ABSTRACT
Congenital segmental dilatation (SD) of the intestine is a rare lesion defined as limited bowel dilatation with a 3- to 4-fold increase in size with an abrupt transition between the normal and dilated bowel and no intrinsic or extrinsic barrier distal to the dilatation. The etiology of SD of the intestine remains unknown. Because the symptom is not specific and the definitive diagnosis is difficult, SD is usually diagnosed incidentally during surgery. The histopathological findings are the most important diagnostic criteria. The presence of ganglion cells that are normal in number and morphology is one of the criteria for the differential diagnosis. Microscopy can reveal some anomalies, essentially a hypertrophic muscular layer and a heterotopic mucosa that can include esophageal, gastric, or pancreatic tissue. The treatment of SD depends on the clinical condition of the patient, the presentation, the surgeon's experience in dealing with such malformations, and the association with other malformations.
