Diagnostic Performance of MR Elastography for Liver Fibrosis in Children and Young Adults with a Spectrum of Liver Diseases.

Purpose To assess the diagnostic performance of magnetic resonance (MR) elastography-derived liver stiffness to detect liver fibrosis in a pediatric and young adult population with a spectrum of liver diseases. Materials and Methods This retrospective study included patients younger than 21 years of age who underwent MR elastography and liver biopsy within 3 months of one another between January 2012 and September 2016 for indications other than liver transplantation or Fontan palliation of congenital heart disease. MR elastography examinations were reprocessed by a single observer, blinded to pathologic findings. Pathology specimens were reviewed by a single pathologist who scored steatosis (lipid in ≥ 5% of hepatocytes) and staged fibrosis. Receiver operating characteristic (ROC) curves were used to assess diagnostic performance. Results A total of 86 patients, 49 (57%) male with a median age of 14.2 years (range, 0.3-20.6 years), were included. Fifty-one patients (59.3%) had Ludwig stage 2 or higher fibrosis; 44 patients (51.2%) had hepatic steatosis. The area under the ROC curve for Ludwig stage 0-1 versus stage 2 or higher fibrosis was 0.70 (95% confidence interval [CI]: 0.59, 0.81) for the whole population and was significantly lower for patients with steatosis versus those without (0.53 [95% CI: 0.35, 0.71] vs 0.82 [95% CI: 0.67, 0.96], P = .014). Optimal stiffness cut-offs for the entire population were 2.27 kPa with 68.6% sensitivity (95% CI: 57.2%, 80.1%) and 74.3% specificity (95% CI: 63.5%, 85.1%) or 1.67 kPa with 35.3% sensitivity (95% CI: 23.5%, 47.1%) and 91.4% specificity (95% CI: 84.5%, 98.3%). Conclusion In children and young adults, MR elastography performs significantly better for distinguishing stage 0-1 versus stage 2 or higher fibrosis in patients without steatosis than in those with steatosis. This suggests a confounding effect of steatosis or inflammation in the population with nonalcoholic fatty liver disease. © RSNA, 2018.

Secretin-Enhanced Magnetic Resonance Cholangiopancreatography for Assessing Pancreatic Secretory Function in Children.

OBJECTIVE: To assess the accuracy and interrater reproducibility of measurements of pancreatic secretory function by magnetic resonance cholangiopancreatography in response to secretin administration and to describe our experience using the technique to noninvasively assess pancreatic secretory function in a pediatric population. STUDY DESIGN: In the accuracy study, phantoms with varying fluid volume (47-206 mL) were imaged using the clinical quantification sequence. Fluid volume was measured by image segmentation (ImageJ). Measurement accuracy was expressed in terms of error (absolute and percent) relative to known fluid volume. In the reproducibility study and clinical experience, 31 patients with suspected pancreatic disease underwent 33 secretin-enhanced magnetic resonance cholangiopancreatography exams. Two-dimensional T2-weighted, fat-saturated single shot fast spin echo sequences were acquired before and after secretin injection (0.2 µg/kg, max 16 µg). Secreted fluid volume (postsecretin minus presecretin) was independently measured by 2 blinded reviewers. Between reviewer measurement reproducibility was assessed based on correlation (Spearman) and bias (Bland-Altman analysis). RESULTS: For the accuracy study, fluid volumes were measured with mean volume errors of -0.3 to +12.5 mL (percent error -0.03% to +9.0%). For the reproducibility study, the mean secreted fluid volumes measured by reviewer 1 and reviewer 2 were 79.1 ± 54.3 mL (range 5.5-215.4) and 77.2 ± 47.1 mL (range 6.7-198.1 mL), respectively. Measured secreted fluid volumes were very strongly correlated (r = 0.922) between reviewers with a bias of only 1.9 mL (95% limits of agreement -40.5 to 44.2). CONCLUSIONS: Measurement of fluid volume by magnetic resonance imaging is highly accurate with <10% (<13 mL) error in measured volume. Measurements of pancreatic secreted fluid volume in response to secretin by magnetic resonance cholangiopancreatography are highly reproducible with a bias of <2 mL between reviewers.

Specialized Imaging and Procedures in Pediatric Pancreatology: A North American Society for Pediatric Gastroenterology, Hepatology, and Nutrition Clinical Report.

OBJECTIVES: An increasing number of children are being diagnosed with pancreatitis and other pancreatic abnormalities. Dissemination of the information regarding existing imaging techniques and endoscopic modalities to diagnose and manage pancreatic disorders in children is sorely needed. METHODS: We conducted a review of the medical literature on the use of the following imaging and procedural modalities in pediatric pancreatology: transabdominal ultrasonography (TUS), computed tomography (CT), magnetic resonance imaging (MRI)/magnetic resonance cholangiopancreatography (MRCP), endoscopic ultrasonography (EUS), and endoscopic retrograde cholangiopancreatography (ERCP). Recommendations for current use and future research were identified. RESULTS: TUS offers noninvasive images of the pancreas but has limitations to details of parenchyma and ductal structures. CT offers improved detail of pancreatic parenchyma, solid masses, and traumatic injuries, but requires relatively high levels of ionizing radiation and does not adequately assess ductal anatomy. MRI/MRCP offers detailed intrinsic tissue assessment and pancreatic ductal characterization, but requires longer image acquisition time and is relatively poor at imaging calcifications. EUS provides excellent evaluation of pancreatic parenchyma and ductal anatomy, but can be subjective and operator dependent and requires sedation or anesthesia. EUS offers the capacity to obtain tissue samples and drain fluid collections and ERCP offers the ability to improve drainage by performing sphincterotomy or placing pancreatic stents across duct injuries and strictures. CONCLUSIONS: Various imaging modalities may be used in pediatric pancreatology, but TUS and MRI/MRCP are favored. Interventional therapeutic maneuvers primarily involve use of ERCP and EUS. Future research is necessary to optimize equipment, expertise, and appropriate indications.

Diagnostic Performance and Dose Comparison of Filtered Back Projection and Adaptive Iterative Dose Reduction Three-dimensional CT Enterography in Children and Young Adults.

PURPOSE: To investigate diagnostic performance and radiation dose with the use of computed tomographic (CT) enterography in children and young adults and to compare reconstruction with filtered back projection (FBP) to that with adaptive iterative dose reduction (AIDR) with three-dimensional (3D) processing. MATERIALS AND METHODS: This retrospective investigation was HIPAA compliant and approved by the institutional review board. Informed consent was waived. CT enterographic examinations performed between October 2008 and June 2009 with FBP and between August 2012 and April 2014 with AIDR 3D in patients who had received histologic evaluation within 45 days of imaging were included. Two reviewers retrospectively and independently evaluated the studies for findings of active inflammation, and diagnostic performance and interreader reliability were assessed. The reference standard was histologic findings. Objective and subjective image quality also was assessed. The size-specific dose estimate was compared between the two groups. Two-sample t tests or analysis of variance tests were performed to assess for differences in diagnostic accuracy, image quality, and radiation dose between the FBP and AIDR 3D examinations. RESULTS: Fifty patients were included in the FBP group (mean age, 14.1 years; range, 8-21 years) and 68 patients were in the AIDR 3D group (mean age, 13.2 years; range, 2-29 years). Sensitivity and specificity for detection of active inflammation were 96% (26 of 27) and 96% (22 of 23), respectively, for the FBP group and 90% (45 of 50) and 89% (16 of 18), respectively, for the AIDR 3D group. Dichotomous interreader reliability (κ) for the entire group was 0.86. The mean size-specific dose estimate for all weights was significantly lower for the AIDR 3D group (6.1 mGy ± 2.1) than that for the FBP group (16.7 mGy ± 5.2; P < .0001). No significant difference was found in objective image noise for soft-tissue structures (P = .2-.8). CONCLUSION: CT enterography is highly accurate for detection of active inflammation in pediatric patients and has excellent interreader reliability. Reduced-dose CT enterography with AIDR 3D allowed substantial dose reduction compared with that used with FBP CT enterographic examinations, while maintaining a high diagnostic performance.

Relationship of MR elastography determined liver stiffness with cardiac function after Fontan palliation.

PURPOSE: To use MR elastography to assess liver stiffness in patients with congenital heart disease palliated with the Fontan procedure and correlate findings with cardiac index and other functional parameters obtained during cardiac MRI. MATERIALS AND METHODS: We performed a retrospective study of 14 patients (15 examinations) with Fontan circulation who underwent both cardiac MRI and liver MR elastography (MRE) on the same day. Liver stiffness was determined by calculating the mean liver stiffness from four slice locations. The cardiac index and other flow measurements were calculated from phase contrast MR imaging. RESULTS: The MRE was abnormal on all examinations with a median liver stiffness of 4.0 kPa (range, 3.4-6.2 kPa; normal adult liver stiffness is < 2.51 kPa). The cardiac index decreased as the duration of Fontan circulation (Fontan duration) increased (P = 0.005). We found a statistically significant inverse correlation between liver stiffness and cardiac index (P = 0.02), as well as the ejection fraction (P = 0.002). Patients with long Fontan durations (≥ 20 years) had greater liver stiffness compared with those having a shorter duration (P = 0.02). CONCLUSION: MRE shows promise as a monitoring tool in the Fontan population, demonstrating an elevated liver stiffness in all patients, which inversely correlated with the MR determined cardiac index.

Magnetic resonance elastography of the liver in patients status-post fontan procedure: feasibility and preliminary results.

OBJECTIVE: The purpose of this study was to evaluate the feasibility of performing magnetic resonance elastography (MRE) as a screening tool for elevated liver stiffness in patients' status-post Fontan procedure. BACKGROUND: With greater numbers of Fontan patients surviving far into adulthood, a factor increasingly affecting long-term prognosis is the presence of hepatic congestion and fibrosis. If detected early, steps can be taken to potentially slow or halt the progression of fibrosis. MRE is a relatively new, noninvasive imaging technique, which can quantitatively measure liver stiffness and provide an estimate of the extent of fibrosis. METHODS: A retrospective study was conducted using MRE to evaluate liver stiffness in patients with a history of Fontan procedure. An MRE was performed in the same session as a clinical cardiac MRI. The liver was interrogated at four slice locations, and a mean liver stiffness value was calculated for each patient using postprocessing software. The medical records were reviewed for demographic and clinical characteristics. RESULTS: During the time frame of this investigation, 17 MRE exams were performed on 16 patients. All patients had elevated liver stiffness values as defined by MRE standards. The median of the individual mean liver stiffness values was 5.1 kPa (range: 3.4-8.2 kPa). This range of liver stiffness elevation would suggest the presence of mild to severe fibrosis in a patient with standard cardiovascular anatomy. We found a significant trend toward higher liver stiffness values with greater duration of Fontan circulation (rs = 0.55, P = .02). CONCLUSION: Our preliminary findings suggest that MRE is a feasible method for evaluating the liver in Fontan patients who are undergoing surveillance cardiac MRI. Further investigation with histologic correlation is needed to determine the contributions of hepatic congestion and fibrosis to the liver stiffness in this population.

CT and MR enterography in children and adolescents with inflammatory bowel disease.

The term inflammatory bowel disease (IBD) is used to describe multiple idiopathic disorders of the gastrointestinal tract. As many as one-quarter of patients with IBD initially present in childhood or adolescence. Multiple methods can be used to diagnose IBD in this age group, including computed tomographic (CT) enterography, magnetic resonance (MR) enterography, small bowel follow-through examination, ileocolonoscopy, and capsule endoscopy. However, CT enterography and MR enterography have become the imaging modalities of choice due to their exquisite image quality, rapid acquisition time, lack of need for bowel preparation, and ability to help diagnose the extraintestinal complications of IBD. In addition to being radiation free, MR enterography can help evaluate peristalsis, has high contrast resolution, and allows the use of diffusion-weighted imaging. The authors discuss the use of CT enterography and MR enterography in the context of pediatric IBD in terms of advantages and disadvantages, protocol, and imaging findings.

Hepatic pathology after Fontan palliation: spectrum of imaging findings.

BACKGROUND: Patients with congenital heart disease corrected by Fontan palliation have chronic liver congestion that commonly progresses to fibrosis and cirrhosis with resultant complications. OBJECTIVE: To define the hepatic imaging characteristics associated with Fontan circulation. MATERIALS AND METHODS: A retrospective study was performed in patients who underwent Fontan palliation who had CT or MR examinations including the liver. The liver was evaluated for parenchymal morphology, abnormal enhancement, nodules and imaging findings of fibrosis and cirrhosis. RESULTS: MRI or CT examinations including the liver were evaluated in 42 Fontan patients. The most common imaging finding was abnormal parenchymal enhancement, present in 38 patients. Hypervascular nodules were present in 13 patients (31%). Imaging findings of cirrhosis were seen in eight patients (19%). One patient with cirrhosis had a large liver mass, subsequently diagnosed as fibrolamellar hepatocellular carcinoma. CONCLUSION: A high percentage of patients had imaging abnormalities of the liver, chiefly abnormal parenchymal enhancement, which became more apparent as the duration of the Fontan circulation increased. The hypervascular nodules sometimes present had imaging characteristics most closely resembling those of focal nodular hyperplasia. The underlying fibrosis and eventual development of cirrhosis raise the risk of developing hepatocellular carcinoma.

Inflammatory bowel disease in children and adolescents: assessing the diagnostic performance and interreader agreement of magnetic resonance enterography compared to histopathology.

RATIONALE AND OBJECTIVES: The purpose of this study was to determine the accuracy of magnetic resonance enterography (MRE) compared to histopathology in the evaluation of pediatric inflammatory bowel disease and to assess interreader reliability for image interpretation. MATERIALS AND METHODS: All magnetic resonance enterography studies performed for known or suspected inflammatory bowel disease between July 2009 and July 2010 were retrospectively reviewed by two pediatric radiologists. Exams were evaluated for signs of enteric inflammation and extraenteric disease. A five-point, Likert-type scale was used to assess the overall likelihood of active inflammation, with scores ≥ 3 considered positive. Cohen's κ coefficient was calculated to assess interreader agreement. A subset of patients who had undergone ileocolonoscopy or surgery with confirmed histopathology within 45 days of MRE were used to assess the accuracy of MRE for detecting active inflammation in the terminal ileum and large bowel. RESULTS: A total of 91 magnetic resonance enterography studies were reviewed. Of these, 45 had comparison histopathology within 45 days. The overall sensitivity of MRE for detecting active inflammation compared to ileocolonoscopy was 92% for both readers, while specificity was 100% for reader 1 and 75% for reader 2. Of the individual parameters evaluated, mucosal hyperenhancement and bowel wall thickening were the most sensitive indicators of active inflammation, each having sensitivity of 86% and specificity of 88%. Cohen's κ coefficient was 0.59, indicating moderate agreement between the readers. CONCLUSIONS: MRE has high overall diagnostic accuracy for detecting active bowel inflammation in pediatric patients compared to ileocolonoscopy and demonstrates moderate interreader reliability.