RESUMEN
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.
Asunto(s)
Corazón Fetal , Cardiopatías Congénitas , Humanos , Femenino , Embarazo , Cardiopatías Congénitas/diagnóstico por imagen , Corazón Fetal/diagnóstico por imagen , Imagen por Resonancia Cinemagnética/métodos , Imagen por Resonancia Magnética/métodos , Técnicas de Imagen Sincronizada Cardíacas/métodosRESUMEN
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.
Asunto(s)
Trasplante de Hígado , Radiología , Niño , Humanos , Ultrasonografía , Tomografía Computarizada por Rayos X , Imagen por Resonancia Magnética/métodosRESUMEN
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.
Asunto(s)
Trasplante de Hígado , Radiología , Niño , Humanos , Ultrasonografía , Radiografía , Complicaciones Posoperatorias/diagnóstico por imagenRESUMEN
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.
Asunto(s)
Trasplante de Hígado , Radiología , Niño , Humanos , Ultrasonografía , Imagen por Resonancia Magnética/métodos , Ultrasonografía Doppler , Complicaciones Posoperatorias/diagnóstico por imagenRESUMEN
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.
Asunto(s)
Imagen por Resonancia Magnética , Padres , Niño , Humanos , Encuestas y Cuestionarios , Calidad de la Atención de Salud , Espectroscopía de Resonancia MagnéticaRESUMEN
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.
Asunto(s)
Canal Anal , Esófago , Cardiopatías Congénitas , Síndrome de Kasabach-Merritt , Riñón , Deformidades Congénitas de las Extremidades , Tráquea , Humanos , Síndrome de Kasabach-Merritt/complicaciones , Síndrome de Kasabach-Merritt/diagnóstico , Síndrome de Kasabach-Merritt/terapia , Masculino , Recién Nacido , Deformidades Congénitas de las Extremidades/complicaciones , Deformidades Congénitas de las Extremidades/diagnóstico , Cardiopatías Congénitas/complicaciones , Cardiopatías Congénitas/diagnóstico , Riñón/anomalías , Tráquea/anomalías , Tráquea/cirugía , Canal Anal/anomalías , Canal Anal/cirugía , Esófago/anomalías , Sirolimus/uso terapéutico , Trombocitopenia/complicaciones , Trombocitopenia/terapia , Trombocitopenia/diagnóstico , Trombocitopenia/congénito , Columna Vertebral/anomalíasRESUMEN
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.
Asunto(s)
Calcinosis , Hipofosfatasia , Desmineralización Dental , Humanos , Hipofosfatasia/complicaciones , Fosfatasa Alcalina/genética , Calcificación Fisiológica , Calcinosis/complicaciones , Desmineralización Dental/complicaciones , Desmineralización Dental/tratamiento farmacológicoRESUMEN
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.
Asunto(s)
Diagnóstico por Imagen de Elasticidad , Hepatopatías , Adulto , Humanos , Niño , Cirrosis Hepática/diagnóstico por imagen , Hepatopatías/patología , Imagen por Resonancia Magnética/métodos , Tomografía Computarizada por Rayos X/efectos adversos , Ultrasonografía , Diagnóstico por Imagen de Elasticidad/métodos , Hígado/diagnóstico por imagenRESUMEN
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.
Asunto(s)
Intubación Intratraqueal , Niño , Humanos , Estudios Retrospectivos , Intubación Intratraqueal/métodos , Análisis de RegresiónRESUMEN
BACKGROUND: Medical simulation training requires realistic simulators with high fidelity. This prospective multi-center study investigated anatomic precision, physiologic characteristics, and fidelity of four commercially available very low birth weight infant simulators. METHODS: We measured airway angles and distances in the simulators Premature AirwayPaul (SIMCharacters), Premature Anne (Laerdal Medical), Premie HAL S2209 (Gaumard), and Preterm Baby (Lifecast Body Simulation) using computer tomography and compared these to human cadavers of premature stillbirths. The simulators' physiologic characteristics were tested, and highly experienced experts rated their physical and functional fidelity. RESULTS: The airway angles corresponded to those of the reference cadavers in three simulators. The nasal inlet to glottis distance and the mouth aperture to glottis distance were only accurate in one simulator. All simulators had airway resistances up to 20 times higher and compliances up to 19 times lower than published reference values. Fifty-six highly experienced experts gave three simulators (Premature AirwayPaul: 5.1 ± 1.0, Premature Anne 4.9 ± 1.1, Preterm Baby 5.0 ± 1.0) good overall ratings and one simulator (Premie HAL S2209: 2.8 ± 1.0) an unfavorable rating. CONCLUSION: The simulator physiology deviated significantly from preterm infants' reference values concerning resistance and compliance, potentially promoting a wrong ventilation technique. IMPACT: Very low birth weight infant simulators showed physiological properties far deviating from corresponding patient reference values. Only ventilation with very high peak pressure achieved tidal volumes in the simulators, as aimed at in very low birth weight infants, potentially promoting a wrong ventilation technique. Compared to very low birth weight infant cadavers, most tested simulators accurately reproduced the anatomic angular relationships, but their airway dimensions were relatively too large for the represented body. The more professional experience the experts had, the lower they rated the very low birth weight infant simulators.
Asunto(s)
Recien Nacido Prematuro , Entrenamiento Simulado , Cadáver , Humanos , Lactante , Recién Nacido , Recién Nacido de muy Bajo Peso , Estudios Prospectivos , Entrenamiento Simulado/métodosRESUMEN
The 12 years old male patient presented here suffers from neuronal ceroid lipofuscinoses 2 (CLN2) (MIM# 204500) and receives intracerebroventricular enzyme replacement therapy (ICV-ERT) every 14 days. After the emergence of the coronavirus disease 2019 (COVID-19) pandemic, routine care of children and adolescents with rare chronic diseases has become challenging. Although, in general, children do not develop severe COVID-19, when severe acute respiratory syndrome coronavirus 2 infection was detected by polymerase chain reaction-screening examination in our CLN2 patient before hospital admission for ICV-ERT, he was regarded to be at risk. Upon diagnosis, the patient developed respiratory deterioration symptoms and was admitted to our pediatric intensive care unit to receive oxygen, remdesivir, and steroids. As far as we know, this is the first CLN2 patient receiving intraventricular enzyme therapy with COVID-19 who required intensive care treatment and specific therapy.
Asunto(s)
COVID-19 , Lipofuscinosis Ceroideas Neuronales , Adolescente , COVID-19/complicaciones , Niño , Dipeptidil-Peptidasas y Tripeptidil-Peptidasas/uso terapéutico , Terapia de Reemplazo Enzimático , Humanos , Masculino , Lipofuscinosis Ceroideas Neuronales/complicaciones , Lipofuscinosis Ceroideas Neuronales/tratamiento farmacológico , Oxígeno , Tripeptidil Peptidasa 1RESUMEN
Anorectal and cloacal malformations are a broad mix of congenital abnormalities related to the distal rectum and anus. Confusion exists between all the forms in this large and heterogeneous group. The spectrum includes everything from anal stenosis, ventral anus, anal atresia (with and without fistula) and the full spectrum of cloacal malformations. Imaging in these conditions is done through the whole armamentarium of radiologic modalities, with very different imaging strategies seen across the centres where these conditions are managed. In 2017, the European Society of Paediatric Radiology (ESPR) abdominal imaging task force issued recommendations on the imaging algorithm and standards for imaging anorectal malformations. This was followed by further letters and clarifications together with an active multispecialty session on the different imaging modalities for anorectal malformations at the 2018 ESPR meeting in Berlin. Through this paper, the abdominal task force updates its guidelines and recommended imaging algorithm for anorectal malformations.
Asunto(s)
Malformaciones Anorrectales , Ano Imperforado , Radiología , Canal Anal/anomalías , Canal Anal/diagnóstico por imagen , Malformaciones Anorrectales/diagnóstico por imagen , Ano Imperforado/diagnóstico por imagen , Niño , Humanos , Recto/anomalías , Recto/diagnóstico por imagenRESUMEN
Ischemias in the extremities are rather rare in the neonatal period. Both intrauterine and postnatal factors can cause ischemias. We present the diagnostic procedure and the course in a female very low birthweight (VLBW) infant with transient ischemia in the lower extremity.
Asunto(s)
Recién Nacido de muy Bajo Peso , Pierna , Enfermedad Aguda , Femenino , Humanos , Lactante , Recién Nacido , Isquemia/diagnóstico , Isquemia/terapia , Extremidad InferiorRESUMEN
OBJECTIVES: To assess epiphyseal growth plate closure of the knee for forensic age estimation using an ultrasound (US)-based method and to compare the findings with MRI. METHODS: Thirty-three healthy male individuals (age, 14.4-19.3 years) were prospectively evaluated for epiphyseal growth plate closure of the right knee by recordings of two-dimensional US volumes and a high-resolution T1-weighted MRI sequence. The degree of epiphyseal growth plate closure was rated independently by two readers for each method using a modality specific three-point scale that differentiates between an open physis (S1), a partially closed physis (S2), and a closed physis (S3). RESULTS: The inter-rater agreement was high for the US (Cohen's kappa (CK): femur 95.2%, tibia 81.3%, fibula 86.3%) and the MRI method (CK: femur 70.2%, tibia 90.8%, fibula 79.8%). The degree of growth plate closure associated positively with advancing age. The US system showed a clearer separation of median ages with lower overlap than the MRI system. Open growth plates on minors (< S3 on femur and tibia) were identified by US with higher sensitivity (1.0 vs. 0.7) and slightly lower specificity (0.7 vs. 0.85) compared with MRI. The examination time was substantially shorter on US than on MRI (2.65 ± 0.91 min vs. 24.72 ± 2.72 min; p < 0.001). CONCLUSIONS: The US method for evaluation of growth plate closure of the knee can reliably assign male individuals to different ossification stages and identifies minors with high accuracy. More studies with larger numbers are needed to further evaluate this method. KEY POINTS: ⢠US is feasible to determine the degree of epiphyseal growth plate closure of the knee, shows a high degree of reliability, and is comparable to MRI. ⢠US of the knee can detect open growth plates on male minors with high accuracy. ⢠US of the knee may be used as a fast, non-invasive imaging tool for forensic age estimation to identify male minors.
Asunto(s)
Epífisis , Articulación de la Rodilla , Adolescente , Adulto , Epífisis/diagnóstico por imagen , Humanos , Articulación de la Rodilla/diagnóstico por imagen , Imagen por Resonancia Magnética , Masculino , Reproducibilidad de los Resultados , Tibia , Adulto JovenRESUMEN
OBJECTIVES: To evaluate systolic cardiac dysfunction in paediatric MFS patients with chest wall deformity using cardiac magnetic resonance (CMR) imaging and feature-tracking strain analysis. METHODS: Forty paediatric MFS patients (16 ± 3 years, range 8-22 years) and 20 age-matched healthy controls (16 ± 4 years, range 11-24 years) were evaluated retrospectively. Biventricular function and volumes were determined using cine sequences. Feature-tracking CMR was used to assess global systolic longitudinal (GLS), circumferential (GCS) and radial strain (GRS). A dedicated balanced turbo field echo sequence was used to quantify chest wall deformity by measuring the Haller index (HI). RESULTS: LV volumes and ejection fraction (EF) were similar in MFS patients and controls. There was a trend for lower right ventricular (RV) volume (75 ± 17 vs. 81 ± 10 ml/m2, p = 0.08), RV stroke volume (41 ± 12 vs. 50 ± 5 ml/m2, p < 0.001) and RVEF (55 ± 10 vs. 62 ± 6%, p < 0.01) in MFS patients. A subgroup of MFS patients had an increased HI compared to controls (4.6 ± 1.7 vs. 2.6 ± 0.3, p < 0.001). They demonstrated a reduced RVEF compared to MFS patients without chest wall deformity (50 ± 11% vs. 58 ± 8%, p = 0.01) and controls (p < 0.001). LV GLS was attenuated when HI ≥ 3.25 (- 16 ± 2 vs. - 18 ± 3%, p = 0.03), but not GCS and GRS. LV GLS (p < 0.01) and GCS (p < 0.0001) were attenuated in MFS patients compared to controls, but not GRS (p = 0.31). RV GLS was attenuated in MFS patients compared to controls (- 21 ± 3 vs. - 23 ± 3%, p < 0.05). CONCLUSION: Chest wall deformity in paediatric MFS patients is associated with reduced RV volume, ejection fraction and GLS. Feature-tracking CMR also indicates impairment of systolic LV function in paediatric MFS patients. KEY POINTS: ⢠Paediatric Marfan patients demonstrate reduced RV volume and ejection fraction compared to healthy controls. ⢠A concordant attenuation in RV global longitudinal strain was observed in Marfan patients, while the RV global circumferential strain was increased, indicating a possible compensatory mechanism. ⢠Subgroup analyses demonstrated alterations in RV ejection fraction and RV/LV global strain parameters, indicating a possible association of severe chest wall deformity with biventricular dysfunction in paediatric Marfan patients.
Asunto(s)
Síndrome de Marfan , Pared Torácica , Adolescente , Adulto , Niño , Humanos , Imagen por Resonancia Cinemagnética , Síndrome de Marfan/complicaciones , Síndrome de Marfan/diagnóstico por imagen , Estudios Retrospectivos , Volumen Sistólico , Pared Torácica/diagnóstico por imagen , Función Ventricular Izquierda , Adulto JovenRESUMEN
Here the impact of donor specific human leukocyte antigen (HLA) class 2 antibodies (DSA cl 2) on long term outcome after liver transplantation (LT) was investigated. Altogether 156 (44 pediatric and 112 adult) LT recipients were included in the study. Graft fibrosis was assessed by liver elastography and biopsy. DSA cl 2 were determined by Luminex technology. 46% of LT recipients were positive for DSA cl 2 after a median follow-up of 15 years. In the multivariate analysis DSA cl 2 were significantly associated with immunosuppressive monotherapy (OR 5.42; 95% CI: 1.02-28.90; p = .048). Compared to DSA cl 2 negative patients, positive recipients had significantly more graft fibrosis based on the liver stiffness (mean 9.4 ± 9.0 kPa vs. 6.5 ± 6.3 kPa; p < .002) and fibrosis stages determined by liver elastography (p = .016) and the performed liver biopsies (p = .002). Also, a significantly higher incidence of chronic rejections (11% vs. 2%; p = .045) and graft losses (6% vs. 0%; p = .043) were found. In the multivariate regression analysis DSA cl 2 were significantly associated with graft fibrosis (OR 4.57; 95% CI 1.59-13.10; p = .005). So, these data suggest that development of DSA cl 2 occurs more often with immunosuppressive monotherapy and may ultimately result in chronic rejection and graft fibrosis.
Asunto(s)
Trasplante de Hígado , Adulto , Niño , Fibrosis , Rechazo de Injerto/etiología , Supervivencia de Injerto , Antígenos HLA , Prueba de Histocompatibilidad , Humanos , Isoanticuerpos , Trasplante de Hígado/efectos adversos , Estudios RetrospectivosRESUMEN
PURPOSE: The aim of this study was to assess the impact of arm position in computed tomography (CT) of the clavicle performed for forensic age estimation on clavicular position, image noise, and radiation dose. METHODS AND MATERIALS: Forty-seven CT scans of the medial clavicular epiphysis performed for forensic age estimation were conducted with either hands and arms held upwards (CTHU, 28 persons) or positioned at the body (CTHD, 19 persons). Presets were identical for both positions (70 mAs/140 kVp; Brilliance iCT, Philips). Each CT scan was reconstructed with an iterative algorithm (i-Dose 4) and evaluated at the middle of the sternoclavicular joint. Clavicular angle was measured on a.p. topograms in relation to a horizontal line. Quantitative image noise was measured in air at the level of medial clavicular epiphysis. Effective dose and scan length were recorded. RESULTS: Hands-up position compared with hands-down position resulted in a lower lateral body diameter (CTHU 41.1 ± 3.6 cm vs. CTHD 44.6 ± 3.1 cm; P = 0.03), a reduced quantitative image noise (CTHU: 39.5 ± 9.2; CTHD: 46.2 ± 8.3; P = 0.02), and lower CTDIvol (5.1 ± 1.4 mGy vs. 6.7 ± 1.8 mGy; P = 0.001). Scan length was longer in patients examined with hands up (HU: 8.5 ± 3.4 cm; HD: 6.2 ± 2.1 cm; P = 0.006). Mean effective dose for CTHU was 0.79 ± 0.32 mSv compared with 0.95 ± 0.38 mSv in CTHD (P = 0.12). Clavicular angle was 17° ± 6° in patients with hands down and 32° ± 7° in patients with hands up (P < 0.001). CONCLUSION: By elevated arm positioning, the image quality of clavicular CT scans can be improved while maintaining radiation dose compared with hands down. Clavicular position differs according to the hand position. Thus, positioning patients with elevated hands is advisable for forensic clavicular CT examinations, but multiplanar CT reconstructions should be adjusted to clavicular position and scan length should be reduced to a minimum.
Asunto(s)
Determinación de la Edad por el Esqueleto/métodos , Clavícula/diagnóstico por imagen , Postura , Tomografía Computarizada por Rayos X/métodos , Adolescente , Algoritmos , Epífisis/crecimiento & desarrollo , Humanos , Masculino , Dosis de Radiación , Adulto JovenRESUMEN
Age estimation is a crucial element of forensic medicine to assess the chronological age of living individuals without or lacking valid legal documentation. Methods used in practice are labor-intensive, subjective, and frequently comprise radiation exposure. Recently, also non-invasive methods using magnetic resonance imaging (MRI) have evaluated and confirmed a correlation between growth plate ossification in long bones and the chronological age of young subjects. However, automated and user-independent approaches are required to perform reliable assessments on large datasets. The aim of this study was to develop a fully automated and computer-based method for age estimation based on 3D knee MRIs using machine learning. The proposed solution is based on three parts: image-preprocessing, bone segmentation, and age estimation. A total of 185 coronal and 404 sagittal MR volumes from Caucasian male subjects in the age range of 13 and 21 years were available. The best result of the fivefold cross-validation was a mean absolute error of 0.67 ± 0.49 years in age regression and an accuracy of 90.9%, a sensitivity of 88.6%, and a specificity of 94.2% in classification (18-year age limit) using a combination of convolutional neural networks and tree-based machine learning algorithms. The potential of deep learning for age estimation is reflected in the results and can be further improved if it is trained on even larger and more diverse datasets.
Asunto(s)
Determinación de la Edad por el Esqueleto/métodos , Aprendizaje Profundo , Procesamiento de Imagen Asistido por Computador , Rodilla/diagnóstico por imagen , Aprendizaje Automático , Imagen por Resonancia Magnética , Redes Neurales de la Computación , Adolescente , Algoritmos , Automatización , Humanos , Imagenología Tridimensional , Rodilla/crecimiento & desarrollo , Masculino , Adulto JovenRESUMEN
Since Francis Fontan first introduced the eponymous technique, the Fontan procedure, this type of surgical palliation has allowed thousands of children affected by specific heart malformations to reach adulthood. Nevertheless, abdominal, thoracic, lymphatic and neurologic complications are the price that is paid by these patients. Our review focuses on Fontan-associated liver disease; the purpose is to summarize the current understanding of its physiopathology, the aim of follow-up and the specific radiologic follow-up performed in Europe. Finally, we as members of the Abdominal Task Force of the European Society of Paediatric Radiology propose a consensus-based imaging follow-up algorithm.
Asunto(s)
Procedimiento de Fontan , Cardiopatías Congénitas , Hepatopatías , Radiología , Adulto , Niño , Procedimiento de Fontan/efectos adversos , Cardiopatías Congénitas/diagnóstico por imagen , Cardiopatías Congénitas/cirugía , Humanos , Hígado/patología , Cirrosis Hepática/patología , Hepatopatías/patología , Complicaciones Posoperatorias/patologíaRESUMEN
Fontan surgery is a life-saving procedure for newborns with complex cardiac malformations, but it originates complications in different organs. The liver is also affected, with development of fibrosis and sometimes cirrhosis and hepatocellular carcinoma. There is no general agreement on how to follow-up these children for the development of liver disease. To understand the current practice on liver follow-up, we invited members of the European Society of Paediatric Radiology (ESPR) to fill out an online questionnaire. The survey comprised seven questions about when and how liver follow-up is performed on Fontan patients. While we found some agreement on the use of US as screening tool, and of MRI for nodule characterization, the discrepancies on timing and the lack of a shared protocol make it currently impossible to compare data among centers.