| Summary: | Background: Though duodenal atresia is relatively common among intestinal atresias, its association with apple peel type of jejunoileal atresia and absent superior mesenteric artery (SMA) is very rare. This association contradicts the well-known embryo pathology of duodenal atresia and could be a management challenge.
Case presentation: A 4-day-old preterm male baby presented with bilious vomiting. The X-ray abdomen showed a double bubble sign. Laparotomy revealed atresia of the 3rd part of the duodenum. The SMA was absent and the remaining small gut was short, narrow, and twisted around a narrow vascular pedicle arising from the marginal artery. We resected the most distal part of the dilated duodenum and made a wide end to side anastomosis with distal chimney stoma (Bishop Koop enterostomy). The baby did well in the postoperative period.
Conclusion: The surgical management of this rare entity was technically challenging. Bishop Koop enterostomy is a safe option when the primary anastomosis is not possible.
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