Apple-peel intestinal atresia

Last revised by Kyriacos S. Evangelou on 29 Jul 2021

Apple-peel intestinal atresia, also known as type IIIb or Christmas tree intestinal atresia, is a rare form of small bowel atresia in which the duodenum or proximal jejunum ends in a blind pouch and the distal small bowel wraps around its vascular supply in a spiral resembling an apple peel. Often a vast segment of small bowel is absent.

While apple-peel intestinal atresia is usually reported as an isolated malformation, it has been associated with malrotation. Additionally, syndromic association has been reported with microcephaly and ocular anomalies.

There is evidence that NOTCH1-gene mutations may contribute to the disease's prevalance, unveiling the possibility of a partially hereditary pattern of pathology 7.

See also

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