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Duodenal atresia and stenosis, congenital

DA: 274




Developmental anomaly information

Definition: Duodenal maldevelopment occurs secondary to either inadequate endodermal proliferation (gut elongation outpaces proliferation) or failure of the epithelial solid cord to recanalize (failure of vacuolization). Recanalization of the common duct frequently appears with 2 lumina and openings into the duodenum with 2 orifices. These 2 major canals create a narrow segment of the duodenum, and this narrow zone is the area of the duodenum that is most prone to incomplete recanalization and atresia formation. Duodenal stenosis is the narrowing or partial blockage of a portion of the duodenum.Duodenal atresia is the most common fetal atresia, occurring in 1 in 10,000 births and in 8% of fetuses with Down syndrome. It may be suspected by the presence of a "double bubble" due to a dilated fluid-filled stomach and proximal duodenum. Duodenal obstruction may be complete or incomplete. Duodenal atresia is an example of complete intrinsic obstruction. Duodenal stenosis is an example of an incomplete intrinsic abnormality; however, duodenal extrinsic stenosis can occur in association with malrotation or a preduodenal portal vein. Strictly speaking, annular pancreas does not cause an extrinsic duodenal obstruction because the duodenum within the collar of an annular pancreas is intrinsically narrowed. Duodenal atresia can take many forms, but proximal and distal intestinal segments always end blindly. The intestine on either side of the defect may be in apposition (type 1), separated by a fibrous cord (type 2), or gap (type 3). Regardless of atresia severity, the proximal intestinal segment is typically dilated and the distal segment empty; these are hallmarks of duodenal atresia. While obstruction may occur anywhere within the duodenum, it occurs most frequently in the vicinity of the ampulla of Vater. In approximately 40% of cases, the anomaly is encountered in an infant with trisomy 21 (Down syndrome).


Synonyms:

  • Congenital atresia and stenosis of the duodenum
  • D2


Developmental anomaly parent: (empty)

Topology: Human body > Abdomen > Digestive tube > Small intestine

System: Human body > Digestive system > Small intestine > Duodenum

Developmental anomaly category: Atresia

Case records MDB121, MDB243, MDB262, MDB422
Links
Observed in MDB cases
Observed in combinaisons (MDB)
Observed in malformative diseases (MDB)
  • () in case MDB
Associated genes in profile
Associated genes in malformative diseases
Associated chromosomal diseases
Associated signaling pathways
Associated developmental anomalies

Here is the list of developmental anomalies associated with this developmental anomaly, based on the cases' information :



Last modified: 2009-01-02 20:05:55 by farideh

Created: 2008-05-26 02:00:00 by farideh



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