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The colon is an unusual site of intestinal atresia. Colonic atresia is subdivided into three phenotypes. Type III is the most common phenotype, where the proximal and distal blind sacs are not connected. Here, we report on the presence of colonic atresia with an “inverse apple-peel” appearance in a neonate with gastroschisis. The lack of mesenteric fixation of the entire small intestine, including the proximal colon, and the twisting around the vascular axis of the superior mesenteric artery led to intrauterine volvulus and hemorrhagic infarction of the ileocolic bowel at 34 weeks of gestation. According to the current nomenclature for small bowel atresia, we introduce type IIIB into the current colonic atresia classification. The occurrence of type IIIB has been mentioned in the literature, but no single cases have been reported until now. Patients with this type of atresia are predisposed to the loss of the ileocecal region.

“Inverse Type” Apple-Peel Syndrome Is Associated with Type III Colonic Atresia in a Neonate with Gastroschisis—A “New” Subtype of Colonic Atresia