Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery

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Classification according to ICD-10
Q41.9 Congenital absence (s), atresia and stenosis of the small intestine, part unspecified
ICD-10 online (WHO version 2019)

The Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery is a very rare congenital disease with a combination of Duodenojejunale atresia and volvulus and partially removed mesentery .

The first description comes from the year 2002 by the Viennese children's surgeon W, Pumberger and colleagues.

So far, only a few people have been described in two families. The cause is not known. The normal preserved length of the small intestine distinguishes this syndrome from the apple peel syndrome .

Clinic, diagnosis and treatment correspond to those mentioned under intestinal atresia .

literature

  • DS Huff: Developmental Anatomy and Anomalies of the Gastrointestinal Tract with Involvement in Major Malformative Syndromes. In: P. Russo, E. Ruchelli, D. Piccoli (Eds.): Pathology of Pediatric Gastrointestinal and Liver Disease. 2014, ISBN 978-3-642-54052-3 , Online ISBN 978-3-642-54053-0
  • V. Martin, C. Shaw-Smith: Review of genetic factors in intestinal malrotation. In: Pediatric Surgery International. 26, 2010, p. 769, doi: 10.1007 / s00383-010-2622-5 .
  • JC Leonidas, RA Amoury et al. a .: Duodenojejunal Atresia with “Apple-Peel” Small Bowel. In: Radiology. 118, 1976, p. 661, doi: 10.1148 / 118.3.661 .

Individual evidence

  1. ↑ Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery.  In: Online Mendelian Inheritance in Man . (English)
  2. ^ W. Pumberger, R. Birnbacher, G. Pomberger, J. Deutinger: Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: a hereditary compound structure in duodenal atresia? In: American journal of medical genetics. Volume 109, Number 1, April 2002, pp. 52-55, doi: 10.1002 / ajmg.10309 , PMID 11932992 .