Stavros N. Stavropoulos

Characteristics of adult celiac disease in the USA: results of a national survey

OBJECTIVE:The clinical spectrum of adults with celiac disease in the United States, where the disease is considered rare, is not known. We sought this information by distributing a survey.METHODS:A questionnaire was distributed by way of a celiac newsletter, directly to celiac support groups, and through the Internet.RESULTS:Respondents (1612) were from all United States except one. Seventy-five percent (1138) were biopsy proven. Women predominated (2.9:1). The majority of respondents were diagnosed in their fourth to sixth decades. Symptoms were present a mean of 11 yr before diagnosis. Diarrhea was present in 85%. Diagnosis was considered prompt by only 52% and 31% had consulted two or more gastroenterologists. Improved quality of life after diagnosis was reported by 77%. Those diagnosed at age ≥60 yr also reported improved quality of life. Five respondents had small intestinal malignancies (carcinoma 2, lymphoma 3) accounting for a relative risk of 300 (60–876) for the development of lymphoma and 67 (7–240) for adenocarcinoma.CONCLUSIONS:Patients with celiac disease in the United States have a long duration of symptoms and consider their diagnosis delayed. Improved quality of life after diagnosis is common. An increased risk of developing small intestine malignancies is present.

EUS-guided trucut needle biopsies in patients with solid pancreatic masses: a prospective study

BACKGROUNDnA trucut needle biopsy device that can be used to obtain specimens from the pancreas and other perigastric organs under EUS guidance has been developed and successfully tested in animals. Moreover, EUS-guided trucut needle biopsy has been used safely in humans and appears to provide more accurate results than EUS-guided FNA. This study prospectively assessed the clinical utility of this new device in patients with solid pancreatic masses.nnnMETHODSnTwenty-three consecutive patients with radiologically detected solid pancreatic masses underwent EUS-guided trucut needle biopsy. Pancreatic malignancy detected by EUS-guided trucut needle biopsy was considered a definitive diagnosis. Further diagnostic procedures and clinical course were used to establish or exclude the presence of malignancy in all other patients.nnnRESULTSnPancreatic tissue was obtained in 17 of the 23 patients (74%), including all patients in whom the transgastric approach was used. No acute or long-term complication was observed. Histopathologic evaluation revealed pancreatic cancer in 12 patients. CT-guided biopsy specimens were obtained in 4 of the 5 patients with a negative EUS-guided trucut needle biopsy result; two were positive for adenocarcinoma. Overall diagnostic accuracy was 61%. Subgroup analysis of the 16 patients in whom EUS-guided trucut needle biopsy was successful and who were available for follow-up revealed a diagnostic accuracy of 87.5%.nnnCONCLUSIONSnThis prospective study demonstrates that EUS-guided trucut needle biopsy, when performed transgastrically, is safe and accurate in the evaluation of patients with solid pancreatic masses.

Presentation of Pediatric Celiac Disease in the United States: Prominent Effect of Breastfeeding

Childhood celiac disease (CD) is considered rare in the United States. Consequently there are few data concerning its clinical presentation. A validated questionnaire was distributed to families of children with CD. One hundred forty-one children with biopsy-proven CD were included in the study. We found significant differences in the clinical spectrum of children based on their infant feeding history. Exclusively breastfed children were significantly less likely to report failure to thrive (69% vs 88%, p<0.05) and short stature (37% vs 62%, p<0.05), and had a higher rate of “atypical” symptoms (p<0.01). Breastfeeding alters the presentation and contributes to atypical presentations of CD and diagnostic delay. Pediatricians need to be aware of the diverse manifestations of celiac disease to reduce diagnostic delay.

The Role of Molecular Analysis in the Diagnosis and Surveillance of Pancreatic Cystic Neoplasms

CONTEXTnMolecular analysis of pancreatic cyst fluid obtained by EUS-FNA may increase diagnostic accuracy. We evaluated the utility of cyst-fluid molecular analysis, including mutational analysis of K-ras, loss of heterozygosity (LOH) at tumor suppressor loci, and DNA content in the diagnoses and surveillance of pancreatic cysts.nnnMETHODSnWe retrospectively reviewed the Columbia University Pancreas Center database for all patients who underwent EUS/FNA for the evaluation of pancreatic cystic lesions followed by surgical resection or surveillance between 2006-2011. We compared accuracy of molecular analysis for mucinous etiology and malignant behavior to cyst-fluid CEA and cytology and surgical pathology in resected tumors. We recorded changes in molecular features over serial encounters in tumors under surveillance. Differences across groups were compared using Students t or the Mann-Whitney U test for continuous variables and the Fishers exact test for binary variables.nnnRESULTSnAmong 40 resected cysts with intermediate-risk features, molecular characteristics increased the diagnostic yield of EUS-FNA (n=11) but identified mucinous cysts less accurately than cyst fluid CEA (P=0.21 vs. 0.03). The combination of a K-ras mutation and ≥2 loss of heterozygosity was highly specific (96%) but insensitive for malignant behavior (50%). Initial data on surveillance (n=16) suggests that molecular changes occur frequently, and do not correlate with changes in cyst size, morphology, or CEA.nnnCONCLUSIONSnIn intermediate-risk pancreatic cysts, the presence of a K-ras mutation or loss of heterozygosity suggests mucinous etiology. K-ras mutation plus ≥2 loss of heterozygosity is strongly associated with malignancy, but sensitivity is low; while the presence of these mutations may be helpful, negative findings are uninformative. Molecular changes are observed in the course of cyst surveillance, which may be significant in long-term follow-up.

Per Oral Endoscopic Myotomy, Equipment and Technique: A Step-by-Step Explanation

Abstract Per oral endoscopic myotomy (POEM) represents a natural orifice endoscopic surgical technique for ‘scarless Heller myotomy for the treatment of achalasia. It utilizes a submucosal tunnel approach that offsets the mucosal and muscle incisions and thus allows secure closure with a few clips on the mucosotomy site. The first POEM procedure was performed in Japan by Haruhiro Inoue in 2008. Since then POEM has steadily grown to several hundred procedures worldwide at this point. Published data are limited but demonstrate high efficacy and low complication rates at expert centers pioneering this procedure. POEM techniques are reviewed, along with tips and tricks, in the submitted video. It should be emphasized that POEM is a challenging endoscopic surgical procedure that requires advanced endoscopic resectional skills, particularly endoscopic submucosal dissection skills. There is unanimity among current operators that POEM should be performed under institutional review board approval and after appropriate laboratory training and proctoring of the initial human POEM case(s). There is very strong consensus in favor of rigorous external accreditation and monitoring of outcomes to ensure competence and safety. This article is part of an expert video encyclopedia.

7021 Intraductal ultrasound (idus): experience in a us tertiary care center.

Introduction: IDUS may be a useful adjunct to ERCP. Aim:To report our initial experience with this modality. Methods:We reviewed all IDUSs performed at our institution over the past 14 months. Results: Using the Olympus UM-3R 20 MHz and UM-2R 12 MHz probes,37 IDUSs were performed on 32 patients (17 M, 15 W) of mean age 67 yrs (50-89). Twentynine biliary, 7 pancreatic, 5 ampullary, and 1 intracystic IDUS were performed. Surgically altered anatomy was present in 5 patients (2 Billroth II, 1 liver transplantation, 1 distal pancreatectomy, 1 choledochojejunostomy). The bile duct (BD) was accessed via a native papilla (10), biliary sphincterotomy (ST) (14), surgical choledochojejunostomy (1), or metal stent (1). The pancreatic duct (PD) was accessed via a native papilla (1), biliary ST (2), pancreatic ST (1) or both (2). Eighteen malignancies and 14 benign conditions were imaged. The malignancies were 8 cholangiocarcinomas (CCAs), (1 intrahepatic, 2 proximal, 2 mid-duct, 3 distal), 2 ampullary adenocarcinomas (adCAs), 2 pancreatic intraductal papillary mucinous tumors (IPMTs), 3 metastatic cancers with BD strictures (1 gastric, 2 adCAs of unknown origin), and 3 pancreatic adCAs. Malignancy was established by surgery in 13 patients and by biopsy/cytology in 5. The benign conditions were ampullary fibrosis/papillary stenosis (7), stones/sludge (7), postoperative PD stricture with communicating pseudocyst (1), PD stricture in chronic pancreatitis (1), periampullary cyst (1), normal duct (1). Malignant lesions imaged by IDUS consisted of masses (4), mural-based nodules (2 CBD, 1 CHD), strictures (7 CBD, 2 CHD, 2 PD),and mucin, mural nodules and epithelial projections in the IPMTs. Of the 18 malignancies, 15 had surgically confirmed vascular staging. Vascular involvement was correctly predicted in 2 cases and correctly excluded in 13 cases. Benign lesions imaged by IDUS included sludge/stones (7), PD strictures (2), hyperechoic areas in pancreatic parenchyma (in the one case with chronic pancreatitis). Malignant strictures appeared as thickened hypoechoic areas that obliterated the duct layers present in normal duct segments. Nodules and mural masses appeared hypoechoic with heterogeneous echotexture in the larger masses. Stones and sludge were highly echogenic.In 6 out of the 7 cases of stones/sludge the diagnosis had not been established during ERCP. Conclusion: IDUS delineates normal wall layers and their effacement in malignant strictures and is accurate for vascular staging and the detection of small stones and sludge.

4608 A randomized comparision of two biliary tissue sampling techniques for the detection of malignant strictures.

Background: Brush cytology is commonly used to evaluate potentially malignant strictures at ERCP. A new device with a second large lumen has been developed to allow the passage of accessories into the duct. We hypothesize that brushing obtained with this device would have a higher yield than a standard system because of the side pressure exerted by the catheter on the brush in the stricture. We report an alternating randomized trial comparing the accuracy of two systems. Methods: Method 1 uses the Cytomax brush system (CBS),Wilson-Cook, with an 8Fr-sheath diameter and a 2.5cm brush length. Brushings are obtained after stricture dilation with a separate 10Fr catheter. Method 2 uses the Howell Biliary system (HBS), Wilson-Cook, a 10Fr system with a separate channel to allow the passage of 5Fr sampling devices. The delivery device is used to dilate the stricture before brushings. In both systems bile was aspirated prior to brushings. The brush, slides, and fluid are sent for cytologic evaluation. Both catheter systems were flushed with sterile saline to salvage any cells within the sheath. Biliary biopsy forceps and FNA catheters were not routinely used. Results: 23 consecutive patients were enrolled, 11 CBS and 12 HBS. No significant demographic difference was present. The CBSgroup had 9 CBD and 2 IHD strictures (4 pancreatic adenocarcinoma, 2 cholangiocarcinoma, 5 benign strictures). This brushing technique alone had an accuracy of 81% with a sensitivity and specificity of 83% and 80%. The combination of brushing and aspirate had an accuracy 82% with sensitivity, specificity, PPV, and NPV of 83%, 80%, 83%, and 80%, respectively. The HBSgroup had 10 CBD, 1 IHD, and 1 pancreatic stricture (6-pancreatic adenocarcinoma, 1-cholangiocarcinoma, 1-metastatic gastric adenoca, 1- metastatic renal cell ca, and 3-benign strictures). This brushing technique alone had an accuracy of 67% with a sensitivity and specificity of 56% and 100%. The combination of brushing and aspirates had an accuracy of 75%, with sensitivity, specificity, PPV, NPV of 67%, 100%, 100%, and 50%, respectively. The biopsy forceps were available in 3/12 patients. All specimens were satisfactory for interpretation and there were 2 true positives and 1 true negative. Overall, the CBS accuracy and HBS accuracy for brush + aspiration cytology were 82% vs. 75% (NS, p>.05, Fisher s Exact). Conclusion: Our preliminary results suggest that the HBS sytem does not increase yield when compared to the CBS system unless intraductal biopsies are performed.