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Apple peel small bowel

Journal of Perinatology volume 28, pages 380–382 (2008)Cite this article

The upper gastrointestinal series demonstrated atresia of the proximal bowel at the duodenojejunal junction. The enema demonstrated a malrotated microcolon in the anterior, lower mid abdomen. The presence of a microcolon with a proximal duodenal or jejunal atresia suggested at least a second more distal atresia or a more complex malformation. Attempts to reflux contrast into the terminal ileum beyond the ileocecal valve were unsuccessful. The patient was then taken to the operating room where a laparotomy disclosed a jejunal atresia just beyond the duodenojejunal junction and the absence of the dorsal mesentery except at the terminal ileum as it inserted into the cecum. The short segment of distal bowel was ‘twisted’ or spiraled just proximal to the cecum. Only 6 cm of distal small bowel were present. The terminal ileum was obstructed by a web-preventing passage of a catheter. This web was incised at enterotomy and the bowel was reanastomosed.

Apple peel small bowel (APSB) is a disorder characterized by proximal jejunal or distal duodenal atresia, agenesis of the dorsal mesentery and variable parts of the distal small bowel, agenesis of the distal superior mesenteric artery and a malrotated microcolon.1 The syndrome was named, however, after the appearance of the small bowel distal to the obstruction, which spirals in the abdomen like a continuous segment of cut apple peel. This spiral or helical appearance of the distal bowel can be seen on preoperative contrast enema if contrast can be refluxed into the ileum.2 APSB is a rare disease, constituting only 10 to 15% of all small bowel atresias.3, 4 APSB has occurred in families and is believed in some cases to be an autosomal recessive condition.3 It also occurs in association with other malformations including microcephaly and corneal leukoma,5 polysplenia,4 limb defects, situs inversus and immune deficiency.6 The prognosis of patients with APSB syndrome is good but there is a high risk of recurrent obstruction, usually at the anastomotic site and most often occurring within the first year of life.1

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  1. Department of Radiology, Mallinckrodt Institute of Radiology, Washington University School of Medicine, St Louis Children's Hospital, St Louis, MO, USA

    T E Herman & M J Siegel

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  1. T E Herman
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  2. M J Siegel
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Correspondence to T E Herman.

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Herman, T., Siegel, M. Apple peel small bowel. J Perinatol 28, 380–382 (2008). https://doi.org/10.1038/sj.jp.7211922

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