Development and Validation of a Pediatric MRI-Based Perianal Crohn Disease (PEMPAC) Index-A Report from the ImageKids Study.

BACKGROUND: As part of the prospective multicenter ImageKids study, we aimed to develop and validate the pediatric MRI-based perianal Crohn disease (PEMPAC) index. METHODS: Children with Crohn disease with any clinical perianal findings underwent pelvic magnetic resonance imaging at 21 sites globally. The site radiologist and 2 central radiologists provided a radiologist global assessment (RGA) on a 100 mm visual analog scale and scored the items selected by a Delphi group of 35 international radiologists and a review of the literature. Two weighted multivariable statistical models were constructed against the RGA. RESULTS: Eighty children underwent 95 pelvic magnetic resonance imaging scans; 64 were used for derivation and 31 for validation. The following items were included: fistula number, location, length and T2 hyperintensity; abscesses; rectal wall involvement; and fistula branching. The last 2 items had negative beta scores and thus were excluded in a contending basic model. In the validation cohort, the full and the basic models had the same strong correlation with the RGA (r = 0.75; P < 0.01) and with the adult Van Assche index (VAI; r = 0.93 and 0.92; P < 0.001). The correlation of the VAI with the RGA was similar (r = 0.77; P < 0.01). The 2 models and the VAI had a similar ability to differentiate remission from active disease (area under the receiver operating characteristic curve, 0.91-0.94). The PEMPAC index had good responsiveness to change (area under the receiver operating characteristic curve, 0.89; 95% confidence interval, 0.69-1.00). CONCLUSIONS: Using a blended judgmental and mathematical approach, we developed and validated an index for quantifying the severity of perianal disease in children with CD. The adult VAI may also be used with confidence in children.

Measurement of total and visceral fat mass in young adult women: a comparison of MRI with anthropometric measurements with and without bioelectrical impedance analysis.

OBJECTIVE: MRI is established for measurement of body fat mass (FM) and abdominal visceral adipose tissue (VAT). Anthropometric measurements and bioelectrical impedance analysis (BIA) have been proposed as surrogates to estimation by MRI. Aim of this work is to assess the predictive value of these methods for FM and VAT measured by MRI. METHODS: Patients were selected from cohort study PPS-Diab (prediction, prevention and subclassification of Type 2 diabetes). Total FM and VAT were quantified by MRI and BIA together with clinical variables like age, waist and hip circumference and height. Least-angle regressions were utilized to select anthropometric and BIA parameters for their use in multivariable linear regression models to predict total FM and VAT. Bland-Altman plots, Pearson correlation coefficients, Wilcoxon signed-rank tests and univariate linear regression models were applied. RESULTS: 116 females with 35 ± 3 years and a body mass index of 25.1 ± 5.3 kg/m2 were included into the analysis. A multivariable model revealed weight (ß = 0.516, p < 0.001), height (ß = -0.223, p < 0.001) and hip circumference (ß = 0.156, p = 0.003) as significantly associated with total FM measured by MRI. A additional multivariable model also showed a significant predictive value of FMBIA (ß = 0.583, p < 0.001) for FM. In addition, waist circumference (ß = 0.054, p < 0.001), weight (ß = 0.016, p = 0.031) in one model and FMBIA (ß = 0.026, p = 0.018) in another model were significantly associated with VAT quantified by MRI. However, deviations reached more than 5 kg for total FM and more than 1 kg for VAT. CONCLUSION: Anthropometric measurements and BIA show significant association with total FM and VAT. ADVANCES IN KNOWLEDGE: As these measurements show significant deviations from the absolute measured values determined by MRI, MRI should be considered the gold-standard for quantification.

3D black-blood 3T-MRI for the diagnosis of abdominal large vessel vasculitis.

OBJECTIVES: To assess the value of a T1-3D black-blood turbo spin echo (TSE) sequence for the diagnosis of abdominal large vessel vasculitis (LVV). MATERIALS AND METHODS: The study included 20 patients with abdominal LVV and 17 controls, who underwent a 3T-MRI scan using a modified T1-3D volumetric isotropic TSE acquisition and a segmented T1-3D turbo field echo sequence (T1-mVISTA/T1-eTHRIVE). Two radiologists independently analyzed the aorta for concentric contrast enhancement, concentric wall thickening, image quality, and flow artifact intensity (CCE/CWT/IQ/FAI; 4-point scales). The mean aortic wall thickness (MAWT) in post-contrast T1-mVISTA was compared between patients and controls. RESULTS: IQ of T1-mVISTA was rated good to excellent in 91.5% of 282 evaluated vessel segments with no or minor FAI present in 85.5%. The inter-observer reproducibility for the identification of CCE/CWT on T1-mVISTA was 0.92 and 0.93 (p < 0.001). The distribution of segmental inflammation in T1-mVISTA significantly correlated with T1-eTHRIVE (CCE, κ = 0.768; CWT, κ = 0.715; p < 0.001), resulting in a sensitivity, specificity, and positive predictive value of 100%, 81.3%, and 83.3%. The MAWT significantly differed between patients and controls (3.29 ± 0.81 vs. 2.24 ± 0.45 mm; p < 0.001). CONCLUSIONS: T1-mVISTA enables the evaluation of the MAWT and allows the detection of abdominal LVV. KEY POINTS: • 3D T1w-mVISTA accurately depicted the large abdominal vessels. • 3D T1w-mVISTA enables accurate measurements of the abdominal aortic wall thickness. • 3D T1w-mVISTA is useful for the detection of abdominal LVV.

Contrast-enhanced modified 3D T1-weighted TSE black-blood imaging can improve detection of infectious and neoplastic meningitis.

OBJECTIVES: To evaluate the diagnostic value of a contrast-enhanced 3D T1-weighted-modified volumetric isotropic turbo spin-echo acquisition sequence (T1-mVISTA) in comparison with a conventional 3D T1-weighted magnetization-prepared rapid gradient-echo (T1-MP-RAGE) sequence for the detection of meningeal enhancement in patients with meningitis. METHODS: Thirty patients (infectious meningitis, n = 12; neoplastic meningitis, n = 18) and 45 matched controls were enrolled in this retrospective case-control study. Sets of randomly selected T1-mVISTA and T1-MP-RAGE images (both with 0.8-mm isotropic resolution) were read separately 4 weeks apart. Image quality, leptomeningeal and dural enhancement, grading of visual contrast enhancement, and diagnostic confidence were compared using the Kruskal-Wallis rank sum test. RESULTS: Image quality was rated to be good to excellent in 75 out of 75 cases (100%) for T1-mVISTA and 74 out of 75 cases (98.7%) for T1-MP-RAGE. T1-mVISTA detected significantly more patients with leptomeningeal enhancement (p = 0.006) compared with T1-MP-RAGE (86.7 vs. 50.0%, p < 0.001), each with specificity of 100%. Similarly, sensitivity of T1-mVISTA for the detection of dural and/or leptomeningeal enhancement was also significantly higher compared with that of T1-MP-RAGE (96.7 vs. 80.0%, p = 0.025) without significant differences regarding specificity (97.8 vs. 95.6%, p = 0.317). No significant differences were found for dural enhancement alone. Diagnostic confidence in T1-mVISTA was significantly higher (p = 0.01). Visual contrast enhancement was tendentially higher in T1-mVISTA. CONCLUSIONS: T1-mVISTA may be an adequate and probably better alternative to T1-MP-RAGE for detection of leptomeningeal diseases. KEY POINTS: • Black-blood T1-mVISTA showed a significant higher sensitivity for the detection of leptomeningeal enhancement compared with MP-RAGE without losses regarding specificity. • Diagnostic confidence was assessed significantly higher in T1-mVISTA. • T1-mVISTA should be considered a supplement or an alternative to T1-MP-RAGE in patients with suspected leptomeningeal diseases.

Simple Endoscopic Score of Crohn Disease and Magnetic Resonance Enterography in Children: Report From ImageKids Study.

OBJECTIVES: We aimed to explore the ability of magnetic resonance enterography (MRE) to impute the simple endoscopic score of Crohn disease (SES-CD) in children with CD, in whom failure of ileal intubation is common and may impair SES-CD calculation in clinical studies. METHODS: This is a substudy of the prospective ImageKids study in which children with CD underwent ileocolonoscopy (scored by SES-CD) and MRE (scored on a 100 mm visual analogue scale [VAS] and by MaRIA). Mucosal healing (MH) was defined as SES-CD <3, MRE-VAS <20 mm, and/or MaRIA <7. RESULTS: A total of 237 children (22 centers, age 11.5 ±â€Š3.3 years), were enrolled. Ileal intubation has failed in 40 of 237 (17%). The agreement between SES-CD and MRE was 75% (k = 0.508, P < 0.001) in the ileum, and 68% to 85% in the colonic segments (k = 0.21-0.50, P < 0.001). The sensitivity and specificity of ileal MRE-VAS for MH were 91.7% (95% confidence interval 0.84-0.96) and 53.1% (95% confidence interval 0.43-0.63), respectively. The ileal MaRIA score (calculated in 33/40) was higher in the children without ileal intubation than in the others (20.5 ±â€Š7.1 vs 15.1 ±â€Š10.8, respectively, P = 0.0018). In 7% (16/237) of children, isolated active ileal disease would have been missed when considering SES-CD only. A multivariable model predicted the ileal SES-CD subscore from the MaRIA: SES-CDileum = 1.145 + 0.169 × MaRIAileum rounded to the nearest whole number (R = 0.17). Applying this model to the children without ileal intubation revealed that 29 of 33 (88%) had ileal disease; 8 of 29 patients (28%) with normal colonic SES-CD had imputed ileal SES-CD ≥3. CONCLUSIONS: MRE is useful for imputing the ileal disease in pediatric clinical studies, overcoming the problem of ileal nonintubation.

Three-Dimensional High-Resolution Black-Blood Magnetic Resonance Imaging for Detection of Arteritic Anterior Ischemic Optic Neuropathy in Patients With Giant Cell Arteritis.

OBJECTIVES: Arteritic anterior ischemic optic neuropathy (A-AION) caused by inflammatory occlusion of the posterior ciliary arteries is the most common reason for irreversible vision loss in patients with giant cell arteritis. Atypical clinical presentation and negative funduscopy can delay systemic high-dose corticosteroid therapy to prevent impending permanent blindness and involvement of the contralateral eye.The purpose of this study was to assess the diagnostic accuracy of 3-dimensional (3D) high-resolution T1-weighted black-blood magnetic resonance imaging (T1-BB-MRI) for the detection of posterior ciliary artery involvement in patients with giant cell arteritis and funduscopic A-AION. MATERIALS AND METHODS: After institutional review board approval and informed consent, 27 patients with suspected giant cell arteritis and vision disturbances were included in this monocentric prospective cohort study. Giant cell arteritis was diagnosed in 18 patients according to the diagnostic reference standard (6 men, 73.8 [69.0-78.0] years); 14 of those were positive for A-AION. Precontrast and postcontrast 3D T1-BB-MRI was performed in all 27 patients. Two radiologists separately assessed image quality and local fat suppression (4-point scale), visual contrast enhancement (3-point scale), and diagnostic confidence (5-point scale) regarding arteritic posterior ciliary artery involvement. Magnetic resonance imaging findings were assessed in comparison to funduscopy. Statistical analysis included accuracy parameters and interrater agreement. RESULTS: Sensitivity of 3D T1-BB-MRI was 92.9% (95% confidence interval, 66.1%-99.8%) and specificity was 92.3% (95% confidence interval, 64.0%-99.8%) for detection of A-AION-positive patients. Image quality and local fat suppression were assessed with 3.2 ± 0.8 (median 3) and 3.8 ± 0.5 (median 4). Visual contrast enhancement with 2.3 ± 0.8 (median 3) and diagnostic confidence was rated at 4.7 ± 0.5 (median 5). Interrater agreement was high (κ = 0.85, P < 0.001). Three-dimensional T1-BB-MRI displayed bilateral findings in 50% of the cases, whereas only unilateral A-AION was detected in funduscopy as a possible indication for the contralateral eye at risk. CONCLUSIONS: Three-dimensional T1-BB-MRI allows accurate detection of arteritic posterior ciliary artery involvement in patients with A-AION. Further, 3D T1-BB-MRI seems to display arteritic involvement of the posterior ciliary arteries earlier than funduscopy and might, therefore, display "vision-at-risk" in patients with visual impairment and suspected giant cell arteritis but unremarkable funduscopy.

No Correlation of Pancreatic Fat and ß-Cell Function in Young Women With and Without a History of Gestational Diabetes.

Context: Pancreatic steatosis may contribute to ß-cell dysfunction in type 2 diabetes (T2D), but data are controversial. Women who had gestational diabetes mellitus (GDM) are at high risk for developing T2D. Objective: To examine the association of pancreatic fat content with early/first-phase insulin secretion (as markers of ß-cell function). Design: Cross-sectional analysis of a subcohort of the monocentric, prospective cohort study titled Prediction, Prevention, and Subclassification of Type 2 Diabetes. Setting: Ludwig Maximilians University Hospital, Munich, Germany. Participants: Ninety-seven women, 3 to 16 months after pregnancy [41 normoglycemic women post-GDM, 19 women post-GDM with pathological glucose metabolism, and 37 normoglycemic women after a normoglycemic pregnancy (controls)]. Main Outcome Measures: Correlation of MRI-measured pancreatic fat content with early insulin release in an oral glucose tolerance test (OGGT) [insulin increment within the first 30 minutes of the OGTT (IR30)] and first-phase insulin response (FPIR) in an intravenous glucose tolerance test (n = 65), both adjusted for insulin sensitivity index (ISI). Results: Pancreatic fat content did not correlate with IR30 and FPIR adjusted for ISI. It correlated positively with body mass index, waist circumference, liver fat, and intraabdominal fat volume. Conclusion: Pancreatic fat content does not correlate with ß-cell function in a cohort of young women with different degrees of T2D risk.

A Simple Endoscopic Score Modified for the Upper Gastrointestinal Tract in Crohn's Disease [UGI-SES-CD]: A Report From the ImageKids Study.

OBJECTIVE: There is no standardized endoscopic description of upper gastrointestinal [UGI] disease in Crohn's disease [CD]. We prospectively applied the Simple Endoscopic Score for CD [SES-CD] to the UGI tract as a planned sub-study of the multicentre prospective ImageKids study. We aimed to assess the utility of the UGI-SES-CD and its clinical significance in paediatric CD. DESIGN: Patients underwent an oesophagogastroduodenoscopy [EGD], ileocolonoscopy, and magnetic resonance enterography [MRE] with explicit clinical data recorded. SES-CD was scored at each region [oesophagus, stomach body, antrum, and duodenum]. Half of the patients were followed for 18 months, when a repeat MRE was performed. RESULTS: A total of 202 children were included 56% males, mean age 11.5 ± 3.2 years, median weighted Paediatric Crohn's Disease Activity Index [wPCDAI 25]). UGI-SES-CD score ranged 0-17, with 95 [47%] having a UGI-SES-CD ≥1; no narrowing was detected. UGI-SES-CD ≥1 was associated with higher: wPCDAI [32.5 vs 20; p = 0.03]; Physician's Global Assessment [PGA] of inflammation (45 mm visual analogue score [VAS] vs 30 mm VAS; p = 0.04); ileocolonoscopic SES-CD [10 vs 7; p = 0.004], faecal calprotectin [717 µg/g vs 654 µ/g; p= 0.046]; and radiological global assessment of damage by MRE [7 mm VAS vs 0; p = 0.04]. In all, 81 patients were followed for 18 months and no association was identified between initial UGI SES-CD and markers of disease course such as surgery, MRE assessment, or treatment escalation. CONCLUSION: UGI-SES-CD is an easily reported objective scoring system and is associated with a more severe disease phenotype but not with disease course.

Muscle MRI in patients with dysferlinopathy: pattern recognition and implications for clinical trials.

BACKGROUND AND OBJECTIVE: Dysferlinopathies are a group of muscle disorders caused by mutations in the DYSF gene. Previous muscle imaging studies describe a selective pattern of muscle involvement in smaller patient cohorts, but a large imaging study across the entire spectrum of the dysferlinopathies had not been performed and previous imaging findings were not correlated with functional tests. METHODS: We present cross-sectional T1-weighted muscle MRI data from 182 patients with genetically confirmed dysferlinopathies. We have analysed the pattern of muscles involved in the disease using hierarchical analysis and presented it as heatmaps. Results of the MRI scans have been correlated with relevant functional tests for each region of the body analysed. RESULTS: In 181 of the 182 patients scanned, we observed muscle pathology on T1-weighted images, with the gastrocnemius medialis and the soleus being the most commonly affected muscles. A similar pattern of involvement was identified in most patients regardless of their clinical presentation. Increased muscle pathology on MRI correlated positively with disease duration and functional impairment. CONCLUSIONS: The information generated by this study is of high diagnostic value and important for clinical trial development. We have been able to describe a pattern that can be considered as characteristic of dysferlinopathy. We have defined the natural history of the disease from a radiological point of view. These results enabled the identification of the most relevant regions of interest for quantitative MRI in longitudinal studies, such as clinical trials. CLINICAL TRIAL REGISTRATION: NCT01676077.

Childhood-Onset Epileptic Encephalopathy Associated With Isolated Focal Cortical Dysplasia and a Novel TSC1 Germline Mutation.

Tuberous sclerosis complex (TSC) is an autosomal-dominant inheritable neurocutaneous disease due to mutations within the TSC1 and TSC2 genes. Many patients present with West syndrome, a severe epilepsy syndrome characterized by the triad of infantile spasms, an interictal electroencephalogram (EEG) pattern termed hypsarrhythmia (continuous slow activity with an amplitude higher than 300 µV and multiregional spikes/polyspikes/sharp waves) and developmental regression. In this study, we report on a previously healthy patient with positive family history of epilepsy with new-onset epileptic encephalopathy at the age of 9 years. Clinical signs alone were not sufficient to establish the diagnosis of TSC but epilepsy panel screening revealed a novel frameshift mutation (c.90delA; p.Glu31Argfs*12) within the TSC1 gene. Segregation gene analysis detected the same mutation in the mother. Cranial magnetic resonance imaging (MRI) studies from the index patient and his mother revealed a similar pattern of isolated subcortical white matter lesions resembling most likely focal cortical dysplasia (FCD) type IIb. In summary, in these 2 related patients, a novel TSC1 frameshift mutation was associated with an isolated FCD type IIb in the absence of further CNS abnormalities usually encountered in patients with TSC, fostering our understanding of the broad mutation spectra in the TSC1 gene and the close relationship between cortical tubers and FCD type IIb.

Multiple Sclerosis: Improved Detection of Active Cerebral Lesions With 3-Dimensional T1 Black-Blood Magnetic Resonance Imaging Compared With Conventional 3-Dimensional T1 GRE Imaging.

OBJECTIVES: The aim of this study was to assess the diagnostic accuracy of a modified high-resolution whole-brain three-dimensional T1-weighted black-blood sequence (T1-weighted modified volumetric isotropic turbo spin echo acquisition [T1-mVISTA]) in comparison to a standard three-dimensional T1-weighted magnetization-prepared rapid gradient echo (MP-RAGE) sequence for detection of contrast-enhancing cerebral lesions in patients with relapsing-remitting multiple sclerosis (MS). MATERIALS AND METHODS: After institutional review board approval and informed consent, 22 patients (8 men; aged 31.0 ± 9.2 years) with relapsing-remitting MS were included in this monocentric prospective cohort study.Contrast-enhanced T1-mVISTA and MP-RAGE, both with 0.8 mm resolution, were performed in all patients. In a substudy of 12 patients, T1-mVISTA was compared with a T1-mVISTA with 1.0 mm resolution (T1-mVISTA_1.0). Reference lesions were defined by an experienced neuroradiologist using all available sequences and served as the criterion standard. T1-mVISTA, T1-mVISTA_1.0, and MP-RAGE sequences were read in random order 4 weeks apart. Image quality, visual contrast enhancement, contrast-to-noise-ratio (CNR), diagnostic confidence, and lesion size were assessed and compared by Wilcoxon and Mann-Whitney U tests. RESULTS: Eleven of 22 patients displayed contrast-enhancing lesions. Visual contrast enhancement, CNR, and diagnostic confidence of contrast-enhancing MS lesions were significantly increased in T1-mVISTA compared with MP-RAGE (P < 0.001). Significantly more contrast-enhancing lesions were detected with T1-mVISTA than with MP-RAGE (71 vs 39, respectively; P < 0.001). With MP-RAGE, 25.6% of lesions were missed in the initial reading, whereas only 4.2% of lesions were missed with T1-mVISTA. Increase of the voxel volume from 0.8 mm to 1.0 mm isotropic in T1-mVISTA_1.0 did not affect the detectability of lesions, whereas scan time was decreased from 4:43 to 1:55 minutes. CONCLUSIONS: Three-dimensional T1-mVISTA improves the detection rates of contrast-enhancing cerebral MS lesions compared with conventional 3D MP-RAGE sequences by increasing CNR of lesions and might, therefore, be useful in patient management.

Clinical Significance of Intraluminal Contrast Enhancement in Patients with Spontaneous Cervical Artery Dissection: A Black-Blood MRI Study.

Purpose Recent studies have suggested that intraluminal thrombi show contrast enhancement on carotid black-blood T1w MRI. The aim of this study was to evaluate the significance of intraluminal contrast enhancement (iCE) regarding symptom status in patients with spontaneous cervical artery dissection (sCAD). Methods 33 consecutive patients (19 men) with sCAD received a brain MRI (DIFF, T2w, T2*w, FLAIR) and a multi-sequence 3T-MRI with fat-saturated high-resolution black-blood T1w-sequences pre- and post-contrast, contrast-enhanced MR angiography and TOF images of carotid and vertebral arteries. Presence/absence of iCE, vessel occlusion and vessel wall hematoma (hyperintense in T1w pre-contrast) were analysed by two radiologists in consensus decision. Results 44 of 132 analysed vessels had a vessel wall hematoma, consistent with sCAD. In 17 of 44 dissected vessels an acute ischemic stroke was found. 16 of 17 (94.1 %) vessels ipsilateral to ischemic stroke demonstrated iCE, compared to 9 of 44 (20.4 %) dissected vessels without stroke (P< 0.001). The presence/absence of iCE resulted in a sensitivity, specificity, positive and negative predictive value and accuracy for ischemic stroke of 0.94, 0.67, 0.64, 0.95, and 0.77, respectively, and an odds ratio of 32.0. Conclusion iCE, which is suggestive of intraluminal thrombus formation, is strongly correlated with ischemic symptoms in patients with sCAD. Key points · Intraluminal contrast enhancement is associated with cerebral ischemia in corresponding vessel territories in patients with cervical artery dissection.. · Our results suggest that intraluminal contrast enhancement represents intraluminal thrombus formation.. · Black-blood imaging might be useful to identify those patients with cervical artery dissection at higher risk for initial or recurrent stroke.. Citation Format · Coppenrath E, Lenz O, Sommer N et al. Clinical Significance of Intraluminal Contrast Enhancement in Patients with Spontaneous Cervical Artery Dissection: A Black-Blood MRI Study. Fortschr Röntgenstr 2017; 189: 624 - 631.

Perianal Pediatric Crohn Disease Is Associated With a Distinct Phenotype and Greater Inflammatory Burden.

OBJECTIVES: Data on the outcomes of children with perianal Crohn disease (pCD) are limited, although its presence is often used for justifying early use of biologics. We aimed to assess whether pCD in children is associated with more severe outcomes as found in adults. METHODS: Data were extracted from the ImageKids database, a prospective, multicenter, longitudinal cohort study. The study enrolled 246 children at disease onset or thereafter. All patients underwent comprehensive clinical, endoscopic, and radiologic evaluation at enrollment; 98 children had repeat evaluation at 18 months. RESULTS: Of the 234 included patients (mean age 14.2 ±â€Š2.4 years; 131 [56%] boys), 57 (24%) had perianal findings, whereas only 21 (9%) had fistulizing perianal disease. Children with pCD had reduced weight and height z scores compared with non-pCD patients (-0.9 vs -0.35, P = 0.03 and -0.68 vs -0.23, respectively; P = 0.04), higher weighted pediatric CD activity index (32 [interquartile range 16-50] vs 20 [8-37]; P = 0.004), lower serum albumin (3.6 ±â€Š0.7 vs 4.5 ±â€Š0.8, P = 0.016), and higher magnetic resonance enterography global inflammatory score (P = 0.04). Children with pCD had more rectal (57% vs 38%, P = 0.04), and jejunal involvement (31% vs 11% P = 0.003) and a higher prevalence of granulomas (64% vs 23%, P = 0.0001). Magnetic resonance enterography-based damage scores did not differ between groups. Patients with skin tags/fissures only, had similar clinical, endoscopic, and radiologic characteristics as patients with no perianal findings. CONCLUSIONS: Pediatric patients with pCD with fistulizing disease have distinct phenotypic features and a predisposition to a greater inflammatory burden.

Increase of Intra-abdominal Adipose Tissue in Pediatric Crohn Disease.

BACKGROUND AND OBJECTIVE: Recent evidence points toward an active immunological role of intra-abdominal adipose tissue in Crohn disease (CD). We quantified the abdominal adipose tissue compartments using magnetic resonance imaging (MRI) in 27 pediatric patients with CD compared with 14 controls undergoing MRI examination for other reasons. METHODS: Total (TAAT), subcutaneous (SCAT) and intra-abdominal (IAAT) adipose tissue areas were measured by semiautomatic segmentation on a transverse slice centered on the umbilicus (mean ±â€Šstandard deviation in square centimeter) using standard T1-weighted sequences. IAAT/TAAT and IAAT/height ratios were calculated and analyzed for associations with disease duration, phenotype, or therapy. RESULTS: Patients with CD (median age 15.0 years, range 7.7-17.9, 18/27 boys, median disease duration 29 months, range 0-136) compared to controls (median age 13.9 years, range 3.3-17.8, 4/14 boys) had higher IAAT area (42.3 ±â€Š21.0 vs 28.7 ±â€Š11.6, P = 0.0494) but similar SCAT and TAAT areas (104.6 ±â€Š72.8 vs 96.5 ±â€Š50.8, P = 0.8170 and 146.9 ±â€Š87.3 vs 125.3 ±â€Š61.5, P = 0.7417, respectively). IAAT/TAAT ratio was higher in patients with CD compared to controls (0.32 ±â€Š0.10 vs 0.24 ±â€Š0.04, P = 0.0081). Patients with disease duration >2 years (n = 14) had higher IAAT/TAAT ratio than those with shorter disease and controls (0.35 ±â€Š0.10 vs 0.28 ±â€Š0.08, P = 0.0288 and 0.24 ±â€Š0.04, P = 0.0009, respectively). In these patients, increased IAAT/height ratio was associated with complicated disease (P = 0.043, r = 0.573). No association was found between IAAT/TAAT ratio and actual disease activity or therapy. CONCLUSIONS: IAAT is increased in pediatric CD and correlates with disease duration. Assessment of IAAT accumulation may be considered in future MRI scores for inflammation and bowel damage in CD and during follow-up of different therapeutic interventions.

3D-black-blood 3T-MRI for the diagnosis of thoracic large vessel vasculitis: A feasibility study.

OBJECTIVES: To evaluate the feasibility of T1w-3D black-blood turbo spin echo (TSE) sequence with variable flip angles for the diagnosis of thoracic large vessel vasculitis (LVV). METHODS: Thirty-five patients with LVV, diagnosed according to the current standard of reference, and 35 controls were imaged at 3.0T using 1.2 × 1.3 × 2.0 mm3 fat-suppressed, T1w-3D, modified Volumetric Isotropic TSE Acquisition (mVISTA) pre- and post-contrast. Applying a navigator and peripheral pulse unit triggering (PPU), the total scan time was 10-12 min. Thoracic aorta and subclavian and pulmonary arteries were evaluated for image quality (IQ), flow artefact intensity, diagnostic confidence, concentric wall thickening and contrast enhancement (CWT, CCE) using a 4-point scale. RESULTS: IQ was good in all examinations (3.25 ± 0.72) and good to excellent in 342 of 408 evaluated segments (83.8 %), while 84.1 % showed no or minor flow artefacts. The interobserver reproducibility for the identification of CCE and CWT was 0.969 and 0.971 (p < 0.001) with an average diagnostic confidence of 3.47 ± 0.64. CCE and CWT were strongly correlated (Cohen's k = 0.87; P < 0.001) and significantly more frequent in the LVV-group (52.8 % vs. 1.0 %; 59.8 % vs. 2.4 %; P < 0.001). CONCLUSIONS: Navigated fat-suppressed T1w-3D black-blood MRI with PPU-triggering allows diagnosis of thoracic LVV. KEY POINTS: • Cross-sectional imaging is frequently applied in the diagnosis of LVV. • Navigated, PPU-triggered, T1w-3D mVISTA pre- and post contrast takes 10-12 min. • In this prospective, single-centre study, T1w-3D mVISTA accurately depicted large thoracic vessels. • T1w-3D mVISTA visualized CWT/CCW as correlates of mural inflammation in LVV. • T1w-3D mVISTA might be an alternative diagnostic tool without ionizing radiation.

Combined Scintigraphy and Tumor Marker Analysis Predicts Unfavorable Histopathology of Neuroblastic Tumors with High Accuracy.

OBJECTIVES: Our aim was to improve the prediction of unfavorable histopathology (UH) in neuroblastic tumors through combined imaging and biochemical parameters. METHODS: 123I-MIBG SPECT and MRI was performed before surgical resection or biopsy in 47 consecutive pediatric patients with neuroblastic tumor. Semi-quantitative tumor-to-liver count-rate ratio (TLCRR), MRI tumor size and margins, urine catecholamine and NSE blood levels of neuron specific enolase (NSE) were recorded. Accuracy of single and combined variables for prediction of UH was tested by ROC analysis with Bonferroni correction. RESULTS: 34 of 47 patients had UH based on the International Neuroblastoma Pathology Classification (INPC). TLCRR and serum NSE both predicted UH with moderate accuracy. Optimal cut-off for TLCRR was 2.0, resulting in 68% sensitivity and 100% specificity (AUC-ROC 0.86, p < 0.001). Optimal cut-off for NSE was 25.8 ng/ml, resulting in 74% sensitivity and 85% specificity (AUC-ROC 0.81, p = 0.001). Combination of TLCRR/NSE criteria reduced false negative findings from 11/9 to only five, with improved sensitivity and specificity of 85% (AUC-ROC 0.85, p < 0.001). CONCLUSION: Strong 123I-MIBG uptake and high serum level of NSE were each predictive of UH. Combined analysis of both parameters improved the prediction of UH in patients with neuroblastic tumor. MRI parameters and urine catecholamine levels did not predict UH.

Three-dimensional black-blood T1-weighted turbo spin-echo techniques for the diagnosis of deep vein thrombosis in comparison with contrast-enhanced magnetic resonance imaging: a pilot study.

OBJECTIVES: The objective of this study was to evaluate the feasibility of a novel 3-dimensional turbo spin-echo technique with isotropic resolution for the diagnosis of deep vein thrombosis (DVT) in comparison with contrast-enhanced magnetic resonance imaging (CE-MRI) and sonography. MATERIALS AND METHODS: Thirteen patients (8 males, 17-93 years) with proven DVT in duplex ultrasound (n = 11) or with pulmonary embolism and suspected to have DVT (n = 2) were consecutively imaged at 3.0 T with 1.2-mm isotropic-resolution volumetric isotropic turbo spin-echo acquisition (VISTA). Sensitivity (SE), specificity (SP), positive and negative predictive values (PPV and NPV, respectively), Cohen κ, as well as accuracy of VISTA-MRI were calculated and compared with CE-MRI and sonography as a standard of reference. Image quality and diagnostic confidence were assessed on a 4-point scale. RESULTS: Image quality and diagnostic confidence level of VISTA-MRI and CE-MRI were comparable (3.54 vs 3.55 and 3.80 vs 3.77; both P values are nonsignificant). Using CE-MRI as the criterion standard, there was a high agreement between the CE-MRI and the 3-dimensional VISTA examinations for the detection of DVT, with κ of 0.89 for reader 1 and κ of 0.88 for reader 2 (both P < 0.001). The SE, SP, PPV, NPV, as well as accuracy of VISTA-MRI were 92.5%, 97.9%, 89.3%, 98.6%, and 97.1% for reader 1 as well as 90.7%, 97.9%, 89.1%, 98.3%, and 96.8% for reader 2. For both readers, combined comparison of VISTA-MRI and sonography resulted in an SE, SP, PPV, and NPV of 77.8%, 94.8%, 85.4%, and 91.6%, respectively. CONCLUSIONS: Volumetric isotropic turbo spin-echo acquisition magnetic resonance imaging can be used to diagnose DVT with good to excellent agreement compared with CE-MRI and sonography. It might be useful when contrast media is prohibited and in patients with suspected thrombosis of the iliac veins, which can be hard to detect with sonography.

High ¹²³I-MIBG uptake in neuroblastic tumours indicates unfavourable histopathology.

PURPOSE: Scintigraphy using (123)I-metaiodobenzylguanidine ((123)I-MIBG) is widely used for the detection of neuroblastic tumours. The aim of this study was to identify a possible correlation between the uptake intensity on (123)I-MIBG SPECT and histopathology of neuroblastic tumours. METHODS: (123)I-MIBG SPECT examinations were performed in 55 paediatric patients with neuroblastic tumour and compared to histopathology after surgical resection or biopsy at a mean of 2 weeks after SPECT. For each lesion International Neuroblastoma Pathology Classification System (INPC) stage, mitosis karyorrhexis index (MKI), location and a semiquantitative tumour-to-liver count-rate ratio (TLCRR) were determined. Also, the presence or absence of MYCN amplification, p1 deletion, urine catecholamine and neuron-specific enolase blood levels at the time of scanning were recorded. RESULTS: In the 55 patients, 61 lesions were evaluated with (123)I-MIBG SPECT and corresponding histopathological findings were reviewed (11 ganglioneuroma, 11 ganglioneuroblastoma and 39 neuroblastoma). TLCRR was significantly higher in the neuroblastoma group (mean TLCRR 2.7) than in the ganglioneuroblastoma group (mean TLCRR 1.0) and ganglioneuroma group (mean TLCRR 0.7) at the time of primary diagnosis (p < 0.001) and at follow-up (p = 0.039). Intense (123)I-MIBG uptake was found in tumour tissue with a high mitotic activity (MKI-high or MKI-intermediate) after treatment. Four ganglioneuromas (36 %), three ganglioneuroblastomas (27 %) and six neuroblastomas (15 %) were (123)I-MIBG-negative. CONCLUSION: In paediatric patients with peripheral neuroblastic tumours, strong (123)I-MIBG uptake indicates unfavourable histopathology. High uptake was seen in neuroblastomas and in tumours with a high mitotic activity.