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Familial hemophagocytic lymphohistiocytosis (HLH) is a life-threatening hyperinflammatory syndrome resulting from defective cytotoxicity. A previously healthy 3-month-old female presented with fever, irritability, abdominal distention, and tachypnea. She ultimately met all eight HLH-2004 diagnostic criteria, accompanied by elevated CXCL9. Initial empiric anti-inflammatory treatment included anakinra and IVIg, which stabilized ferritin and cytopenias. She had molecular and genetic confirmation of perforin deficiency and was started on dexamethasone and etoposide per HLH-94. She clinically improved, though CXCL9 and sIL-2Ra remained elevated. She was readmitted at week 8 for relapsed HLH without clear trigger and HLH-94 induction therapy was reinitiated. Her systemic HLH symptoms failed to respond and she soon developed symptomatic CNS HLH. She was incidentally found to have multifocal lung and kidney nodules, which were sterile and consisted largely of histiocytes and activated, oligoclonal CD8 T cells. The patient had a laboratory response to salvage therapy with alemtuzumab and emapalumab, but progressive neurologic decline led to withdrawal of care. This report highlights HLH foci manifest as pulmonary/renal nodules, demonstrates the utility of monitoring an array of HLH biomarkers, and suggests possible benefit of earlier salvage therapy.

Background Abnormalities in size and position of the acetabulum have been linked to both developmental dysplasia of the hip and femoroacetabular impingement. Owing to its 3-dimensional (3D) complexity, plain radiography and cross-sectional studies [computed tomography (CT) and magnetic resonance imaging] have limitations in their ability to capture the complexity of the acetabular 3D anatomy. The goal of the study was to use 3D computed tomography reconstructions to identify the acetabular lunate cartilage and measure its size at varying ages of development and between sexes. Methods Patients aged 10 to 18 years with asymptomatic hips and a CT pelvis for appendicitis were reviewed. Patients were stratified by sex and age: preadolescent (10 to 12), young adolescent (13 to 15), and old adolescent (16 to 18) in equal proportions. Materialise 3-matic was used to generate a 3D pelvic model, and the acetabular lunate cartilage surface area was calculated. The lunate cartilage was divided into anatomic segments: superior (11:00 to 1:00), anterior (1:00 to 4:00), and posterior (8:00 to 11:00). The femoral head surface area was calculated to control for patient size. Mixed effects models were generated predicting segment size where side was treated as a repeated measure. Absolute and relative (lunate cartilage to femoral head) models were generated. Results Sixty-two patients (124 hips) were included. Females showed a significant decrease in femoral head coverage as age increased overall and in the 3 subsegments. The majority of changes occurred between the preadolescent and young adolescent groups. Males did not show an overall change, but the superior and anterior anatomic subgroups showed a significant decrease in coverage between the young and old adolescent groups. Male lunate cartilages were absolutely, but not relatively, larger than females. No clinically significant side-to-side differences were noted. Conclusions The relative femoral head coverage by the acetabular lunate cartilage reduced with increasing age, suggesting the growth of the femoral head outpaces the acetabular lunate cartilage's growth. This was more prominent in females. This study has important implications for expected acetabular coverage changes in the latter aspects of pediatric and adolescent development. Level of evidence Level III-diagnostic study.

OBJECTIVE. The contrast-enhanced ultrasound (CEUS) imaging features of hepatic vascular tumors in infants, including infantile hemangioma (IH) and congenital hemangioma (CH), are not well reported....

To compare steady-state magnetic resonance angiography (SS-MRA), using a blood pool contrast agent, with the established technique of time-resolved MRA (TR-MRA), in pulmonary vein mapping and left atrial patency.Twenty-one patients (12 males, age 58.3 ± 8.4 years; 9 females; 57 ± 10 years) undergoing pulmonary vein mapping were evaluated with TR-MRA (TWIST) and SS-MRA. Orthogonal measurements and areas for four veins per patient per technique were assessed by Friedman's test.Overall intertechnique mean difference for any pulmonary vein orthogonal measurement and area was 0.02 ± 0.34 cm (P = 0.705), and 0.2 ± 0.08 cm(2) (P 0.001). Interobserver correlation was strong for diameter and area measurements using the three methods with a range of 0.72-0.94, and 0.87-0.97, respectively. Left atrial appendage image quality score for TR-MRA was significantly lower than the other two methods (P 0.001). Both observers detected more stenosis on inversion recovery (IR)-True FISP compared to TR-MRA and IR-FLASH.SS-MRA with a blood pool agent compared favorably to the established technique of TR-MRA for quantitative assessment of pulmonary venous anatomy. SS-MRA offers greater spatial resolution than TR-MRA with increased confidence for ruling out left atrial appendage filling defect.

We present an analysis of 3D blood flow in two cases of Sinus of Valsalva to right heart fistulae based on 4D flow MRI. Despite similar underlying pathology, 3D visualization revealed intricate differences in flow patterns connecting the systemic and pulmonary circulation. The cases illustrates the potential of 4D flow MRI to complement the evaluation of complex structural heart disease by assessing complex flow dynamics and providing quantitative information of flow ratios and flow rates.

We present the case of a 39-year-old physically active male amateur hockey player with a history of preductal coarctation of the aorta repaired at the age of 10 years with an ascending-to-descending aortic conduit. He presented with neurological symptoms, which were present at rest but paradoxically resolved with physical exertion, including left upper extremity weakness, dizziness, visual changes, and facial tingling. A 2008 echocardiogram demonstrated a bicuspid aortic valve with moderate regurgitation and a mildly to moderately dilated and hypertrophied left ventricle with ejection fraction of 50%. A 2009 carotid Doppler ultrasound examination demonstrated high-velocity retrograde flow in the left vertebral artery, which reduced on exertion. Stenosis of the subclavian artery, and thus subclavian steal, which has been shown to cause neurological symptoms, was ruled out by a 2010 arteriogram.1 A recent magnetic resonance imaging (MRI) evaluation (in 2011) demonstrated worsening (moderate to severe) aortic insufficiency with a clearly visible asymmetrical diastolic flow jet (Figure 1, white arrows), mild decrease of left ventricular ejection fraction, and a patent aortic bypass conduit. Figure 1. Cardiac magnetic resonance angiography demonstrating aortic valve insufficiency and a clearly visible asymmetrical flow jet (white arrows, regions with signal void in the left ventricle). The individual images represent …