Interpretation difficulty of normal versus abnormal radiographs using a pediatric example

Background: Radiograph teaching files are usually dominated by abnormal cases, implying that normal radiographs are easier to interpret. Our main objective was to compare the interpretation difficulty of normal versus abnormal radiographs of a set of common pediatric radiographs.Methods: We developed a 234-item digital case bank of pediatric ankle radiographs, recruited a convenience sample of participants, and presented the cases to each participant who then classified the cases as normal or abnormal. We determined and contrasted the interpretation difficulty of the normal and abnormal x-rays items using Rasch Measurement Theory. We also identified case features that were associated with item difficulty.Results: 139 participants (86 medical students, 7 residents, 29 fellows, 5 emergency physicians, and 3 radiologists) rated a minimum of 50 cases each, which resulted in 16,535 total ratings. Abnormal cases were more difficult (+0.99 logits) than were normal ones (-0.58 logits), difference 1.57 logits (95% CI 1.2, 2.0), but there was considerable overlap in difficulty scores. Patient variables associated with a more difficult normal radiograph included younger patient age (β = -0.16, 95% CI -0.22, -0.10), history of distal fibular tenderness (β = 0.55, 95% CI 0.17, 0.93), and presence of a secondary ossification centre (β = 0.84, 95% CI 0.27, 1.41).Conclusions: While abnormal images were more difficult to interpret, normal images did show a range of interpretation difficulties. Including a significant proportion of normal cases may be of benefit to learners

A novel biallelic loss-of-function variant in DAND5 causes heterotaxy syndrome

Funding J.A.B. acknowledges funding from iNOVA4Health-UID/Multi/04462/2013, a program financially supported by Fundação para a Ciência e Tecnologia/Ministério da Educação e Ciência through national funds and cofunded by Fundo Europeu de Desenvolvimento Regional (FEDER) under the PT2020 Partnership Agreement.The majority of heterotaxy cases do not obtain a molecular diagnosis, although pathogenic variants in more than 50 genes are known to cause heterotaxy. A heterozygous missense variant in DAND5, a nodal inhibitor, which functions in early development for establishment of right-left patterning, has been implicated in heterotaxy. Recently, the first case was reported of a DAND5 biallelic loss-of-function (LoF) variant in an individual with heterotaxy. Here, we describe a second unrelated individual with heterotaxy syndrome and a homozygous frameshift variant in DAND5 (NM_152654.2:c.197del [p.Leu66ArgfsTer22]). Using an in vitro assay, we demonstrate that the DAND5 c.197del variant is unable to inhibit nodal signaling when compared with the wild-type expression construct. This work strengthens the genetic and functional evidence for biallelic LoF variants in DAND5 causing an autosomal recessive heterotaxy syndrome.publishersversionpublishe

Computed Tomography With Intravenous Contrast Alone: The Role of Intra‐abdominal Fat on the Ability to Visualize the Normal Appendix in Children

Background Computed tomography ( CT ) with enteric contrast is frequently used to evaluate children with suspected appendicitis. The use of CT with intravenous ( IV ) contrast alone ( CT IV ) may be sufficient, however, particularly in patients with adequate intra‐abdominal fat ( IAF ). Objectives The authors aimed 1) to determine the ability of radiologists to visualize the normal (nondiseased) appendix with CT IV in children and to assess whether IAF adequacy affects this ability and 2) to assess the association between IAF adequacy and patient characteristics. Methods This was a retrospective 16‐center study using a preexisting database of abdominal CT scans. Children 3 to 18 years who had CT IV scan and measured weights and for whom appendectomy history was known from medical record review were included. The sample was chosen based on age to yield a sample with and without adequate IAF . Radiologists at each center reread their site's CT IV scans to assess appendix visualization and IAF adequacy. IAF was categorized as “adequate” if there was any amount of fat completely surrounding the cecum and “inadequate” if otherwise. Results A total of 280 patients were included, with mean age of 10.6 years (range = 3.1 to 17.9 years). All 280 had no history of prior appendectomy; therefore, each patient had a presumed normal appendix. A total of 102 patients (36.4%) had adequate IAF . The proportion of normal appendices visualized with CT IV was 72.9% (95% confidence interval [ CI ] = 67.2% to 78.0%); the proportions were 89% (95% CI  = 81.5% to 94.5%) and 63% (95% CI  = 56.0% to 70.6%) in those with and without adequate IAF (95% CI for difference of proportions = 16% to 36%). Greater weight and older age were strongly associated with IAF adequacy (p < 0.001), with weight appearing to be a stronger predictor, particularly in females. Although statistically associated, there was noted overlap in the weights and ages of those with and without adequate IAF . Conclusions Protocols using CT with IV contrast alone to visualize the appendix can reasonably include weight, age, or both as considerations for determining when this approach is appropriate. However, although IAF will more frequently be adequate in older, heavier patients, highly accurate prediction of IAF adequacy appears challenging solely based on age and weight. Resumen Tomografía Computarizada Únicamente con Contraste Intravenoso: El Papel de la Grasa Intrabadominal en la Capacidad para Visualizar el Apéndice Normal en los Niños Introduction La tomografía computarizada ( TC ) con contraste entérico es usada frecuentemente para evaluar a los niños con sospecha de apendicitis. El uso de la TC únicamente con contraste intravenoso ( TC IV ) puede ser suficiente, especialmente en pacientes con adecuada grasa intrabdominal ( GIA ). Objetivos 1) Determinar la capacidad de los radiólogos para visualizar el apéndice normal (sin enfermedad) con TC IV en niños, y valorar si la cantidad de GIA afecta a esta capacidad; y 2) valorar la asociación entre la idoneidad de la GIA y las características del paciente. Metodología Estudio retrospectivo de 16 hospitales que utilizó una base de datos prexistente de TC abdominales. Se incluyó a los niños entre 3 y 18 años que tenían una TC IV , una medida del peso e historia de apendectomía conocida por la revisión de la historia clínica. La muestra se eligió en base a la edad con el fin de conseguir una muestra con y sin GIA adecuada. Los radiólogos de cada centro releyeron las TC IV de sus centros para valorar la visualización del apéndice y la adecuación de la GIA . La GIA se clasificó como “adecuada” si había cualquier cantidad de grasa completamente alrededor del ciego e “inadecuada” si era de otra manera. Resultados Se incluyeron 280 pacientes, con una media de edad de 10,6 años (rango 3,1 a 17,9 años). Ninguno tenía historia previa de apendectomía; por lo tanto todos los pacientes tuvieron un apéndice presumiblemente normal. Ciento dos pacientes (36,4%) tuvieron GIA adecuada. El porcentaje de apéndices normales visualizados con TC IV fue de 72,9% ( IC 95% = 67,2% a 78,0%); la proporción fue 89% ( IC 95% = 81,5% a 94,5%), y 63% ( IC 95% = 56,0% a 70,6%) en aquéllos con y sin GIA adecuada ( IC 95% para la diferencia de proporciones = 16% a 36%). El mayor peso y la mayor edad se asociaron fuertemente con la adecuación de la GIA (p < 0,001), y el peso resultó ser el mayor factor predictivo, especialmente en mujeres. Aunque se asoció estadísticamente, se vio un solapamiento en los pesos y edades de aquéllos con y sin GIA adecuada. Conclusiones Los protocolos que usan la TC IV para visualizar el apéndice pueden razonablemente incluir el peso, la edad, o ambas como consideraciones para determinar cuándo esta aproximación es apropiada. Sin embargo, aunque la cantidad de GIA será frecuentemente más apropiada en los pacientes más mayores y de mayor peso, la predicción certera de adecuación de GIA es altamente desafiante si se basa sólo en la edad y el peso.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/99695/1/acem12185.pd

Pediatric paraduodenal dermoid cyst: Clinical presentation, minimally invasive management and literature review

AbstractDermoid cysts (mature cystic teratomas) are congenital masses composed of all three germ cell layers: commonly occurring in the head, neck, and gonads and rarely in the abdomen. We present the first documented case of a paraduodenal dermoid cyst in a child, and describe the minimally invasive surgical approach utilized for resection. The patient was an asymptomatic five-month old female diagnosed with a cystic lesion in the posterior mid-abdomen on a prenatal ultrasound, followed up by MRI at three months of age. We proceeded with a laparoscopic resection for both diagnosis and definitive management. Intraoperatively, the lesion was noted to be separate of the pancreas and the stomach, located between the superior mesenteric vessels and splenic vein. It was dissected out with histopathology confirming the diagnosis of a dermoid cyst. We believe that the use of a laparoscopic technique allowed for better post-operative pain control, less overall morbidity and a shorter hospital stay compared to an open approach. Given the significant recurrence rate and possibility of malignant degeneration with incomplete resection, it is imperative to perform a complete resection if this lesion is suspected

New automated fluoroscopic systems for pediatric applications.” J Appl Clin Med Phys 6:88–105

Pediatric patients are at higher risk to the adverse effects from exposure to ionizing radiation than adults. The smaller sizes of the anatomy and the reduced X-ray attenuation of the tissues provide special challenges. The goal of this effort is to investigate strategies for pediatric fluoroscopy in order to minimize the radiation exposure to these individuals, while maintaining effective diagnostic image quality. Modern fluoroscopy systems are often entirely automated and computer controlled. In this paper, various selectable and automated modes are examined to determine the influence of the fluoroscopy parameters upon the patient radiation exposures and image quality. These parameters include variable X-ray beam filters, automatic brightness control programs, starting kilovolt peak levels, fluoroscopic pulse rates, and other factors. Typical values of radiation exposure rates have been measured for a range of phantom thicknesses from 5 cm to 20 cm of acrylic. Other factors that have been assessed include spatial resolution, lowcontrast discrimination, and temporal resolution. The selection menu for various procedures is based upon the examination type, anatomical region, and patient size. For pediatric patients, the automated system can employ additional filtration, special automatic brightness control curves, pulsed fluoroscopy, and other features to reduce the patient radiation exposures without significantly compromising the image quality. The benefits gained from an optimal selection of automated programs and settings for fluoroscopy include ease of operation, better image quality, and lower patient radiation exposures. PACS numbers: 87.59.-e, 87.62.+n Key words: digital fluoroscopy, pediatric radiation exposur

A clinically relevant model of acute respiratory distress syndrome in human-size swine

Despite over 30 years of intensive research for targeted therapies, treatment of acute respiratory distress syndrome (ARDS) remains supportive in nature. With mortality upwards of 30%, a high-fidelity pre-clinical model of ARDS, on which to test novel therapeutics, is urgently needed. We used the Yorkshire breed of swine to induce a reproducible model of ARDS in human-sized swine to allow the study of new therapeutics, from both mechanistic and clinical standpoints. For this, animals were anesthetized, intubated and mechanically ventilated, and pH-standardized gastric contents were delivered bronchoscopically, followed by intravenous infusion of Escherichia coli-derived lipopolysaccharide. Once the ratio of arterial oxygen partial pressure (PaO(2)) to fractional inspired oxygen (F(I)O(2)) had decreased to <150, the animals received standard ARDS treatment for up to 48 h. All swine developed moderate to severe ARDS. Chest radiographs taken at regular intervals showed significantly worse lung edema after induction of ARDS. Quantitative scoring of lung injury demonstrated time-dependent increases in interstitial and alveolar edema, neutrophil infiltration, and mild to moderate alveolar membrane thickening. This pre-clinical model of ARDS in human-sized swine recapitulates the clinical, radiographic and histopathologic manifestations of ARDS, providing a tool to study therapies for this highly morbid lung disease