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Enteric duplications and mesenteric, omental, and retroperitoneal cysts are rare congenital anomalies. Most are recognized by the age of 2 years. Enteric duplications can occur anywhere in the gastrointestinal tract. Duplications and cysts can present in various fashions from being asymptomatic and an incidental finding causing obstruction, bleeding, or inflammation. Surgery is indicated in all instances to prevent or treat these complications. The surgical management can vary significantly depending on the location as well as the degree of involvement of adjacent structure, in particular the intestine.

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Epidemiology

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Enteric duplications occur sporadically without any apparent genetic predisposition. The reported incidence of enteric duplications is 0.02%.1 Enteric duplications are commonly associated with vertebral anomalies such as bifid, fused, or missing vertebrae.2 When associated with vertebral anomalies, enteric duplications may communicate with the spinal canal and are termed neuroenteric cysts. Thoracic duplications are more common in the presence of esophageal anomalies, and duplications of the small intestine are commonly identified in association with jejunoileal atresia. Colonic duplications are frequently associated with genitourinary anomalies.3 Multiple duplications are present in 10% to 20% of cases.

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Pathophysiology

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Enteric duplications are defined by the following three characteristics: (1) the presence of smooth muscle coat, (2) an intimate association with the alimentary tract (often sharing a common wall), and (3) an inner lining of intestinal epithelium. The incidence of enteric duplications by anatomic location is shown in Table 401-1. While a duplication may be identified anywhere from the mouth to the anus, the most common location is in the distal ileum. Duplications can by cystic or tubular (eFig. 401.1). Some traverse the diaphragm from the chest into the abdomen. Ectopic mucosa, most commonly gastric, is identified in up to a third of duplication cysts. Less commonly, the duplication may communicate with adjacent bowel.4 The abnormal embryogenesis that leads to an intestinal duplication is controversial. Because of the frequent association with the spinal canal the split notochord syndrome was proposed to explain neuroenteric cysts (eFig. 401.2). Others have postulated duplications arise as a consequence of failed regression of embryonic diverticula or aberrant recanalization of the solid stage. Environmental stressors such as hypoxia, trauma, and vascular insufficiency have all been proposed as causative factors. Lastly, the development of colorectal duplications in association with urogenital duplications has been attributed to partial or abortive twinning.4

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Table Graphic Jump Location
Table 401-1 Incidence of Enteric Duplications by Anatomical Location
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eFigure 401.1.
Graphic Jump Location

Panel A,B and C show various configurations of the openings of tubular duplications and panel D shows a cystic duplication ...

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