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: Case report  Israel

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

Challenging differential diagnosis, Management of emergency care, Rare disease, Rare coexistence of disease or pathology

Rachel Gefen ORCID logo1ABCDEF, Brigitte Helou1B, Noam Shussman1BE, Anna Elia ORCID logo2BCD, Liat Appelbaum3CD, Alon Pikarsky1AE, Jonathan Abraham Demma1ABCDEFG

Am J Case Rep In Press; DOI:   :: ID: 937677

Available online: , In Press, Corrected Proof

Publication in the "In-Press" formula aims at speeding up the public availability of the pending manuscript while waiting for the final publication. The assigned DOI number is active and citable. The availability of the article in the Medline, PubMed and PMC databases as well as Web of Science will be obtained after the final publication according to the journal schedule


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.
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.
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.

Keywords: Behcet Syndrome; Intussusception; Pneumatosis Cystoides Intestinalis


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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923