Segmental dilatation of the intestine (SD) is a rare lesion defined as limited bowel dilatation with a three- to fourfold increase in size with an abrupt transition between the normal and dilated bowel and no intrinsic or extrinsic barrier distal to the dilatation. It was first described by Swenson and Rathauser in 1959, and over 100 cases have been reported since then. Several theories were proposed to explain this malformation; however, its cause remains unknown.
Most pediatric cases are discovered in neonatal periods, so the SD cases in neonates were frequently called congenital segmental dilatation (CSD). Neonates with CSD usually present with features of intestinal obstruction within days of birth. Older children present with anemia, hypoproteinemia, malabsorption, and gastrointestinal bleeding. Preoperative diagnosis is sometimes difficult because of the clinical polymorphism and the lack of specificity of radiological investigations. Patients with an unexplained obstructive intestinal pattern are occasionally found at surgical exploration. The usual finding on laparotomy is localized dilatation of an isolated, well-defined segment of bowel with apparently normal bowel proximal and distal to this segment.
The definitive treatment is resection of the dilated segment and anastomosis of the normal segments of intestine. Most patients have an uneventful course after surgical resection, and the prognosis is excellent. In most cases, histology of the resected segment is usually normal. However, some of the cases showed hypertrophied or very thin muscle layer in the involved segment in histopathological evaluation. The dislocation of the myenteric plexus and the ectopic pancreatic or gastric tissues are reported in dilated intestinal segment.
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