CT findings in idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) and comparison to other colitides.

PURPOSE: The purpose of this study is to determine computed tomography (CT) findings that aid in differentiating idiopathic myointimal hyperplasia of mesenteric veins (IMHMV) from other colitides. METHODS: Retrospective review of histiologic proven cases of IMHMV (n = 12) with contrast enhanced CT (n = 11) and/or computed tomography angiography (CTA) (n = 9) exams. Control groups comprised of CT of infectious colitis (n = 13), CT of inflammatory bowel disease (IBD) (n = 12), and CTA of other colitides (n = 13). CT exams reviewed by 2 blinded gastrointestinal radiologists for maximum bowel wall thickness, enhancement pattern, decreased bowel wall enhancement, submucosal attenuation value, presence and location of IMV occlusion, peripheral mesenteric venous occlusion, dilated pericolonic veins, subjective IMA dilation, maximum IMA diameter, maximum peripheral IMA branch diameter, ascites, and mesenteric edema. Presence of early filling veins was an additional finding evaluated on CTA exams. RESULTS: Statistically significant CT findings of IMHMV compared to control groups included greater maximum bowel wall thickness, decreased bowel enhancement, IMV occlusion, and peripheral mesenteric venous occlusion (p < 0.05). Dilated pericolonic veins were seen more frequently in IMHMV compared to the infectious colitis group (64% versus 15%, p = 0.02). Additional statistically significant finding on CTA included early filling veins in IMHMV compared to the CTA control group (100% versus 46%, p = 0.008). CONCLUSION: IMHMV is a rare chronic non-thrombotic ischemia predominantly involving the rectosigmoid colon. CT features that may aid in differentiating IMHMV from other causes of left-sided colitis include marked bowel wall thickening with decreased enhancement, IMV and peripheral mesenteric venous occlusion or tapering, and early filling of dilated veins on CTA.

Primary Pancreatic Undifferentiated Pleomorphic Sarcoma.

Primary pancreatic sarcomas are rare malignancies with an incidence of 0.1%. This case report is of a 48-year-old man who presented with this condition. The patient's treatment plan consisted of distal pancreatectomy and splenectomy with intraoperative immunohistochemistry and adjuvant chemotherapy. To correctly identify and treat undifferentiated pleomorphic sarcoma, a stepwise strategy involving cross-sectional imaging and extensive histopathology analysis is necessary.

Cronkhite-Canada syndrome: treatment responses and improved overall survival.

BACKGROUND: Cronkhite-Canada syndrome (CCS) is considered a relentlessly progressive disease with high mortality rates. Although disease understanding and treatment options have greatly improved, the prognosis from these advancements has not been well documented. This study aimed to evaluate treatment outcomes and overall survival of CCS. METHODS: Seventeen patients who were diagnosed and treated over a 20-year period at Mayo Clinic (Rochester, Minnesota) were included. Data were abstracted, which included clinical and endoscopic manifestations, treatment course, and survival outcomes. RESULTS: The median (interquartile range) duration of follow-up was 8.3 (3.7-15.8) years. All patients received an initial prednisone dose equivalence of 30-80 mg daily, and five patients required steroids at the time of the last follow-up. Twelve patients trialed thiopurine therapy, and ten patients continued with a thiopurine until the last follow-up. Fifteen patients achieved clinical complete remission, and eleven patients achieved endoscopic complete remission after pharmacotherapy initiation. Seven patients required gastrointestinal surgeries during their disease course. The 5-year overall survival was 93.3% (95% confidence interval (CI): 81.5-100%), and the 3-year relapse-free survival was 82.4% (95% CI: 66.1-100%). CONCLUSION: The prognosis and overall survival of patients with CCS have markedly improved with advancement in disease understanding and therapies. Pharmacotherapy, including corticosteroids and immunomodulators, is effective in inducing and maintaining remission, and gastrointestinal surgery is commonly needed as an adjunct for managing CCS disease complications.

Three-Year Interval for the Multi-Target Stool DNA Test for Colorectal Cancer Screening: A Longitudinal Study.

Data supporting the clinical utility of multi-target stool DNA (mt-sDNA) at the guideline-recommended 3-year interval have not been reported.Between April 2015 and July 2016, candidates for colorectal cancer screening whose providers prescribed the mt-sDNA test were enrolled. Participants with a positive baseline test were recommended for colonoscopy and completed the study. Those with a negative baseline test were followed annually for 3 years. In year 3, the mt-sDNA test was repeated and colonoscopy was recommended independent of results. Data were analyzed using the Predictive Summary Index (PSI), a measure of the gain in certainty for dichotomous diagnostic tests (where a positive value indicates a net gain), and by comparing observed versus expected colorectal cancers and advanced precancerous lesions.Of 2,404 enrolled subjects, 2,044 (85%) had a valid baseline mt-sDNA result [284 (13.9%) positive and 1,760 (86.1%) negative]. Following participant attrition, the year 3 intention to screen cohort included 591 of 1,760 (33.6%) subjects with valid mt-sDNA and colonoscopy results, with no colorectal cancers and 63 advanced precancerous lesions [22 (34.9%) detected by mt-sDNA] and respective PSI values of 0% (P = 1) and 9.3% (P = 0.01). The observed 3-year colorectal cancer yield was lower than expected (one-sided P = 0.09), while that for advanced precancerous lesions was higher than expected (two-sided P = 0.009).Repeat mt-sDNA screening at a 3-year interval resulted in a statistically significant gain in detection of advanced precancerous lesions. Due to absence of year 3 colorectal cancers, the PSI estimate for colorectal cancer was underpowered and could not be reliably quantified. Larger studies are required to assess the colorectal cancer study findings. PREVENTION RELEVANCE: Understanding the 3-year yield of mt-sDNA for colorectal cancer and advanced precancerous polyps is required to ensure the clinical appropriateness of the 3-year interval and to optimize mt-sDNA's screening effectiveness.

A Practical 5-Step Approach to Nausea and Vomiting.

Nausea and vomiting (N/V) are common presenting complaints in the outpatient and inpatient settings. These symptoms can be associated with high morbidity and poor quality of life, particularly in those with chronic symptoms. The clinical approach to N/V can be challenging, given the numerous possible underlying causes as well as the vast array of diagnostic and therapeutic options. In this concise review, we provide a practical 5-step approach to the clinical evaluation and treatment of N/V, suitable for application in the primary care and subspecialty settings. The 5-step approach includes (1) defining what the patient means by N/V, (2) determining whether symptoms are acute or chronic, (3) considering medication or toxin adverse effects, (4) using the patient's presentation, severity of symptoms, and physical examination findings to formulate a differential diagnosis and to guide evaluation, and (5) directing treatment on the basis of knowledge of neurotransmitters and receptors involved in the emetic pathways. We discuss the pathophysiology (neuronal pathways and neurotransmitters), differential diagnosis (medication and toxin adverse effects, neurologic causes, gastrointestinal diseases, metabolic and endocrine conditions, and psychogenic disorders), initial evaluation and risk stratification, and management and treatment options. Management of symptoms that are acute in onset or mild in severity may involve an empirical trial of antiemetics without extensive testing. In contrast, when symptoms are chronic or moderate-severe, testing for an underlying cause should be performed, and medication adverse effects, neurologic causes, gastrointestinal diseases, metabolic or endocrine conditions, and psychogenic disorders should be considered in particular.

SMAD4 mutation and the combined juvenile polyposis and hereditary hemorrhage telangiectasia syndrome: a single center experience.

PURPOSE: Mutations of the SMAD4 gene can result in a distinct syndrome with combined clinical features of both juvenile polyposis syndrome (JPS) and hereditary hemorrhagic telangiectasia (HHT). Even though it is known that patients with the overlap syndrome are at increased risk for colorectal malignancies and bleeding, the outcomes of this patient population have not been extensively studied. METHODS: Retrospective study aiming to describe the phenotype and clinical outcomes of patients with genetically confirmed JP-HHT combined syndrome in a single large tertiary center in North America. RESULTS: A total of 22 patients were identified, the majority females (59%) with a median age diagnosis at 24 years. Polyps were more commonly seen in the lower gastrointestinal (GI) tract, and tubular adenomas were seen in 50%. Epistaxis and pulmonary arteriovenous malformations (AVM) were the most common manifestations of HHT, with a median Curacao score of 3 [1-4]. Hospitalization for gastrointestinal bleeding and cerebrovascular events occurred at a rate of 28% and 4%, respectively. Two patients had GI malignancies, one rectal and one small bowel adenocarcinoma. Overall mortality was 14%. CONCLUSIONS: Patients with the combined JP-HHT syndrome remain at risk for life-threatening vascular complications and gastrointestinal malignancies; close follow-up is necessary to minimize morbidity and mortality in this patient population.

Colonic Manifestations and Complications Are Relatively Under-Reported in Systemic Sclerosis: A Systematic Review.

OBJECTIVES: Although systemic sclerosis (SSc) is known to affect the gastrointestinal (GI) tract, most of the literature focuses on esophageal, small intestinal, or anorectal manifestations. There have been no reviews focused on large bowel SSc complications in over 30 years. The aim of this study is to perform a systematic review of colonic manifestations and complications of SSc. METHODS: An experienced librarian conducted a search of databases, including English and Spanish articles. The search used keywords including "systemic sclerosis," "scleroderma," and "colon." A systematic review was performed using Preferred Reporting Items for Systematic Review and Meta-Analyses guidelines. Case reports/series were screened for validity by adapting from criteria published elsewhere. RESULTS: Of 1,890 articles, 74 met selection criteria. Fifty-nine of the 77 articles were case reports/series. The most common article topics on colonic SSc complications were constipation/dysmotility (15), colonic volvulus (8), inflammatory bowel disease (7), microscopic colitis (6), megacolon (6), and telangiectasia (6). Colonic manifestations constituted 24% of articles on GI complications of SSc. There were a total of 85 cases (84% women, with a median age of onset of colon complication of 52 years). Limited cutaneous SSc phenotype (65.6%) was more common than diffuse (26.2%). Patients frequently had poor outcomes with high mortality related to colonic complications (27%). Recent studies explore contemporary topics such as the microbiome in SSc and prucalopride for chronic constipation in SSc. DISCUSSION: Colonic complications comprise a large proportion of the published reports on GI symptoms afflicting patients with SSc and require raised diagnostic suspicion and deliberate action to avoid potentially serious complications including death.

Efficacy of Difluoromethylornithine and Aspirin for Treatment of Adenomas and Aberrant Crypt Foci in Patients with Prior Advanced Colorectal Neoplasms.

Difluoromethylornithine (DFMO), an inhibitor of polyamine synthesis, was shown to act synergistically with a NSAID for chemoprevention of colorectal neoplasia. We determined the efficacy and safety of DFMO plus aspirin for prevention of colorectal adenomas and regression of rectal aberrant crypt foci (ACF) in patients with prior advanced adenomas or cancer. A double-blinded, placebo-controlled trial was performed in 104 subjects (age 46-83) randomized (1:1) to receive daily DFMO (500 mg orally) plus aspirin (325 mg) or matched placebos for one year. All polyps were removed at baseline. Adenoma number (primary endpoint) and rectal ACF (index cluster and total) were evaluated at a one year colonoscopy. ACF were identified by chromoendoscopy. Toxicity was monitored, including audiometry. Eighty-seven subjects were evaluable for adenomas or ACF modulation (n = 62). At one year of treatment, adenomas were detected in 16 (38.1%) subjects in the DFMO plus aspirin arm (n = 42) versus 18 (40.9%) in the placebo arm (n = 44; P = 0.790); advanced adenomas were similar (n = 3/arm). DFMO plus aspirin was associated with a statistically significant reduction in the median number of rectal ACF compared with placebo (P = 0.036). Total rectal ACF burden was also reduced in the treatment versus the placebo arm relative to baseline (74% vs. 45%, P = 0.020). No increase in adverse events, including ototoxicity, was observed in the treatment versus placebo arms. While adenoma recurrence was not significantly reduced by one year of DFMO plus aspirin, the drug combination significantly reduced rectal ACF number consistent with a chemopreventive effect.