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World J Gastroenterol. Aug 7, 2014; 20(29): 9990-9997
Published online Aug 7, 2014. doi: 10.3748/wjg.v20.i29.9990
Spontaneous free perforation of the small intestine in adults
Hugh James Freeman
Hugh James Freeman, Department of Medicine, University of British Columbia, Vancouver V6T 1W5, Canada
Author contributions: Freeman HJ contributed to this paper.
Correspondence to: Hugh James Freeman, MD, CM, FRCPC, FACP, Professor, Department of Medicine, University of British Columbia, 2211 Wesbrook Mall, Vancouver V6T 1W5, Canada.
Telephone: +1-604-8227216 Fax: +1-604-8227236
Received: January 20, 2014
Revised: March 21, 2014
Accepted: April 8, 2014
Published online: August 7, 2014

Spontaneous free perforation of the small intestine is uncommon, especially if there is no prior history of visceral trauma. However, free, even recurrent, perforation may complicate a defined and established clinical disorder, such as Crohn’s disease. In addition, free perforation may be the initial clinical presentation of an occult intestinal disorder, such as a lymphoma complicating celiac disease, causing diffuse peritonitis and an acute abdomen. Initial diagnosis of the precise cause may be difficult, but now has been aided by computerized tomographic imaging. The site of perforation may be helpful in defining a cause (e.g., ileal perforation in Crohn’s disease, jejunal perforation in celiac disease, complicated by lymphoma or collagenous sprue). Urgent surgical intervention, however, is usually required for precise diagnosis and treatment. During evaluation, an expanding list of other possible causes should be considered, even after surgery, as subsequent management may be affected. Free perforation may not only complicate an established intestinal disorder, but also a new acute process (e.g., caused by different infectious agents) or a longstanding and unrecognized disorder (e.g., congenital, metabolic and vascular causes). Moreover, new endoscopic therapeutic and medical therapies, including use of emerging novel biological agents, have been complicated by intestinal perforation. Recent studies also support the hypothesis that perforation of the small intestine may be genetically-based with different mutations causing altered connective tissue structure, synthesis and repair.

Keywords: Free small bowel perforation, Crohn’s disease, Non-traumatic perforation, Celiac disease, Small bowel lymphoma, Vasculitis, Collagenous sprue, Biological agents, Monoclonal antibodies

Core tip: Clinical presentation with “an acute abdomen” due to spontaneous “free” perforation usually requires urgent surgical intervention for survival. Often, the clinician is aware of an underlying disorder, but in others, this emergent situation may represent the initial clinical presentation of unrecognized Crohn’s disease or, even celiac disease already complicated by a superimposed lymphoma. Other rare causes include medical treatments for a variety of immune-mediated, inflammatory and neoplastic disorders, including some novel biological agents. Evidence also suggests that intestinal perforation could also reflect an occult genetically-based defect causing impaired connective tissue structure, synthesis and repair.