[A Uncommon Case: Kasabach-Merritt syndrome with VACTERL Association]. / Ein seltener Fall: Kasabach-Merrit-Syndrom bei VACTERL-Assoziation.

The Kasabach-Merrit syndrome is characterized as the association of a vascular tumor, typically a caposiform hemangioendothelioma and rarely a tufted hemangioma, and a severe consumptive coagulopathy with potentially life-threatening thrombocytopenia. The severe coagulopathy with increased bleeding tendency must be considered before invasive procedures and often requires repeated platelet concentrate substitutions. We present a case of a mature male neonate with Kasabach-Merritt- Syndrome as well as VACTERL association. The VACTERL association describes a group of malformations. Our patient presented with anal atresia combined with tethered cord, and left renal agenesis. The VACTERL association as well as Kasabach-Merritt syndrome were found to be independent pathologies within this patient. A common occurrence or an association with each other has not been described in the literature so far. The challenging coagulation setting due to severe thrombocytopenia complicated the surgical management so far. Finally, mTOR-inhibitor sirolimus was successful in terms of tumor reduction and especially reduction of platelet consumption.

Hepatic Artery Delineation on Ultrasound Volumes Comparing B-Flow and Color Doppler for Postoperative Monitoring of Pediatric Liver Transplants.

(1) Background: Accurate hepatic artery (HA) depiction following pediatric liver transplantation (LT) is essential for graft surveillance but challenging on ultrasound (US). This study assesses if improved HA delineation can be achieved by recording two-dimensional US volumes in Color Doppler (CD) and B-flow technique. (2) Methods: Of 42 consecutive LT, 37 cases were included, and HA delineation was retrospectively rated using a four-point score (0 = HA not detectable, 3 = HA fully detectable, separable from portal vein) within 48 h post-LT (U1) and before discharge (U2). (3) Results: Adding B-flow compared with CD alone showed superior results at neohilar (U1: 2.2 ± 1.0 vs. 1.1 ± 0.8, p < 0.0001; U2: 2.5 ± 0.8 vs. 1.5 ± 0.9, p < 0.0001) and segmental levels (U1: 2.8 ± 0.6 vs. 0.6 ± 0.8, p < 0.0001; U2: 2.8 ± 0.6 vs. 0.7 ± 0.5, p < 0.0001). (4) Conclusions: Standardized US volume recordings combining B-flow and CD can effectively delineate the HA along its vascular course in pediatric LT. The technique should be further evaluated as a standard monitoring instrument to rule out vascular complications after LT.

Non-contrast free-breathing 2D CINE compressed SENSE T1-TFE cardiovascular MRI at 3T in sedated young children for assessment of congenital heart disease.

Cardiac MRI is a crucial tool for assessing congenital heart disease (CHD). However, its application remains challenging in young children when performed at 3T. The aim of this retrospective single center study was to compare a non-contrast free-breathing 2D CINE T1-weighted TFE-sequence with compressed sensing (FB 2D CINE CS T1-TFE) with 3D imaging for diagnostic accuracy of CHD, image quality, and vessel diameter measurements in sedated young children. FB 2D CINE CS T1-TFE was compared with a 3D non-contrast whole-heart sequence (3D WH) and 3D contrast-enhanced MR angiography (3D CE-MRA) at 3T in 37 CHD patients (20♂, 1.5±1.4 years). Two radiologists independently assessed image quality, type of CHD, and diagnostic confidence. Diameters and measures of contrast and sharpness of the aorta and pulmonary vessels were determined. A non-parametric multi-factorial approach was used to estimate diagnostic accuracy for the diagnosis of CHD. Linear mixed models were calculated to compare contrast and vessel sharpness. Krippendorff's alpha was determined to quantify vessel diameter agreement. FB 2D CINE CS T1-TFE was rated superior regarding image quality, diagnostic confidence, and diagnostic sensitivity for both intra- and extracardiac pathologies compared to 3D WH and 3D CE-MRA (all p<0.05). FB 2D CINE CS T1-TFE showed superior contrast and vessel sharpness (p<0.001) resulting in the highest proportion of measurable vessels (740/740; 100%), compared to 3D WH (530/620; 85.5%) and 3D CE-MRA (540/560; 96.4%). Regarding vessel diameter measurements, FB 2D CINE CS T1-TFE revealed the closest inter-reader agreement (Krippendorff's alpha: 0.94-0.96; 3D WH: 0.78-0.94; 3D CE-MRA: 0.76-0.93). FB 2D CINE CS T1-TFE demonstrates robustness at 3T and delivers high-quality diagnostic results to assess CHD in sedated young children. Its ability to function without contrast injection and respiratory compensation enhances ease of use and could encourage widespread adoption in clinical practice.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (2) intraoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of intraoperative and postoperative complications. So far, only limited data regarding the best radiological approach to monitor children during liver transplantation is available. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra- and postoperative phase. This paper reports the responses related to intraoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: Intraoperative ultrasound (US) is used by all sites to assess the quality of the vascular anastomosis in order to ensure optimal perfusion of the liver transplant. Vessel depiction is commonly achieved using color Doppler (95.3%). Additional US-based techniques are employed by fewer centers (power angio mode, 28.6%; B-flow, 19%; contrast-enhanced US, 14.3%). Most centers prefer a collaborative approach, with surgeons responsible for probe handling, while radiologists operate the US machine (47.6%). Less commonly, the intraoperative US is performed by the surgeon alone (28.6%) or by the radiologist alone (23.8%). Timing of US, imaging frequency, and documentation practices vary among centers. CONCLUSION: Intraoperative US is consistently utilized across all sites during pediatric liver transplantation. However, considerable variations were observed in terms of the US setup, technique preferences, timing of controls, and documentation practices. These differences provide valuable insights for future optimization and harmonization studies.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (3) postoperative imaging.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment for end-stage liver disease. Imaging is a key element in the detection of postoperative complications. So far, limited data is available regarding the best radiologic approach to monitor children after liver transplantation. OBJECTIVE: To harmonize the imaging of pediatric liver transplantation, the European Society of Pediatric Radiology Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phases. This paper reports the responses related to postoperative imaging. MATERIALS AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted, and 22 institutions from 11 countries returned the survey. RESULTS: All sites commence ultrasound (US) monitoring within 24 h after liver transplantation. Monitoring frequency varies across sites, ranging from every 8 h to 72 h in early, and from daily to sporadic use in late postoperative phases. Predefined US protocols are used by 73% of sites. This commonly includes gray scale, color Doppler, and quantitative flow assessment. Alternative flow imaging techniques, contrast-enhanced US, and elastography are applied at 31.8%, 18.2%, and 63.6% of sites, respectively. Computed tomography is performed at 86.4% of sites when clarification is needed. Magnetic resonance imaging is used for selected cases at 36.4% of sites, mainly for assessment of biliary abnormalities or when blood tests are abnormal. CONCLUSION: Diagnostic imaging is extensively used for postoperative surveillance of children after liver transplantation. While US is generally prioritized, substantial differences were noted in US protocol, timing, and monitoring frequency. The study highlights potential areas for future optimization and standardization of imaging, essential for conducting multicenter studies.

Myocardial strain assessment in the human fetus by cardiac MRI using Doppler ultrasound gating and feature tracking.

OBJECTIVES: Assessment of myocardial strain by feature tracking magnetic resonance imaging (FT-MRI) in human fetuses with and without congenital heart disease (CHD) using cardiac Doppler ultrasound (DUS) gating. METHODS: A total of 43 human fetuses (gestational age 28-41 weeks) underwent dynamic cardiac MRI at 3 T. Cine balanced steady-state free-precession imaging was performed using fetal cardiac DUS gating. FT-MRI was analyzed using dedicated post-processing software. Endo- and epicardial contours were manually delineated from fetal cardiac 4-chamber views, followed by automated propagation to calculate global longitudinal strain (GLS) of the left (LV) and right ventricle (RV), LV radial strain, and LV strain rate. RESULTS: Strain assessment was successful in 38/43 fetuses (88%); 23 of them had postnatally confirmed diagnosis of CHD (e.g., coarctation, transposition of great arteries) and 15 were heart healthy. Five fetuses were excluded due to reduced image quality. In fetuses with CHD compared to healthy controls, median LV GLS (- 13.2% vs. - 18.9%; p < 0.007), RV GLS (- 7.9% vs. - 16.2%; p < 0.006), and LV strain rate (1.4 s-1 vs. 1.6 s-1; p < 0.003) were significantly higher (i.e., less negative). LV radial strain was without a statistically significant difference (20.7% vs. 22.6%; p = 0.1). Bivariate discriminant analysis for LV GLS and RV GLS revealed a sensitivity of 67% and specificity of 93% to differentiate between fetuses with CHD and healthy fetuses. CONCLUSION: Myocardial strain was successfully assessed in the human fetus, performing dynamic fetal cardiac MRI with DUS gating. Our study indicates that strain parameters may allow for differentiation between fetuses with and without CHD. CLINICAL RELEVANCE STATEMENT: Myocardial strain analysis by cardiac MRI with Doppler ultrasound gating and feature tracking may provide a new diagnostic approach for evaluation of fetal cardiac function in congenital heart disease. KEY POINTS: • MRI myocardial strain analysis has not been performed in human fetuses so far. • Myocardial strain was assessed in human fetuses using cardiac MRI with Doppler ultrasound gating. • MRI myocardial strain may provide a new diagnostic approach to evaluate fetal cardiac function.

Child-centredness in paediatric magnetic resonance imaging: Information needs and experiences of children requiring magnetic resonance imaging and their parents.

BACKGROUND: Assessing patient experiences is essential to provide high quality health-care. The objectives of this study were to examine (1) child- and parent-reported information status before magnetic resonance imaging (MRI), (2) experiences during an MRI and (3) needs and suggestions for improvement. METHODS: Children (≥8 years) and parents answered questionnaires (before and after planned MRI examination) covering mental condition, information status/needs, preparation for MRI, and potential stressors. Before MRI n = 132 accompanying parents and n = 91 children provided data (after MRI: n = 93 parents; n = 71 children). The mean age of the children undergoing MRI was 10.5 years (SD = 4.9). Children had on average seven previous MRIs before our survey (Range: 1-33). Twenty-three percent of the children were to be sedated during the examination. RESULTS: Parents and children reported low to medium levels of burden, high knowledge and high information status. For the children, most stressful factors during the MRI were boredom and noise. Main information resources were the radiologists or self-searches. Parents of children with their first MRI reported higher anxiety levels in their children and stated a worse information status (main information source: self-searches/internet). Parents reported needs regarding organization of the MRI (e.g., reduction of waiting times), age-appropriate information and communication during the MRI. Children wished to visit the MRI room prior the examination (44%) and to get more information (44%). Children suggested improvements such as better sound quality of films/music, more transparency about the procedure, being in contact with a parent and shorter examinations. CONCLUSION: Results suggest that preparation, information provision and care is perceived positively by families. Needs and suggestions for improvement were, for example, higher transparency of procedures, better communication and reduction of stress. Findings indicate that preparation in routine-care should follow an individualized child-focused approach, should focus on families without previous MRIs and should address children with high anxiety levels.

European Society of Pediatric Radiology survey of perioperative imaging in pediatric liver transplantation: (1) pre-transplant evaluation.

BACKGROUND: Liver transplantation is the state-of-the-art curative treatment in end-stage liver disease. Imaging is a key element for successful organ-transplantation to assist surgical planning. So far, only limited data regarding the best radiological approach to prepare children for liver transplantation is available. OBJECTIVES: In an attempt to harmonize imaging surrounding pediatric liver transplantation, the European Society of Pediatric Radiology (ESPR) Abdominal Taskforce initiated a survey addressing the current status of imaging including the pre-, intra-, and postoperative phase. This paper reports the responses on preoperative imaging. MATERIAL AND METHODS: An online survey, initiated in 2021, asked European centers performing pediatric liver transplantation 48 questions about their imaging approach. In total, 26 centers were contacted and 22 institutions from 11 countries returned the survey. From 2018 to 2020, the participating centers collectively conducted 1,524 transplantations, with a median of 20 transplantations per center per annum (range, 8-60). RESULTS: Most sites (64%) consider ultrasound their preferred modality to define anatomy and to plan surgery in children before liver transplantation, and additional cross-sectional imaging is only used to answer specific questions (computed tomography [CT], 90.9%; magnetic resonance imaging [MRI], 54.5%). One-third of centers (31.8%) rely primarily on CT for pre-transplant evaluation. Imaging protocols differed substantially regarding applied CT scan ranges, number of contrast phases (range 1-4 phases), and applied MRI techniques. CONCLUSION: Diagnostic imaging is generally used in the work-up of children before liver transplantation. Substantial differences were noted regarding choice of modalities and protocols. We have identified starting points for future optimization and harmonization of the imaging approach to multicenter studies.

Evaluation of Artifact Appearance and Burden in Pediatric Brain Tumor MR Imaging with Compressed Sensing in Comparison to Conventional Parallel Imaging Acceleration.

Clinical magnetic resonance imaging (MRI) aims for the highest possible image quality, while balancing the need for acceptable examination time, reasonable signal-to-noise ratio (SNR), and lowest artifact burden. With a recently introduced imaging acceleration technique, compressed sensing, the acquisition speed and image quality of pediatric brain tumor exams can be improved. However, little attention has been paid to its impact on method-related artifacts in pediatric brain MRI. This study assessed the overall artifact burden and artifact appearances in a standardized pediatric brain tumor MRI by comparing conventional parallel imaging acceleration with compressed sensing. This showed that compressed sensing resulted in fewer physiological artifacts in the FLAIR sequence, and a reduction in technical artifacts in the 3D T1 TFE sequences. Only a slight difference was noted in the T2 TSE sequence. A relatively new range of artifacts, which are likely technique-related, was noted in the 3D T1 TFE sequences. In conclusion, by equipping a basic pediatric brain tumor protocol for 3T MRI with compressed sensing, the overall burden of common artifacts can be reduced. However, attention should be paid to novel compressed-sensing-specific artifacts.

Recommendations for endotracheal tube insertion depths in children.

BACKGROUND: Endotracheal tube (ETT) malposition is frequent in paediatric intubation. The current recommendations for ETT insertion depths are based on formulae that hold various limitations. This study aimed to develop age-based, weight-based and height-based curve charts and tables for ETT insertion depth recommendations in children. METHODS: In this retrospective single-centre study, we determined the individual optimal ETT insertion depths in paediatric patients by evaluating postintubation radiographic images. Age-based, weight-based and height-based ETT insertion depth recommendations were developed using regression analysis. We compared the insertion depths predicted by the models with previously published formulae. RESULTS: Intubations of 167 children (0-17.9 years) were analysed. Best-fit curves generated with logistic regression analysis revealed R2 values between 0.784 and 0.880. The insertion depths predicted by the models corresponded well with published age-based and height-based formulae. However, they demonstrated the unsuitability of weight-related linear formulae to predict ETT depth in children. CONCLUSION: The recommendations developed in this study facilitate a fast and accurate determination of recommended ETT insertion depths in children. Our recommendations provide greater accuracy than previously published formulae and demonstrate that weight-related linear formulae are unsuitable for predicting ETT depth in children.

Impact of probe-induced abdominal compression on two-dimensional shear wave elastography measurement of split liver transplants in children.

PURPOSE: To evaluate the effect of probe-induced abdominal compression of split liver transplants (SLT) in children on 2D-shear wave elastography (SWE) values. MATERIALS AND METHODS: Data from 11 children (4.7 ±â€Š4.8 years) who had undergone SLT and SWE were evaluated retrospectively. Elastograms were obtained with probes placed in an epigastric, midline position on the abdominal wall, with no and slight compression, using convex and linear transducers. For each identically positioned probe and condition, 12 serial elastograms were obtained and the SLT diameter was measured. Liver stiffness and degree of SLT compression were compared. RESULTS: Slight probe pressure resulted in SLT compression, with a shorter distance between the cutis and the posterior margin of the liver transplant than in the measurement with no pressure (curved array, 5.0 ±â€Š1.1 vs. 5.9 ±â€Š1.3 cm, mean compression 15 %±â€Š8 %; linear array, 4.7 ±â€Š0.9 vs. 5.3 ±â€Š1.0 cm, mean compression 12 %±â€Š8 %; both p < 0.0001). The median liver stiffness was significantly greater with slight pressure than with no pressure (curved transducer, 13.38 ±â€Š3.0 vs. 7.02 ±â€Š1.7 kPa, p < 0.0001; linear transducer, 18.53 ±â€Š7.1 vs. 9.03 ±â€Š1.5 kPa, p = 0.0003). CONCLUSION: Slight abdominal compression can significantly increase SWE values in children with left-lateral SLT. To obtain meaningful results and reduce operator dependency in free-hand examinations, probe pressure must be controlled carefully. KEY POINTS: · Probe-induced compression can increase elastography values in split liver transplants in children. · In free-hand examination, probe pressure must be controlled carefully. · Pressure loading can be determined indirectly by the anteroposterior transplant diameter. CITATION FORMAT: · Groth M, Fischer L, Herden U et al. Impact of probe-induced abdominal compression on two-dimensional shear wave elastography measurement of split liver transplants in children. Fortschr Röntgenstr 2023; 195: 905 - 912.

Recommendations for Peripherally Inserted Central Catheter Insertion Depths in Neonates.

OBJECTIVE: Malposition of peripherally inserted central catheters (PICCs) is common. Recommendations for PICC insertion depths are scarce and comprise complex equations. This study aimed to develop diagrams and tables for the recommendation of PICC insertion depths in neonates based on anthropometric parameters. STUDY DESIGN: In this retrospective single-center study, the individual optimal PICC insertion depths were correlated with body weight, length, and head circumference. Using linear regression analysis, line charts and tables for the recommendation of PICC insertion depth were generated and compared with previously published recommendations. RESULT: PICC insertion depths of 204 infants (gestational age at PICC installation: 24.0-44.9 weeks) with 131 (64%) PICC in the upper extremities and 73 (36%) in the lower extremities were analyzed. Linear logistic regression models revealed R2 values between 0.387 and 0.884. CONCLUSION: The charts and table developed in this study enable a fast and accurate determination of recommended PICC insertion depths in neonates.

Effects of Infantile Hypophosphatasia on Human Dental Tissue.

Hypophosphatasia (HPP) is an inherited, systemic disorder, caused by loss-of-function variants of the ALPL gene encoding the enzyme tissue non-specific alkaline phosphatase (TNSALP). HPP is characterized by low serum TNSALP concentrations associated with defective bone mineralization and increased fracture risk. Dental manifestations have been reported as the exclusive feature (odontohypophosphatasia) and in combination with skeletal complications. Enzyme replacement therapy (asfotase alfa) has been shown to improve respiratory insufficiency and skeletal complications in HPP patients, while its effects on dental status have been understudied to date. In this study, quantitative backscattered electron imaging (qBEI) and histological analysis were performed on teeth from two patients with infantile HPP before and during asfotase alfa treatment and compared to matched healthy control teeth. qBEI and histological methods revealed varying mineralization patterns in cementum and dentin with lower mineralization in HPP. Furthermore, a significantly higher repair cementum thickness was observed in HPP compared to control teeth. Comparison before and during treatment showed minor improvements in mineralization and histological parameters in the patient when normalized to matched control teeth. HPP induces heterogeneous effects on mineralization and morphology of the dental status. Short treatment with asfotase alfa slightly affects mineralization in cementum and dentin. Despite HPP being a rare disease, its mild form occurs at higher prevalence. This study is of high clinical relevance as it expands our knowledge of HPP and dental involvement. Furthermore, it contributes to the understanding of dental tissue treatment, which has hardly been studied so far.

Liver cirrhosis in children - the role of imaging in the diagnostic pathway.

Liver cirrhosis in children is a rare disease with multifactorial causes that are distinct from those in adults. Underlying reasons include cholestatic, viral, autoimmune, hereditary, metabolic and cardiac disorders. Early detection of fibrosis is important as clinical stabilization or even reversal of fibrosis can be achieved in some disorders with adequate treatment. This article focuses on the longitudinal evaluation of children with chronic liver disease with noninvasive imaging tools, which play an important role in detecting cirrhosis, defining underlying causes, grading fibrosis and monitoring patients during follow-up. Ultrasound is the primary imaging modality and it is used in a multiparametric fashion. Magnetic resonance imaging and computed tomography are usually applied second line for refined tissue characterization, clarification of nodular lesions and full delineation of abdominal vessels, including portosystemic communications.

Non-invasive pediatric cardiac imaging-current status and further perspectives.

BACKGROUND: Non-invasive cardiac imaging has a growing role in diagnosis, differential diagnosis, therapy planning, and follow-up in children and adolescents with congenital and acquired cardiac diseases. This review is based on a systematic analysis of international peer-reviewed articles and additionally presents own clinical experiences. It provides an overview of technical advances, emerging clinical applications, and the aspect of artificial intelligence. MAIN BODY: The main imaging modalities are echocardiography, CT, and MRI. For echocardiography, strain imaging allows a novel non-invasive assessment of tissue integrity, 3D imaging rapid holistic overviews of anatomy. Fast cardiac CT imaging new techniques-especially for coronary assessment as the main clinical indication-have significantly improved spatial and temporal resolution in adjunct with a major reduction in ionizing dose. For cardiac MRI, assessment of tissue integrity even without contrast agent application by mapping sequences is a major technical breakthrough. Fetal cardiac MRI is an emerging technology, which allows structural and functional assessment of fetal hearts including even 4D flow analyses. Last but not least, artificial intelligence will play an important role for improvements of data acquisition and interpretation in the near future. CONCLUSION: Non-invasive cardiac imaging plays an integral part in the workup of children with heart disease. In recent years, its main application congenital heart disease has been widened for acquired cardiac diseases.

Recommendations for nasotracheal tube insertion depths in neonates.

Background: Endotracheal tube (ETT) malposition is common in neonatal intubation. Recommendations for ETT insertion depths predominantly address orotracheal intubation. The aim of this study was to develop gestational age-, weight-, and length-based curve charts and tables for nasotracheal ETT insertion depth recommendations in neonates. Method: In this retrospective single-center study, the individual optimal ETT insertion depths in neonates were determined by evaluating postintubation radiographic images. Gestational age-, weight-, and length-based best-fit curves and tables were generated using regression analysis to calculate related ETT insertion depths. The insertion depths predicted by the models were compared with previously published recommendations. Results: We analyzed intubations of 178 neonates (gestational age range at intubation: 23.7-43.0 weeks). Applying sigmoidal logistic regression models, curves, and tables revealed R 2 values between 0.766 and 0.837. The insertion depths predicted by the models revealed certain deviations when compared with four previously published recommendations for nasotracheal ETT depth estimation in neonates. Conclusion: The charts and tables developed in this study enable a fast and accurate determination of recommended nasotracheal ETT insertion depths in neonates.