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Down's syndrome, duodenal atresia with paradoxical presence of distal bowel gas

Journal of Perinatology volume 31, pages 223–224 (2011)Cite this article

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The upper GI demonstrates duodenal atresia with so-called paradoxical presence of distal bowel gas. The gas is able to pass through the major and minor duodenal papillae, which straddle the area of atresia. The patient underwent duodenoduodenostomy for duodenal atresia on the fourth day of life and subsequent repair of AV canal was done as well.

Paradoxical presence of gas in the distal bowel with duodenal atresia appears to have been first recognized in 1972.1 It must be differentiated from cases of partial duodenal obstruction with the presence of distal gas, such as duodenal stenosis, incomplete duodenal diaphragm, Ladd's bands, annular pancreas or pre-duodenal portal vein. Duodenal atresia is not an uncommon congenital anomaly and occurs in 1 in 10 000 births.2 In fetal life, this anomaly is associated with polyhydramnios. Duodenal atresia, unlike jejunal and ileal atresia, is a developmental anomaly due to failure to recanalize the duodenum after its solid phase. The solid phase of the duodenum occurs because of proliferation of the endodermal epithelium. Subsequent recanalization is by apoptosis. Duodenal atresia is often associated with other anomalies and has a slight male predominance (1.4:1).2 Associated anomalies are Down's syndrome, seen in 46% of patients with duodenal atresia. Other anomalies include the VACTERL association (vertebral, anus, cardiac, tracheal esophageal and limb anomalies), occurring in 5 to 15% of cases, Feingold's syndrome (various combinations of microcephaly, limb malformations, esophageal and duodenal atresia) and alveolar capillary dysplasia.3, 4

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References

  1. Kassner EG, Sutton AL, deGroot TJ . Bile duct anomalies associated with duodenal atresia; paradoxical presence of small bowel gas. Am J Roentgenol 1972; 116: 577–583.

    Article  CAS  Google Scholar 

  2. Choudhry MS, Rahman N, Boyd P, Lakhoo K . Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int 2009; 25: 727–730.

    Article  CAS  Google Scholar 

  3. Chopra M, James C, Adams S, Mowat D . Duodenal atresia: consider Feingold syndrome. J Pediatr Child Health 2010; 46: 136–137.

    Article  Google Scholar 

  4. Gutierrez C, Rodriguez A, Palenzuela S, Forteza C, Diaz-Rossello JL . Congenital misalignment of pulmonary veins with alveolar capillary dysplasia causing persistent neonatal pulmonary hypertension: report of 2 affected siblings. Pediatr Dev Pathol 2000; 3: 271–276.

    Article  CAS  Google Scholar 

  5. Bang S, Suh JH, Park BR, Park SW, Song SY . The relationship of anatomic variation of pancreatic duct and pancreaticobiliary diseases. Yonsei Med J 2006; 2: 243–248.

    Article  Google Scholar 

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  1. Mallinckrodt Institute of Radiology, Washington University School of Medicine, 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. Down's syndrome, duodenal atresia with paradoxical presence of distal bowel gas. J Perinatol 31, 223–224 (2011). https://doi.org/10.1038/jp.2010.151

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