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      Over-The-Scope-Clip pre-mounted onto a double balloon enteroscope for fast and successful closure of post-EMR jejunal perforation: case report

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          Abstract

          Background

          Familial adenomatous polyposis (FAP) is a rare, autosomal dominant disease clinically characterized by the early onset of many adenomatous polyps throughout the colon, which turn into colon cancer, if left untreated. In FAP patients, polyps can also occur in the upper gastrointestinal (GI) tract, especially in the duodenum. Adenomas beyond duodenum are rare and mostly located in the proximal jejunum and distal ileum. The management of such polyps can be either surgical or endoscopic, depending on the features of the polyp, Spigelman stage and patient’s clinical conditions. Endoscopic mucosal resection (EMR) of jejunal polyps can be challenging, because of the thinner wall of jejunum, compared to the rest of the GI tract, and of the difficulty of maintaining control and stability of the scope. For these reasons, jejunal perforation is a likely occurrence.

          Case presentation

          A 65-year-old woman with a stage IV FAP, who had previously undergone abdominal surgery because of her disease, came to our attention because of numerous adenomatous-looking duodenal polyps and a 25 mm lesion in proximal jejunum. According to Spigelman staging system, patient was candidate for surgical resection, in light of the risk of developing small bowel cancer. Despite the benefits of surgery were clearly explained to her, she refused to undergo small bowel resection. Therefore, EMR of the largest duodenal polyp and of the jejunal lesion was planned. After the removal of the jejunal polyp, a small perforation was noted. We were able to rapidly close such perforation by using the Over-The-Scope-Clip system (OTSC, 12/6 t; Ovesco, Tübingen, Germany) pre-mounted onto a double balloon (DB) enteroscope.

          Conclusions

          The endoscopic management of jejunal perforation can be tricky and the placement of traditional through-the-scope clips in a narrow space like jejunum may be difficult and time consuming.

          This case describes the use of the OTSC system pre-mounted onto a DB enteroscope for the closure of post-EMR jejunal perforation.

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          Most cited references 10

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          ACG clinical guideline: Genetic testing and management of hereditary gastrointestinal cancer syndromes.

          This guideline presents recommendations for the management of patients with hereditary gastrointestinal cancer syndromes. The initial assessment is the collection of a family history of cancers and premalignant gastrointestinal conditions and should provide enough information to develop a preliminary determination of the risk of a familial predisposition to cancer. Age at diagnosis and lineage (maternal and/or paternal) should be documented for all diagnoses, especially in first- and second-degree relatives. When indicated, genetic testing for a germline mutation should be done on the most informative candidate(s) identified through the family history evaluation and/or tumor analysis to confirm a diagnosis and allow for predictive testing of at-risk relatives. Genetic testing should be conducted in the context of pre- and post-test genetic counseling to ensure the patient's informed decision making. Patients who meet clinical criteria for a syndrome as well as those with identified pathogenic germline mutations should receive appropriate surveillance measures in order to minimize their overall risk of developing syndrome-specific cancers. This guideline specifically discusses genetic testing and management of Lynch syndrome, familial adenomatous polyposis (FAP), attenuated familial adenomatous polyposis (AFAP), MUTYH-associated polyposis (MAP), Peutz-Jeghers syndrome, juvenile polyposis syndrome, Cowden syndrome, serrated (hyperplastic) polyposis syndrome, hereditary pancreatic cancer, and hereditary gastric cancer.
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            Guidelines for the clinical management of familial adenomatous polyposis (FAP).

            Familial adenomatous polyposis (FAP) is a well-described inherited syndrome, which is responsible for <1% of all colorectal cancer (CRC) cases. The syndrome is characterised by the development of hundreds to thousands of adenomas in the colorectum. Almost all patients will develop CRC if they are not identified and treated at an early stage. The syndrome is inherited as an autosomal dominant trait and caused by mutations in the APC gene. Recently, a second gene has been identified that also gives rise to colonic adenomatous polyposis, although the phenotype is less severe than typical FAP. The gene is the MUTYH gene and the inheritance is autosomal recessive. In April 2006 and February 2007, a workshop was organised in Mallorca by European experts on hereditary gastrointestinal cancer aiming to establish guidelines for the clinical management of FAP and to initiate collaborative studies. Thirty-one experts from nine European countries participated in these workshops. Prior to the meeting, various participants examined the most important management issues according to the latest publications. A systematic literature search using Pubmed and reference lists of retrieved articles, and manual searches of relevant articles, was performed. During the workshop, all recommendations were discussed in detail. Because most of the studies that form the basis for the recommendations were descriptive and/or retrospective in nature, many of them were based on expert opinion. The guidelines described herein may be helpful in the appropriate management of FAP families. In order to improve the care of these families further, prospective controlled studies should be undertaken.
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              Duodenal cancer in patients with familial adenomatous polyposis (FAP): results of a 10 year prospective study.

              Duodenal cancer is one of the leading causes of death in familial adenomatous polyposis (FAP) patients. An endoscopic surveillance programme was therefore initiated in 1988, the outcome of which is described in this paper. We report the 10 year follow up of 114 patients with FAP who were prospectively screened for the presence and severity of duodenal adenomas. Six of 114 patients (median age 67 years) developed duodenal adenocarcinoma. Four of these were from 11 patients who originally had Spigelman stage IV disease (advanced duodenal polyposis), which gives a 36% risk within this group of developing cancer. One case of duodenal cancer arose from 41 patients who originally had stage III disease (2%) and one cancer arose from 44 patients with original stage II disease (2%). All six patients have died: five were inoperable and one had recurrence three years after a pancreaticoduodenectomy. There was no association between duodenal cancer and site of germline mutation of the APC gene. Surveillance for duodenal adenocarcinoma and subsequent early referral for curative surgery has not been effective. Selection of patients with advanced but benign (Spigelman stage IV) duodenal polyposis for prophylactic pancreaticoduodenectomy should therefore be considered and can now be justified on the basis of these results. More comprehensive endoscopic surveillance of high risk (stage III and IV) patients is needed in an attempt to avoid underestimating the severity of duodenal polyposis, and to evaluate the role of endoscopic therapy in preventing advanced disease.
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                Author and article information

                Contributors
                flaminia.purchiaroni@hotmail.it
                tnakajim@ncc.go.jp
                +81-3-3542-2511 , tasakamo@ncc.go.jp
                seabe@ncc.go.jp
                ytsaito@ncc.go.jp
                Journal
                BMC Gastroenterol
                BMC Gastroenterol
                BMC Gastroenterology
                BioMed Central (London )
                1471-230X
                8 December 2017
                8 December 2017
                2017
                : 17
                Affiliations
                [1 ]GRID grid.416510.7, Wolfson Unit for Endoscopy, St Mark’s Hospital, ; London, UK
                [2 ]ISNI 0000 0001 2168 5385, GRID grid.272242.3, Endoscopy Division, National Cancer Center Hospital, ; 5-1-1 Tsukiji, Chuo-ku, Tokyo, 104-0045 Japan
                [3 ]ISNI 0000 0001 2168 5385, GRID grid.272242.3, Department of Genetic Medicine and Services, National Cancer Center Hospital, ; Tokyo, Japan
                Article
                718
                10.1186/s12876-017-0718-2
                5721481
                29216840
                © The Author(s). 2017

                Open AccessThis article is distributed under the terms of the Creative Commons Attribution 4.0 International License ( http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

                Categories
                Case Report
                Custom metadata
                © The Author(s) 2017

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