Duodenal atresia with apple-peel configuration of the ileum and absent superior mesenteric artery

BACKGROUND: Embryologically, duodenal atresia results from inadequate recanalisation and proliferation of gut epithelius in the 6th week of gestation, while apple-pee atresia of small bowel is a consequence of a vascular accident in subsequent embryonic development, and the two are rather rarely manifested as a joint clinical entity. CASE PRESENTATION: We present here a 29 week preterm boy admitted to the intensive care unit due to breathing difficulties and low birthweight. Following clinical, radiographic and ultrasound examination, he was diagnosed with duodenal obstruction and subjected to surgical treatment. The exploration of abdominal cavity verified duodenal atresia in the second portion with the absence of third and fourth portions of duodenum, superior mesenteric artery, as well as apple-peel atresia of jejunum. Resection of the apple-peel segment of jejunum was done and the continuity of digestive tube was established by the end-to-end duodeno-ileal anastomosis. CONSLUSION: This rare case of ours questions the embryology of duodenal atresia suggesting that a mesenteric vascular disruption phenomenon in subsequent embryonic life might be the aetiological factor.