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      Pneumatosis Cystoides Coli Presenting as Acute Abdomen in a Patient with Complicated Behcet’s Disease: A Case Report

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          Abstract

          Patient: Male, 40-year-old

          Final Diagnosis: Pneumatosis cystoides coli

          Symptoms: Abdominal pain • acute abdomen • anorexia

          Medication: —

          Clinical Procedure: Exploratory laparotomy • ileostomy placement • right hemicolectomy

          Specialty: Gastroenterology and Hepatology • Rheumatology • Surgery

          Objective:

          Rare disease

          Background:

          Behcet’s disease (BD) is defined as vasculitis involving arteries and veins of any size and affecting almost any organ system. Abdominal manifestations of BD are diverse and nonspecific. Mucosal ulcerations can be seen in the gastrointestinal tract. Extensive ulcerations, especially ileocecal lesions, can lead to perforation, strictures, fistulas, and abscesses. Pneumatosis cystoides intestinale is a rare benign condition characterized by multiple submucosal or subserosal, gas-filled cysts in the gastrointestinal tract wall. Pneumatosis cystoides coli (PCC) affects the colon, can present with a wide range of manifestations, and can mimic many different systemic diseases. We describe a case of PCC in a patient with Behcet’s disease who presented to the Emergency Department with a clinical suspicion of acute abdomen.

          Case Report:

          A 40-year-old man with complicated Behcet’s disease, treated with high-dose steroids, presented with acute abdomen and CT scan findings highly suggestive of intestinal obstruction due to ileocolic intussusception. He underwent laparoscopic right hemicolectomy. Pathology demonstrated PCC disease.

          Conclusions:

          Pneumatosis cystoides coli can present with a broad range of symptoms and can be secondary to many systemic and autoimmune diseases. With radiological evidence and a high level of suspicion, unnecessary surgery can be prevented.

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          Most cited references9

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          Gastrointestinal Behçet's disease: a review.

          Behçet's disease (BD) is an idiopathic, chronic, relapsing, multi-systemic vasculitis characterized by recurrent oral and genital aphthous ulcers, ocular disease and skin lesions. Prevalence of BD is highest in countries along the ancient silk road from the Mediterranean basin to East Asia. By comparison, the prevalence in North American and Northern European countries is low. Gastrointestinal manifestations of Behçet's disease are of particular importance as they are associated with significant morbidity and mortality. Although ileocecal involvement is most commonly described, BD may involve any segment of the intestinal tract as well as the various organs within the gastrointestinal system. Diagnosis is based on clinical criteria - there are no pathognomonic laboratory tests. Methods for monitoring disease activity on therapy are available but imperfect. Evidence-based treatment strategies are lacking. Different classes of medications have been successfully used for the treatment of intestinal BD which include 5-aminosalicylic acid, corticosteroids, immunomodulators, and anti-tumor necrosis factor alpha monoclonal antibody therapy. Like inflammatory bowel disease, surgery is reserved for those who are resistant to medical therapy. A subset of patients have a poor disease course. Accurate methods to detect these patients and the optimal strategy for their treatment are not known at this time.
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            Adult intussusception: a systematic review and meta-analysis

            Perhaps partly because intussusception in adults is rare, optimal treatment remains controversial. The aim of this study was to determine the appropriate surgical procedure for adult intussusception.
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              Pneumatosis cystoides intestinalis: a case report and literature review

              Background Pneumatosis cystoides intestinalis (PCI) is a low-incidence disease that confuses many doctors. A vast number of factors are suspected to contribute to its pathogenesis, such as Crohn’s disease, intestinal stenosis, ulcerative colitis, drug use, extra-gastrointestinal diseases, and chronic obstructive pulmonary disease. Most consider its pathogenesis interrelated to an increase in intra-intestinal pressure and the accumulation of gas produced by aerogenic bacteria, and patients with atypical symptoms and imaging manifestations tend to be misdiagnosed. Case presentation A 64-year-old man complained of a 3-month history of bloody stool without mucopurulent discharge, abdominal pain, or diarrhea. Colonoscopy revealed multiple nodular projections into the segmental mucosa of the sigmoid colon. Crohn’s disease and malignant disease ware suspected first according to the patient’s history, but laboratory examinations did not confirm either. Endoscopic ultrasound (EUS) revealed multiple cystic lesions in the submucosa. Moreover, computer tomography scan showed multiple bubble-like cysts. Combined with ultrasonography, computed tomography, and pathology findings, we ultimately made a diagnosis of PCI. Instead of surgery, we recommended conservative treatment consisting of endoscopy and oral drug administration. His symptoms improved with drug therapy after discharge, and no recurrence was noted on follow-up. Conclusions The incidence of PCI is low. Due to a lack of specificity in clinical manifestations and endoscopic findings, it often misdiagnosed as intestinal polyps, tumors, inflammatory bowel disease, or other conditions. Colonoscopy, computed tomography, and ultrasonography have demonstrated benefit in patients with multiple nodular projections in colon. Compared to the treatment of the above diseases, PCI treatment is effective and convenient, and the prognosis is optimistic. Therefore, clinicians should increase their awareness of PCI to avoid unnecessary misdiagnosis.
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                Author and article information

                Journal
                Am J Case Rep
                Am J Case Rep
                amjcaserep
                The American Journal of Case Reports
                International Scientific Literature, Inc.
                1941-5923
                2022
                29 October 2022
                : 23
                : e937677-1-e937677-4
                Affiliations
                [1 ]Department of General Surgery and Traumatology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
                [2 ]Department of Pathology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
                [3 ]Department of Radiology, Hadassah Medical Organization and Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem, Israel
                Author notes
                Corresponding Author: Jonathan Abraham Demma, e-mail: yonatand@ 123456hadassah.org.il

                Authors’ Contribution:

                [A]

                Study Design

                [B]

                Data Collection

                [C]

                Statistical Analysis

                [D]

                Data Interpretation

                [E]

                Manuscript Preparation

                [F]

                Literature Search

                [G]

                Funds Collection

                Financial support: None declared

                Conflict of interest: None declared

                Author information
                https://orcid.org/0000-0002-8501-131X
                https://orcid.org/0000-0002-0755-2065
                Article
                937677
                10.12659/AJCR.937677
                9627685
                36307965
                40f540b3-0e23-4e61-b156-d64607587efc
                © Am J Case Rep, 2022

                This work is licensed under Creative Common Attribution-NonCommercial-NoDerivatives 4.0 International ( CC BY-NC-ND 4.0)

                History
                : 30 June 2022
                : 16 August 2022
                : 21 September 2022
                Categories
                Articles

                behcet syndrome,intussusception,pneumatosis cystoides intestinalis

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