Abstract
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.
Highlights
Duodenal atresia is common among gastrointestinal atresias.[1]
We describe an extremely rare association where duodenal atresia was associated with the absence of superior mesenteric artery and apple peel appearance of the small gut in a preterm baby
Seven cases have been reported so far. This rare presentation reflects a vascular accident in the embryology of duodenal atresia and at the same time poses a tough challenge during the operative correction.[4,5]
Summary
Duodenal atresia is common among gastrointestinal atresias.[1]. Failure of the recanalization during embryonic development is thought to be the cause that is different from the ‘vascular accident’ theory of atresia in other parts of the intestine.[1,2] In the most common form of duodenal atresia (more than 90%), the obstruction is caused by a mucosal web. Kimura’s duodenoduodenostomy is the most preferred surgical procedure in duodenal atresia.[3] We describe an extremely rare association where duodenal atresia was associated with the absence of superior mesenteric artery and apple peel appearance of the small gut in a preterm baby. This rare presentation reflects a vascular accident in the embryology of duodenal atresia and at the same time poses a tough challenge during the operative correction.[4,5]
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