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Apple peel small bowel. A review of twelve cases
Insights
This review examines 12 cases of apple peel small bowel syndrome, a rare malformation involving intestinal atresia and agenesis of the dorsal mesentery. Findings discuss associated anomalies, surgical outcomes, and causes of mortality in affected infants.
Area of Science:
- Pediatric Surgery
- Gastrointestinal Malformations
- Developmental Biology
Background:
- Apple peel small bowel syndrome is a rare congenital anomaly characterized by a unique long, atretic segment of small intestine resembling an apple peel.
- It is associated with agenesis of the dorsal mesentery, leading to malrotation and potential complications.
Purpose of the Study:
- To review cases of apple peel small bowel syndrome treated at a single institution over a decade.
- To analyze associated anomalies, surgical interventions, and outcomes.
- To discuss the etiology and pathogenesis of this rare malformation.
Main Methods:
- Retrospective review of 12 pediatric cases diagnosed with apple peel small bowel syndrome.
- Analysis of patient records including clinical presentation, associated anomalies, surgical procedures, and mortality data.
- Review of etiological and pathogenetic factors based on available literature and case details.
Main Results:
- The study included 12 patients with apple peel small bowel syndrome, predominantly jejunal atresia (11 cases) and one duodenal atresia.
- Associated anomalies and surgical treatments were documented for each case.
- Causes of death and contributing factors were identified and analyzed.
Conclusions:
- Apple peel small bowel syndrome presents significant surgical challenges and carries a considerable risk of mortality.
- Understanding associated anomalies and pathogenetic mechanisms is crucial for improving patient management and outcomes.
- Further research into the etiology of this rare intestinal malformation is warranted.
Abstract:
This is a review of 12 cases with apple peel small bowel with agenesis of the dorsal mesentery and intestinal atresia, 11 of them jejunal and 1 duodenal in type who were treated at the Clinica Infantil "La Paz" Madrid over a period of ten years (1969--1979). The associated anomalies, the surgical treatment and the cause of death as well as the etiology and pathogenesis of this malformation are discussed.