Reappraising imaging features of pancreatic acinar cystic transformation: be aware of differential diagnoses.

OBJECTIVES: Imaging features of pancreatic acinar cystic transformation (ACT) have been published. We aimed to describe the clinical and radiological characteristics of patients with a presumed pancreatic ACT diagnosis, reappraising the value of these published imaging criteria. MATERIALS AND METHODS: Single-center retrospective study (2003-2021) of consecutive patients with a presumed diagnosis of ACT as suggested by the local expert multidisciplinary case review board. Patients without available imaging (CT or MRI) for review were excluded. Patients were classified into "certain" ACT (if ≥ 2 imaging criteria and no differential diagnosis) or "uncertain" ACT (if ≥ 1 imaging criteria and suggested differential diagnoses). RESULTS: Sixty-four patients (35 males, [55%]) were included. ACT was considered "certain" for 34 patients (53%) and "uncertain" for 30 patients (47%). The number of ACT criteria did not differ between groups, with 91.2% of patients with ≥ 3 ACT imaging criteria in the "certain" group vs 93.3% in the "uncertain" group (p = 0.88). In the "uncertain" group, the main suggested differentials were branch-duct intraductal papillary mucinous neoplasm (18/30 patients, 60%), calcifying chronic pancreatitis (8/30 patients, 27%), both (three patients, 10%) and serous cystadenoma (one patient, 3%). Calcifications were significantly more frequent in the "uncertain" group (89% vs 63% in the "certain" group, p = 0.02). CONCLUSION: Published ACT imaging criteria are frequently associated with features suggesting differential diagnoses. They appear insufficient to reach a final diagnosis in a subset of patients. CLINICAL RELEVANCE STATEMENT: ACT displays a heterogeneous morphological imaging presentation challenging the non-invasive diagnostic work-up. Physicians' and radiologists' awareness of this entity is important to better understand its natural history and improve non-invasive diagnostic criteria. KEY POINTS: The criteria to help diagnose ACT are frequently associated with features suggestive of differentials. The main alternatives suggested when ACT diagnosis was "uncertain" were branch-duct intraductal papillary mucinous neoplasm and calcifying chronic pancreatitis. Published ACT diagnostic imaging criteria can be insufficient for a definite non-invasive diagnosis.

Pancreatic Acinar Cystic Transformation: Familial Forms Do Exist.

ABSTRACT: Pancreatic acinar cystic transformation (ACT) is a recently described entity of the pancreatic cysts' spectrum. It is a nonneoplastic pancreatic lesion, mostly asymptomatic, and is considered a rare disease, with less than 100 reported cases. Its benign nature and the absence of reported cases of malignant transformation or invasive lesions were a plea for a conservative approach. As a consequence, little is known about the pathogenesis of this lesion. No familial history of ACT has been previously described. Here, we report a familial case of 2 siblings with typical ACT imaging lesions giving evidence for familial aggregation. No obvious environmental exposure was identified as a potential risk factor for ACT development. No physiological data exist to suggest a congenital nature of these lesions, but for the first patient, the calcifications seem to have appeared with time. Further research, with high throughput sequencing technologies, may elucidate genetic polymorphisms explaining potential ACT familial phenotype. In practice, careful family history collection must be performed to identify a possible familial form of ACT when this diagnosis is proposed.

MRI for the Detection of Small Bowel Ischemic Injury in Arterial Acute Mesenteric Ischemia: Preclinical Study in a Porcine Model.

BACKGROUND: MRI is the reference for the diagnosis of arterial cerebral ischemia, but its role in acute mesenteric ischemia (AMI) is poorly known. PURPOSE: To assess MRI detection of early ischemic bowel lesions in a porcine model of arterial AMI. STUDY TYPE: Prospective/cohort. ANIMAL MODEL: Porcine model of arterial AMI obtained by embolization of the superior mesenteric artery (seven pigs). FIELD STRENGTH/SEQUENCE: A 5-T. T1 gradient-echo-weighted-imaging (WI), half-Fourier-acquisition-single-shot-turbo-spin-echo, T2 turbo-spin-echo, true-fast-imaging-with-steady-precession (True-FISP), diffusion-weighted-echo-planar (DWI). ASSESSMENT: T1-WI, T2-WI, and DWI were performed before and continuously after embolization for 6 hours. The signal intensity (SI) of the ischemic bowel was assessed visually and quantitatively on all sequences. The apparent diffusion coefficient (ADC) was assessed. STATISTICAL TESTS: Paired Student's t-test or Mann-Whitney U-test, significance at P < 0.05. RESULTS: One pig died from non-AMI-related causes. The remaining pigs underwent a median 5 h53 (range 1 h24-6 h01) of ischemia. Visually, the ischemic bowel showed signal hyperintensity on DWI-b800 after a median 85 (57-276) minutes compared to the nonischemic bowel. DWI-b800 SI significantly increased after 2 hours (+19%) and the ADC significant decrease within the first hour (-31%). The ischemic bowel was hyperintense on precontrast T1-WI after a median 87 (70-171) minutes with no significant quantitative changes over time (P = 0.46-0.93). The ischemic bowel was hyperintense on T2-WI in three pigs with a significant SI increase on True-FISP after 1 and 2 hours. DATA CONCLUSION: Changes in SI and ADC can be seen early after the onset of arterial AMI with DWI. The value of T2-WI appears to be limited. EVIDENCE LEVEL: 1 TECHNICAL EFFICACY: Stage 2.

Crohn-like Disease Affecting Small Bowel Due to Monogenic SLCO2A1 Mutations: First Cases of Chronic Enteropathy Associated with SLCO2A1 Gene [CEAS] in France.

INTRODUCTION: Multiple chronic ulcers of small intestine are mainly ascribed to Crohn's disease. Among possible differential diagnoses are chronic ulcers of small bowel caused by abnormal activation of the prostaglandin pathway either in the archetypal but uncommon non-steroidal anti-inflammatory drug [NSAID]-induced enteropathy, or in rare monogenic disorders due to PLA2G4A and SLCO2A1 mutations. SLCO2A1 variants are responsible for CEAS [chronic enteropathy associated with SLCO2A1], a syndrome which was exclusively reported in patients of Asian origin. Herein, we report the case of two French female siblings, P1 and P2, with CEAS. CASE REPORT: P1 underwent iterative bowel resections [removing 1 m of small bowel in total] for recurrent strictures and perforations. Her sister P2 had a tight duodenal stricture which required partial duodenectomy. Next-generation sequencing was performed on P1's DNA and identified two compound heterozygous variants in exon 12 in SLCO2A1, which were also present in P2. CONCLUSION: CEAS can be detected within the European population and raises the question of its incidence and recognition outside Asia. Presence of intractable recurrent ulcerations of the small intestine, mimicking Crohn's disease with concentric strictures, should motivate a genetic search for SLCO2A1 mutations, particularly in the context of family history or consanguinity.

Colonic involvement in acute mesenteric ischemia: prevalence, risk factors, and outcomes.

OBJECTIVES: This study aimed to investigate the prevalence, risk factors, and outcomes of colonic involvement in patients with acute mesenteric ischemia (AMI). METHODS: CT scans from a prospective cohort of 114 AMI patients treated in an intestinal stroke center between 2009 and 2018 were blindly reviewed by two radiologists. Colon involvement was defined on CT scan by the presence of at least one of the following CT colonic features: wall thickening, pneumatosis, decreased wall enhancement, dilatation, or perforation. In addition, the clinical, biological, and radiological characteristics of patients with and without colonic involvement were compared to identify risk factors for colonic involvement on CT and its impact on morbidity and mortality. RESULTS: Colonic involvement was identified in 32/114 (28%) patients with AMI, the right colon being more frequently involved (n = 29/32, 91%). Wall thickening (n = 27/32) was the most common CT finding. Occlusion of the inferior mesenteric artery was the only statistically significant risk factor for colonic involvement (35% vs. 15%, p = 0.02). Patients with colonic involvement on CT vs. those without had more frequently transmural colonic necrosis (13% vs. 0%, p = 0.006), short bowel syndrome (16% vs. 4%, p = 0.04), need for long-term parenteral support (19% vs. 5%, p = 0.03), and death during follow-up (22% vs. 10%, p = 0.03). DISCUSSION: In patients with AMI, colonic involvement is associated with increased morbidity and mortality and should be carefully searched for during initial CT scan assessment. KEY POINTS: • In a prospective cohort of acute mesenteric ischemia patients from an intestinal stroke center, 28% had an associated colonic involvement on CT. • Colonic involvement on CT most commonly affected the right colon, and the occlusion of the inferior mesenteric artery was the only risk factor. • Colonic involvement on CT was associated with increased morbidity and mortality and should be carefully searched for during initial CT scan assessment.

Enhancing capsule in hepatocellular carcinoma: intra-individual comparison between CT and MRI with extracellular contrast agent.

PURPOSE: The purpose of this study was to compare the value of contrast-enhanced computed tomography (CT) to that of magnetic resonance imaging obtained with extracellular contrast agent (ECA-MRI) for the diagnosis of a tumor capsule in hepatocellular carcinoma (HCC) using histopathologic findings as the standard of reference. MATERIALS AND METHODS: This retrospective study included patients with pathologically-proven resected HCCs with available preoperative contrast-enhanced CT and ECA-MRI examinations. Two blinded radiologists independently reviewed contrast-enhanced CT and ECA-MRI examinations to assess the presence of an enhancing capsule. The histopathological analysis of resected specimens was used as reference for the diagnosis of a tumor capsule. The sensitivity and specificity of CT and ECA-MRI for the diagnosis of a tumor capsule were determined, and an intra-individual comparison of imaging modalities was performed using McNemar test. Inter-reader agreement was assessed using Kappa test. RESULTS: The study population included 199 patients (157 men, 42 women; mean age: 61.3 ± 13.0 [SD] years) with 210 HCCs (mean size 56.7 ± 43.7 [SD] mm). A tumor capsule was present in 157/210 (74.8%) HCCs at histopathologic analysis. Capsule enhancement was more frequently visualized on ECA-MRI (R1, 68.6%; R2, 71.9%) than on CT (R1, 44.3%, P < 0.001; R2, 47.6%, P < 0.001). The sensitivity of ECA-MRI was better for the diagnosis of histopathological tumor capsule (R1, 76.4%; R2, 79.6%; P < 0.001), while CT had a greater specificity (R1, 84.9%; R2, 83.0%; P < 0.001). Inter-reader agreement was moderate both on CT (kappa = 0.55; 95% confidence interval [CI]: 0.43-0.66) and ECA-MRI (kappa = 0.57; 95% CI: 0.45-0.70). CONCLUSION: Capsule enhancement was more frequently visualized on ECA-MRI than on CT. The sensitivity of ECA-MRI was greater than that of CT, but the specificity of CT was better than that of ECA-MRI.

Imaging as predictor of clinical response to teduglutide in adult patients with short bowel syndrome with chronic intestinal failure.

BACKGROUND: Teduglutide (TED) is a glucagon-like peptide 2 analogue approved in patients with short bowel syndrome with chronic intestinal failure. Bowel epithelial hyperplasia has been reported after TED treatment. OBJECTIVE: The aim of this study was to describe small bowel modifications at imaging in patients with SBS-CIF receiving TED and to assess their predictive value for clinical response. METHODS: Monocentric retrospective study including patients with SBS-CIF treated with TED from 2009 to 2018 with available computed tomography (CT) scans at baseline and during follow-up (≥12 mo). Small bowel (SB) wall thickness was measured as the average of 3 measurements on different SB segments. Clinical response to TED was defined as a ≥20% reduction of weekly parenteral support (PS) volume at 12 mo. RESULTS: Thirty-one patients [20 male (65%), median age 51 y (IQR: 37-59)] were included. Baseline weekly PS volume was a median 7500 mL (IQR: 3500-15,000). After a median (IQR) follow-up of 16 mo (14-27), 26 of 31 patients (84%) had a clinical response. During follow-up, patients underwent 1 (n = 18/31, 58%), 2 (10/31, 32%), or 3 (3/31 10%) CT scans. Median SB wall thickness was 4.0 mm (IQR: 2.8-4.7) and 8.5 mm (IQR: 6.1-9.8) at baseline and after treatment, respectively [paired P < 0.001, median +122% increase (IQR: +65% to +172%)]. Patients with a clinical response had a trend toward a higher SB wall thickness increase [median +133% (IQR: +70% to +176%) compared with +90% (IQR: +52% to +93%), P = 0.061]. All patients with a ≥95% SB wall thickness increase (n = 18) had a clinical response, whereas only 8 of 13 (62%) patients with a <95% SB thickness increase did (P = 0.008). CONCLUSIONS: Teduglutide induces a significant SB wall thickness increase that can be depicted by imaging <6 mo after treatment initiation, and the degree of such increase may be associated with clinical response. Bowel imaging in response to pharmacologic treatments may represent an important outcome to follow.