Aisling Snow

The radiological diagnosis and treatment of renal angiomyolipoma-current status.

Angiomyolipomas (AMLs) are the most common benign renal neoplasm and are often discovered incidentally. Due to both an increase in the use of imaging, as well as advances in imaging technology, they are being increasing identified in the general population. As these lesions are benign, there is good evidence that the majority of them can be safely followed up without treatment. However, there is an increasing wealth of information available suggesting there are individuals with AMLs where prophylactic treatment is indicated to prevent complications such as haemorrhage. In such cases, treatment with radiological interventional techniques with subselective particle embolization has superseded surgical techniques in most cases. Even in emergency cases with catastrophic rupture, prompt embolization may save the patient with the additional benefit of renal salvage. Confident identification of a lesion as an AML is important as its benign nature obviates the need for surgery in most cases. The presence of fat is paramount in the confirmatory identification and characterization of these lesions. Although fat-rich AMLs are easy to diagnose, some lesions are fat poor and it is these cases where newer imaging techniques, such as in-phase and out-of-phase magnetic resonance imaging (MRI) may aid in making a confident diagnosis of AML. In this paper, we comprehensively review the imaging techniques in making a diagnosis of AML, including features of both characteristic lesions as well as atypical lesions. In addition, we discuss current guidelines for follow-up and prophylactic treatment of these lesions, as well as the increasing role that the interventional radiologist has to play in these cases.

Imaging of Budd-Chiari syndrome

Budd-Chiari syndrome occurs when venous outflow from the liver is obstructed. The obstruction may occur at any point from the hepatic venules to the left atrium. The syndrome most often occurs in patients with underlying thrombotic disorders such as polycythemia rubra vera, paroxysmal nocturnal hemoglobinuria and pregnancy. It may also occur secondary to a variety of tumours, chronic inflammatory diseases and infections. Imaging plays an important role both in establishing the diagnosis of Budd-Chiari syndrome as well as evaluating for underlying causes and complications such as portal hypertension. In this review article, we discuss the role of modern imaging in the evaluation of Budd-Chiari syndrome.

European obesity and the radiology department. What can we do to help

Obesity is a chronic disease that is now a global epidemic. The numbers of obese people are exponentially rising in Europe, and it is projected that in Europe by 2010 there will be 150 million obese people. The obesity-related health crisis does not only affect adults, with one in four European children now overweight. Radiologists, both adult and paediatric, need to be aware of the magnitude of the problem, and obese patients cannot be denied radiologic evaluation due to their size. Missed diagnosis, appointment cancellation and embarrassing situations for patients when they are referred for a radiological examination for which they are not suitable are all issues that can be avoided if careful provision is made to accommodate the needs of the obese patient requiring radiologic evaluation. This paper will discuss the epidemiology of obesity and the role of radiology in the assessment of obesity and disorders of fat metabolism. The limitations obesity poses to current radiological equipment and how the radiologist can optimise imaging in the obese patient will be described. Dose reference levels and dose control are discussed. Examples of how obesity both hinders and helps the radiologist will be illustrated. Techniques and pre-procedural preparation to help the obese patient in the interventional suite are discussed.

Geographic Origin of Publications in Radiological Journals as a Function of GDP and Percentage of GDP Spent on Research

RATIONALE AND OBJECTIVES The aim of this study was to examine the geographic origin of publications in the highest impacting radiology journals and to examine the link between the percentage of gross domestic product (GDP) spent on research by a country and the output of radiology publications. MATERIALS AND METHODS The five highest impacting general radiology journals (according to the ISI Web of Knowledge database) were selected over a 6-year period from January 2002 to December 2007. Publications were totaled according to the country of the corresponding author. Publications (total and corrected for population size) were assessed according to the GDP of a given country and the percentage of GDP spent on research in that country. Correlation was determined using Spearmans rank. RESULTS In total, 10,925 papers were identified. The top 10 nations produced 83.9% of the total number of papers. The United States was the most prolific country, with 41.7% of the total. The second-ranked and third-ranked countries were Germany (11.6%) and Japan (6.7%). Corrected for GDP, smaller European countries outperformed larger nations. Switzerland (0.925 publications per billion of GDP), Austria (0.694 publications per billion of GDP), and Belgium (0.648 publications per billion of GDP) produced the most papers per billion of GDP. When corrected for percentage of GDP spent on research, European countries again ranked highest, with Greece, Turkey, and Belgium having the best ratios. The percentage of GDP spent on research was positively correlated with the number of publications in high-ranking radiology journals (r = 0.603, P < .001). CONCLUSIONS The United States is the most productive country in absolute number of publications. The flaws of using population size to compare publication output are clear, and a comparison using GDP and the percentage of GDP spent on research may give more meaningful results. When GDP is taken into consideration, smaller European countries are more productive. The importance of investment in radiologic research is emphasized by the association between increased funding of research and the number of publications in high-impacting radiology journals.

Quality of pediatric abdominal CT scans performed at a dedicated children’s hospital and its referring institutions: a multifactorial evaluation

BackgroundPediatric patients requiring transfer to a dedicated children’s hospital from an outside institution may undergo CT imaging as part of their evaluation. Whether this imaging is performed prior to or after transfer has been shown to impact the radiation dose imparted to the patient. Other quality variables could also be affected by the pediatric experience and expertise of the scanning institution.ObjectiveTo identify differences in quality between abdominal CT scans and reports performed at a dedicated children’s hospital, and those performed at referring institutions.Materials and methodsFifty consecutive pediatric abdominal CT scans performed at outside institutions were matched (for age, gender and indication) with 50 CT scans performed at a dedicated freestanding children’s hospital. We analyzed the scans for technical parameters, report findings, correlation with final clinical diagnosis, and clinical utility. Technical evaluation included use of intravenous and oral contrast agents, anatomical coverage, number of scan phases and size-specific dose estimate (SSDE) for each scan. Outside institution scans were re-reported when the child was admitted to the children’s hospital; they were also re-interpreted for this study by children’s hospital radiologists who were provided with only the referral information given in the outside institution’s report. Anonymized original outside institutional reports and children’s hospital admission re-reports were analyzed by two emergency medicine physicians for ease of understanding, degree to which the clinical question was answered, and level of confidence in the report.ResultsMean SSDE was lower (8.68) for children’s hospital scans, as compared to outside institution scans (13.29, P = 0.03). Concordance with final clinical diagnosis was significantly lower for original outside institution reports (38/48, 79%) than for both the admission and study children’s hospital reports (48/50, 96%; P = 0.005). Children’s hospital admission reports were rated higher than outside institution reports for completeness, ease of understanding, answering of clinical question, and level of confidence of the report (P < 0.001).ConclusionPediatric abdominal CT scans performed and interpreted at a dedicated children’s hospital are associated with higher technical quality, lower radiation dose and a more clinically useful report than those performed at referring institutions.

Percutaneous Embolization of a Chylous Leak from Thoracic Duct Injury in a Child

Postoperative chylous leak may result from thoracic duct injury during surgical procedures in the chest or neck and can be successfully treated with percutaneous embolization. We report the case of a child with persistent chylothorax and chyloperitoneum following multivisceral transplantation, which was performed due to unresectable inflammatory myofibroblastic tumor of the retroperitoneum. Intranodal lymphangiography was used to demonstrate the site of chylous leak from the lower segment of the thoracic duct and the leak resolved within days following percutaneous embolization of the thoracic duct.

Re: Paget's disease of the bone: a review.

We read with great interest the excellent article in Rheumatology International by Colina et al. entitled ‘Paget’s disease of the bone: a review’ [1]. In this article, the authors give a thorough overview of the pathogenesis, clinical features and treatment of Paget’s disease of the bone (PDB). They correctly stated that PDB is either polyostotic or monostotic and rarely aVects the bones in the feet. Recently at our institution, a patient with prostate carcinoma underwent routine screening with a whole body radionuclide bone scan to exclude metastases. The bone scan showed high uptake at the left calcaneus with the rest of the skeleton normal. Subsequent radiographs showed classical changes of PDB with coarsened trabecular markings and cortical thickening (Fig. 1). A diagnosis of monostotic PDB of the calcaneus was made. While monostotic PDB is unusual, monostotic PDB of the calcaneus is extremely rare. Claustre et al. found 18 cases of calcaneum involvement in Paget’s disease, however, in all these case the calcaneal disease was part of a polyostotic process [2]. The diagnosis may be diYcult due to the rarity of the location and because radiographic Wndings may take time to develop. Whilst the radiograph is the principal imaging modality for the diagnosis of PDB, radionuclide bone scan frequently shows increased uptake in the early stages; prior to the development of radiographic changes. Because of the similar features of high uptake that both Paget’s and metastasis may have on radioscintigraphy, it is open to consider the former even in patients being evaluated for metastatic disease. In our case, we were fortunate that the plain radiographic changes were classical allowing the diagnosis to be made. In conclusion, monostotic Paget’s disease, although rare in the extremities, should always be considered in patients being evaluated on bone scintigraphy to avoid a mis-diagnosis of metastatic disease.

Aortic Calcification in a Patient with a Gain-of-Function STAT1 Mutation

An 8-year-old Caucasian girl, the first child of nonconsanguineous parents, had a history of recurrent respiratory tract infections from infancy and established bronchiectasis by the age of 4 years. She suffered from chronic mucocutaneous candidiasis (CMC) from early childhood. She developed hypothyroidism at the age of 4 years requiring replacement therapy with levothyroxine. She had a history of recurrent oral ulceration, failure to thrive, intermittent diarrhea, iron deficiency anemia with evidence of fecal occult blood, and an elevated fecal calprotectin. However, upper and lower gastrointestinal endoscopy has not demonstrated histological evidence of inflammatory bowel disease. Immunological investigations undertaken at the age of 6 years demonstrated T cell lymphopenia with a helper T cell count of 620 cells/μL (19% naïve), a cytotoxic T cell count of 407 cells/μL (65% naïve), and a relative low proportion (1%) of class-switched (CD27+ IgD-) memory B cells with a relatively increased proportion (8.5%) of CD21low B cells. Serum immunoglobulin levels were in the normal range but vaccinespecific antibody titers to pneumococcus were protective (> 0.35 μg/mL) to only two of 12 serotypes measured. Postbooster vaccination antibody titers demonstrated a good response with protective antibody titers to all 12 serotypes measured. Because of her history of autoimmunity, CMC, and bronchiectasis, sequencing of STAT1 was undertaken and a novel mutation (c848T>C [p.Leu283Ser]) was found in the coiled coil (CC) domain predicted by in silico analysis to be pathogenic. To confirm GOF, peripheral blood mononuclear cells (PBMC) from the patient and two healthy controls were stimulated with IFN-α (10 U/mL) (Miltenyi Biotec) for 30 min, 1 h, and 3 h. Stimulated cells were stained with anti-phospho-STAT1 (BD PhosFlow Alexa Fluor 647 Mouse anti-STAT1 [pY701]) after fixing and permeabilization as recommended by the manufacturer. Flow cytometry was undertaken on the BD LSR Fortessa and analyzed with FCS Express (DeNovo Software). Results confirming gain of function in the patient’s sample are presented in Supplementary Fig. 1. Despite antibiotic prophylaxis and booster vaccinations, our patient continued to suffer from recurrent respiratory tract infections and was commenced on immunoglobulin replacement therapy. A repeat non-contrast CT thorax was performed at the age of 7 years. The CT demonstrated a progression of the left lower lobe bronchiectasis but also showed interval development of extensive circumferential calcification of the ascending aorta and of the aortic arch (Fig. 1). There was no calcification within the common carotid arteries or in the upper abdominal vessels. A contrast-enhanced CT thorax was performed and there was no aneurysm of the thoracic vessels. No aneurysm was identified on a MR angiogram of the neck and intracranial vasculature. There was no abnormal Electronic supplementary material The online version of this article (https://doi.org/10.1007/s10875-018-0513-z) contains supplementary material, which is available to authorized users.

Clinical application of three-dimensional printing to the management of complex univentricular hearts with abnormal systemic or pulmonary venous drainage*

In recent years, three-dimensional printing has demonstrated reliable reproducibility of several organs including hearts with complex congenital cardiac anomalies. This represents the next step in advanced image processing and can be used to plan surgical repair. In this study, we describe three children with complex univentricular hearts and abnormal systemic or pulmonary venous drainage, in whom three-dimensional printed models based on CT data assisted with preoperative planning. For two children, after group discussion and examination of the models, a decision was made not to proceed with surgery. We extend the current clinical experience with three-dimensional printed modelling and discuss the benefits of such models in the setting of managing complex surgical problems in children with univentricular circulation and abnormal systemic or pulmonary venous drainage.

Unexpected coronary arterial calcification and thrombosis late after Kawasaki disease

A 14 year-old boy, with previous Kawasaki disease and coronary artery aneurysms, presented to hospital with severe angina-like chest pain which occurred suddenly while at rest. There was associated pallor, sweating and nausea. Symptoms lasted 1 hour. Initial investigations revealed negative cardiac enzymes and normal ECG. Initial chest radiograph demonstrated an ovoid area of calcification projected over the left side of the heart. CT imaging confirmed the presence of calcification of the left coronary aneurysm (figure 1). Selective angiography demonstrated a giant calcified left main coronary aneurysm with thrombosis and …