Orphanet: Atresia of small intestine

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Atresia of small intestine

Disease definition

A special form of intestinal atresia with absence of mesentery, which is most likely due to an intrauterine intestinal vascular accident. Newborns are usually preterm infants with low birth-weights, that encounter feeding difficulties (including vomiting with initial feeds, which may later worsened and the abdomen becomes progressively distended) as well as failure to thrive. Affected children present disrupted bowel loops assuming a spiral configuration resembling an 'apple peel' and may have less than half of the normal length of the small bowel and a physiologically short bowel. This disorder is characterized by jejunal atresia near the ligament of Treitz, foreshortened bowel, and a large mesenteric gap. The bowel distal to the atresia is precariously supplied. It may be a manifestation of cystic fibrosis and the most important cause of mortality is short bowel syndrome, encountered in 65% of cases.


Classification level: Disorder
  • Synonym(s):
    • Apple peel syndrome
    • Intestinal atresia type IIIb
    • Jejunal atresia
    • Jejunoileal atresia
    • Small intestinal atresia
  • Prevalence: 1-5 / 10 000
  • Inheritance: Autosomal recessive or Multigenic/multifactorial or Not applicable 
  • Age of onset: Neonatal
  • ICD-10: Q41.0  Q41.1  Q41.2  Q41.8  Q41.9
  • OMIM: 243600
  • UMLS: C0021828  C0266172  C0266175
  • MeSH: C538260
  • GARD: 140
  • MedDRA: 10010626

Detailed information


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