-based ratio of fetal lung volume (FLV) to fetal body volume (FBV) as a marker for development of chronic lung disease (CLD) in fetuses with congenital diaphragmatic hernia (CDH). Patients and Methods FLV and FBV were measured and the individual FLV/FBV ratio was calculated in 132 fetuses. Diagnosis of CLD was established following prespecified criteria and graded into mild/moderate/severe if present. Logistic regression analysis was used to calculate the probability of postnatal development of CLD in dependence of the FLV/FBV ratio. Receiver operating characteristic curves were analysed by calculating the area under the curve to evaluate the prognostic accuracy of this marker. Results 61 of 132 fetuses developed CLD (46.21%). The FLV/FBV ratio was significantly lower in fetuses with CLD (p=0.0008; AUC 0.743). Development of CLD was significantly associated with thoracic herniation of liver parenchyma (p<0.0001), requirement of extracorporal membrane oxygenation (ECMO) (p<0.0001) and gestational age at delivery (p=0.0052). Conclusion The MRI-based ratio of FLV to FBV is a highly valuable prenatal parameter for development of CLD. The ratio is helpful for early therapeutic decisions by estimating the probability to develop CLD. Perinatally, gestational age at delivery and ECMO requirement are useful additional parameters to further improve prediction of CLD.

Separate Evaluation of the Ipsilateral and Contralateral MR Fetal Lung Volume in Patients With Congenital Diaphragmatic Hernia.

OBJECTIVE: Our study had two objectives. First, we separately evaluated observed-to-expected MR fetal lung volume (FLV) of lungs ipsilateral and contralateral to a congenital diaphragmatic hernia (CDH). Second, we compared the prognostic value of observed-to-expected MR FLV of the ipsilateral and contralateral lungs with that of observed-to-expected MR FLV of both lungs with respect to survival, need for extracorporeal membrane oxygenation (ECMO), and development of chronic lung disease (CLD). MATERIALS AND METHODS: We evaluated observed-to-expected MR FLV of the lung ipsilateral to the diaphragmatic defect as well as in the contralateral lung in 107 fetuses with isolated CDH. ROC analysis and logistic regression analysis were performed to assess the prognostic value of the observed-to-expected MR FLV for association with outcome. RESULTS: In all fetuses with CDH, values in the ipsilateral (mean observed-to-expected MR FLV ± SD, 9.4% ± 9.6%) and the contralateral lung (mean observed-to-expected MR FLV, 48.9% ± 18.5%; p < 0.0001) were significantly lower than values measured in healthy fetuses. Observed-to-expected MR FLV of both lungs and of the contralateral and ipsilateral lung revealed significant differences regarding survival (p < 0.0001, p < 0.0001, and p = 0.0170, respectively), need for ECMO (p < 0.0001, p < 0.0001, and p = 0.0051, respectively), and development of CLD (p = 0.0004, p = 0.0002, and p = 0.0460, respectively). Compared with the observed-to-expected MR FLV of both lungs, the observed-to-expected MR FLV of the contralateral lung showed a slightly higher prognostic accuracy regarding survival (AUC = 0.859 vs 0.825) and development of CLD (AUC = 0.734 vs 0.732) and a similar prognostic accuracy regarding need for ECMO (AUC = 0.805 vs 0.826). Observed-to-expected MR FLV of the ipsilateral lung did not show good prognostic value regarding survival (AUC = 0.617), need for ECMO (AUC = 0.673), and development of CLD (AUC = 0.636). These AUCs were significantly smaller than the AUCs resulting from the observed-to-expected MR FLV of both lungs (each p < 0.05) and considerably smaller than the AUCs of the observed-to-expected MR FLV of the contralateral lung (each p < 0.10). CONCLUSION: Patients with CDH showed a substantially lower observed-to-expected MR FLV of both lungs compared with healthy fetuses. The observed-to-expected MR FLV of both lungs as well as of the lung contralateral to the CDH were reliable prenatal predictors of survival, need for ECMO, and development of CLD.

Lung Perfusion MRI After Congenital Diaphragmatic Hernia Repair in 2-Year-Old Children With and Without Extracorporeal Membrane Oxygenation Therapy.

OBJECTIVE: In severe cases of congenital diaphragmatic hernia (CDH), extracorporeal membrane oxygenation (ECMO) therapy improves survival. Later on, lung morbidity mainly defines development. The purpose of this study was to investigate whether 2-year-old children who need ECMO therapy after delivery have reduced perfusion MRI values as a sign of more severe lung hypoplasia than do children who do not need ECMO. MATERIALS AND METHODS: After CDH repair, 38 children underwent dynamic contrast-enhanced MRI with a 3D time-resolved angiography with stochastic trajectories sequence. Fifteen (39%) of the children had received ECMO therapy in the neonatal period. Pulmonary blood flow (PBF), pulmonary blood volume (PBV), and mean transit time were calculated for both lungs. In addition, the ratio of ipsilateral to contralateral lung was calculated for all parameters. RESULTS: In all children, those with and those without ECMO requirement, PBF and PBV were significantly reduced on the ipsilateral side (p < 0.05). Children who had received ECMO therapy had significantly reduced PBF and PBV values on the ipsilateral side (p < 0.05) compared with children who had not needed ECMO therapy. The ratios of ipsilateral to contralateral lung for PBF and PBV were also significantly reduced after ECMO. CONCLUSION: Two-year-old children undergoing CDH repair who had needed neonatal ECMO had significantly reduced perfusion MRI values in the ipsilateral lung in comparison with children who had not needed ECMO. Perfusion MRI measurements are associated with the severity of lung hypoplasia and may therefore be helpful in follow-up investigations.

Correlation of Observed-to-Expected MRI Fetal Lung Volume and Ultrasound Lung-to-Head Ratio at Different Gestational Times in Fetuses With Congenital Diaphragmatic Hernia.

OBJECTIVE: The purpose of this study is to compare the observed-to-expected MRI fetal lung volume and the observed-to-expected ultrasound lung-to-head ratio intraindividually for prediction of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD) at different times of gestation (< 28, 28-32, and > 32 weeks) in fetuses with congenital diaphragmatic hernia (CDH), and to analyze the intraindividual correlation of observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio. MATERIALS AND METHODS: Two hundred one fetuses were included in our study. Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio were calculated for 270 MRI and ultrasound examinations performed within 72 hours of each other. The prognostic accuracy of observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio was assessed by performing ROC analysis. Correlation was determined using linear regression analysis. RESULTS: The results revealed statistically significant differences between the observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio for the prediction of survival, the need for ECMO therapy, and the development of CLD in fetuses with left-sided CDH (p ≤ 0.0444), with the highest prognostic accuracy for survival (AUC = 0.863). Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio correlated statistically significantly (p < 0.0001) for left-sided CDH with correlation coefficients (r) of 0.71, 0.71, and 0.56 for early, intermediate, and late times of gestation, respectively. The correlation was not statistically significant at any time for right-sided CDH (p ≥ 0.3947; r ≤ 0.26). CONCLUSION: Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio are valuable prognostic parameters for prenatal prediction of survival, the need for ECMO therapy, and the development of CLD in fetuses with left-sided CDH at all times of gestation. Observed-to-expected MRI fetal lung volume and observed-to-expected ultrasound lung-to-head ratio correlate statistically significantly for left-sided CDH, and the correlation is best before 32 weeks' gestation, but they do not correlate statistically significantly for right-sided CDH.

Comparison of image quality and radiation dose between an image-intensifier system and a newer-generation flat-panel detector system ­ technical phantom measurements and evaluation of clinical imaging in children.

BACKGROUND: Many image-intensifier fluoroscopy systems have been replaced by flat-panel detectors in recent years. OBJECTIVE: To compare the level of contrast, image resolution and radiation dose between an image-intensifier and a newer-generation flat-panel detector system in a pediatric radiology unit. MATERIALS AND METHODS: We compared two systems ­ a conventional image intensifier and a newer-generation flat-panel system. We measured image quality and radiation dose using a technical phantom. Additionally, we retrospectively compared age-matched fluoroscopic pediatric voiding cystourethrography (n = 15) and upper gastrointestinal investigations (n = 25). RESULTS: In phantom studies image contrast was equal while image resolution was higher and mean radiation dose lower using the flat-panel system (P < 0.0001). In pediatric investigations, mean dose area product was significantly reduced on the flat-panel system for upper gastrointestinal investigation (45 ± 38 µGy*m2 vs. 11 ± 9 µGy*m2; P < 0.0001) and for voiding cystourethrography (18 ± 20 µGy*m2 vs. 10 ± 12 µGy*m2; P = 0.04). CONCLUSION: The newer flat-panel system performs at lower dose levels with equal to better image quality and therefore seems to be the more suitable technique for pediatric fluoroscopy in comparison to image-intensifier systems.

Pleural Effusion and Chylothorax in Congenital Diaphragmatic Hernia-Risk Factors, Management and Outcome.

Background: Pleural effusion and chylothorax are common complications in the treatment of congenital diaphragmatic hernia (CDH). We set out to identify risk factors for chylothorax development in patients with CDH and to investigate the association of pleural effusion and chylothorax with neonatal morbidity and mortality. Methods: In this retrospective cohort study, we included 396 neonates with CDH treated at our institution between January 2013 and June 2019. Preoperative and postoperative chest radiographs and clinical data were evaluated and correlated with morbidity, complications and mortality. Results: Laboratory-confirmed chylothorax occurred in 58 (18.6%) of all CDH cases. Pleural effusion was frequently observed as a postoperative complication but also occurred as a pre-existing condition. Neonates with large defects of size C and D, patch repair, the need for presurgical and/or postsurgical ECMO support, pulmonary hypertension, liver-up phenomenon and lower relative fetal lung volume were associated with higher occurrences of chylothorax. After stepwise logistic regression, larger CDH defects (p < 0.0001) and the need for postsurgical ECMO (p = 0.0158) remained significant risk factors for CTX to occur (AUC 0.71). The same potential risk factors were used to assess their association with both presurgical and postsurgical pleural effusion. After stepwise logistic regression, only the need for presurgical ECMO remained significantly associated with presurgical PE (p < 0.01, AUC 0.65) and patch repair as the therapeutic intervention remained significantly associated with the occurrence of postsurgical PE (p < 0.0001, AUC 0.80). Patients with CTX had longer durations of both MV (p < 0.0001) and subsequent ventilatory assistance with spontaneous breathing (p = 0.0004), increased total lengths of hospitalization (p < 0.0001), increased durations of ECMO (p < 0.01) and increased incidences of CLD (p < 0.0001) compared to patients without CTX. No significant difference could be found for survival in both groups (p = 0.12). Conclusions: Our data suggest that the incidence of chylothorax is associated with large diaphragmatic defects, the need for postsurgical ECMO and the development of chronic lung disease, but not with survival.

Fetal MRI-Based Mediastinal Shift Angle (MSA) and Percentage Area of Left Ventricle (pALV) as Prognostic Parameters for Congenital Diaphragmatic Hernia.

OBJECTIVE: Fetal magnetic resonance imaging (MRI) is broadly used as a method for assessing prognosis in congenital diaphragmatic hernia (CDH). In addition to the extent of lung hypoplasia, determined by measuring the lung volume, cardiac impairment due to pulmonary hypertension and left cardiac hypoplasia is decisive for the prognosis. The percentage area of left ventricle (pALV) describes the percentage of the inner area of the left ventricle in relation to the total area, whereas the mediastinal shift angle (MSA) quantifies the extent of cardiac displacement. The prognostic value of pALV and MSA should be evaluated in terms of survival, the need for extracorporeal membrane oxygenation (ECMO) therapy, and the development of chronic lung disease (CLD). METHODS: In a total of 122 fetal MRIs, the MSA and pALV were measured retrospectively and complete outcome parameters were determined regarding survival for all 122 subjects, regarding ECMO therapy in 109 cases and about the development of CLD in 78 cases. The prognostic value regarding the endpoints was evaluated using logistic regression and ROC analysis. RESULTS: The MSA was significantly higher in children who received ECMO therapy (p = 0.0054), as well as in children who developed CLD (p = 0.0018). ROC analysis showed an AUC of 0.68 for ECMO requirement and 0.77 with respect to CLD development. The pALV showed a tendency towards higher levels in children who received ECMO therapy (p = 0.0824). The MSA and the pALV had no significant effect on survival (MSA: p = 0.4293, AUC = 0.56; pALV: p = 0.1134, AUC = 0.57). CONCLUSIONS: The MSA determined in fetal MRI is a suitable prognostic parameter for ECMO requirement and CLD development in CDH patients and can possibly be used as a supplement to the established parameters.

Long-Term Evaluation of the Shape of the Reconstructed Diaphragm in Patients with Left-Sided Congenital Diaphragmatic Hernia Using Serial Chest Radiographs and Correlation to Further Complications.

Background: Defining risk factors for long-term comorbidities in patients after neonatal repair of congenital diaphragmatic hernia (CDH) is an important cornerstone of the implementation of targeted longitudinal follow-up programs. Methods: This study systematically assessed serial chest radiographs of 89 patients with left-sided CDH throughout a mean follow-up of 8.2 years. These geometrical variables for the left and right side were recorded: diaphragmatic angle (LDA, RDA), diaphragmatic diameter (LDD, RDD), diaphragmatic height (LDH, RDH), diaphragmatic curvature index (LDCI, RDCI), lower lung diameter (LLLD, RLLD) and thoracic area (LTA, RTA). Results: It was demonstrated that the shape of the diaphragm in patients with large defects systematically differs from that of patients with small defects. Characteristically, patients with large defects present with a smaller LDCI (5.1 vs. 8.4, p < 0.001) at 6 months of age, which increases over time (11.4 vs. 7.0 at the age of 15.5 years, p = 0.727), representing a flattening of the patch and the attached rudimentary diaphragm as the child grows. Conclusions: Multiple variables during early follow-up were significantly associated with comorbidities such as recurrence, scoliotic curves of the spine and a reduced thoracic area. Some geometrical variables may serve as surrogate parameters for disease severity, which is associated with long-term comorbidities.

Fetoscopic endoluminal tracheal occlusion vs expectant management for fetuses with severe left-sided congenital diaphragmatic hernia.

BACKGROUND: The treatment of fetuses with a congenital diaphragmatic hernia is challenging, but there is evidence that fetoscopic endoluminal tracheal occlusion has a benefit over expectant care. In addition, standardization and expertism have a great impact on survival and are probably crucial in centers that rely on expectant management with extracorporeal membrane oxygenation after birth. OBJECTIVE: This study aimed to examine the survival and morbidity rates of fetuses with a severe isolated left-sided congenital diaphragmatic hernia who underwent fetoscopic endoluminal tracheal occlusion vs expectant management in high-volume centers. STUDY DESIGN: This was a multicenter, retrospective study that included all consecutive fetuses with severe isolated left-sided congenital diaphragmatic hernia who were expectantly managed in a German center or who underwent fetoscopic endoluminal tracheal occlusion in 3 other European centers (Belgium, France, and Italy). Severe congenital diaphragmatic hernia was defined as having an observed to expected total fetal lung volume ≤35% with intrathoracic position of the liver diagnosed with magnetic resonance imaging. All magnetic resonance images were centralized, and lung volumes were measured by 2 experienced operators who were blinded to the pre- and postnatal data. Multiple logistic regression analyses were performed to examine the effect of the management strategy in the 2 groups on the short- and long-term outcomes. RESULTS: A total of 147 patients who were managed expectantly and 47 patients who underwent fetoscopic endoluminal tracheal occlusion were analyzed. Fetuses who were managed expectantly had lower observed to expected total fetal lung volumes (20.6%±7.5% vs 23.7%±6.8%; P=.013), higher gestational age at delivery (median weeks of gestation, 37.4; interquartile range, 36.6-38.00 vs 35.1; interquartile range, 33.1-37.2; P<.001), and more frequent use of extracorporeal membrane oxygenation (55.8% vs 4.3%; P<.001) than the fetuses who underwent fetoscopic endoluminal tracheal occlusion. The survival rates at discharge and at 2 years of age in the expectant management group were higher than the survival rates of the fetoscopic endoluminal tracheal occlusion group (74.3% vs 44.7%; P=.001 and 72.8% vs 42.5%; P=.001, respectively). After adjustment for maternal age, gestational age at birth, observed to expected total fetal lung volume, and birth weight Z-score, the odds ratios were 4.65 (95% confidence interval, 1.9-11.9; P=.001) and 4.37 (95% confidence interval, 1.8-11.0; P=.001), respectively. CONCLUSION: Fetuses with a severe isolated left-sided congenital diaphragmatic hernia had a higher survival rate when treated in an experienced center in Germany with antenatal expectant management and frequent use of extracorporeal membrane oxygenation during the postnatal period than fetuses who were treated with fetoscopic endoluminal tracheal occlusion in 3 centers in Belgium, France, and Italy.

Photon-counting computed tomography - clinical application in oncological, cardiovascular, and pediatric radiology. / Photon-Counting Computertomographie ­ klinische Anwendungen in der onkologischen, kardiovaskulären und pädiatrischen Radiologie.

BACKGROUND: Photon-counting detector computed tomography (PCD-CT) is a promising new technology with the potential to fundamentally change workflows in the daily routine and provide new quantitative imaging information to improve clinical decision-making and patient management. METHOD: The contents of this review are based on an unrestricted literature search of PubMed and Google Scholar using the search terms "photon-counting CT", "photon-counting detector", "spectral CT", "computed tomography" as well as on the authors' own experience. RESULTS: The fundamental difference with respect to the currently established energy-integrating CT detectors is that PCD-CT allows for the counting of every single photon at the detector level. Based on the identified literature, PCD-CT phantom measurements and initial clinical studies have demonstrated that the new technology allows for improved spatial resolution, reduced image noise, and new possibilities for advanced quantitative image postprocessing. CONCLUSION: For clinical practice, the potential benefits include fewer beam hardening artifacts, a radiation dose reduction, and the use of new or combinations of contrast agents. In particular, critical patient groups such as oncological, cardiovascular, lung, and head & neck as well as pediatric patient collectives benefit from the clinical advantages. KEY POINTS: · Photon-counting computed tomography (PCD-CT) is being used for the first time in routine clinical practice, enabling a significant dose reduction in critical patient populations such as oncology, cardiology, and pediatrics.. · Compared to conventional CT, PCD-CT enables a reduction in electronic image noise.. · Due to the spectral data sets, PCD-CT enables fully comprehensive post-processing applications.. CITATION FORMAT: · Hagen F, Soschynski M, Weis M et al. Photon-counting computed tomography - clinical application in oncological, cardiovascular, and pediatric radiology. Fortschr Röntgenstr 2024; 196: 25 - 34.

aCGH Analysis Reveals Novel Mutations Associated with Congenital Diaphragmatic Hernia Plus (CDH+).

Congenital diaphragmatic hernia (CDH) is a major birth anomaly that often occurs with additional non-hernia-related malformations, and is then referred to as CDH+. While the impact of genetic alterations does not play a major role in isolated CDH, patients with CDH+ display mutations that are usually determined via array-based comparative genomic hybridization (aCGH). We analyzed 43 patients with CDH+ between 2012 and 2021 to identify novel specific mutations via aCGH associated with CDH+ and its outcome. Deletions (n = 32) and duplications (n = 29) classified as either pathological or variants of unknown significance (VUS) could be detected. We determined a heterozygous deletion of approximately 3.75 Mb located at 8p23.1 involving several genes including GATA4, NEIL2, SOX7, and MSRA, which was consequently evaluated as pathological. Another heterozygous deletion within the region of 9p23 (9,972,017-10,034,230 kb) encompassing the Protein Tyrosine Phosphatase Receptor Type Delta gene (PTPRD) was identified in 2 patients. This work expands the knowledge of genetic alterations associated with CDH+ and proposes two novel candidate genes discovered via aCGH.