duodenal atresia
MIM.223400
Image Gallery
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Types
duodenal mucosal atresia (duodenal membranous atresia) by mucosal diaphragm (web)
blind ends duodenal atresia
- blind ends separated by a fibrous cord
- blind ends separated by a V-shaped mesenteric defect
- blind ends with apple peel appearance
- multiple atresia (string of suasages appearance)
Etiology
familial duodenal atresia (MIM.223400)
syndromic duodenal atresia
- Down syndrome (MIM.190685)
- oculodigitoesophagoduodenal syndrome (MIM.164280)
- Fryns syndrome (MIM.229850)
- Townes-Brocks syndrome (TBS) (MIM.107480)
- Miller-Dieker lissencephaly syndrome (MIM.247200)
duodenal atresia with biliary atresia associated with facial, thyroid and auditory apparatus abnormalities (#16957470#)
Associations
complex intestinal malformations
- apple peel small bowel syndrome (MIM.243600)
- familial intestinal polyatresia syndrome (FIPA) (MIM.243150).
- duodenojejunal atresia associated with malrotation, volvulus, absent dorsal mesentery (absent parietal attachment of the mesentery) and absent superior mesenteric artery (MIM.606894)
proximal jejunal perforations (#14523829#)
Case records
Case 12697: Duodenal atresia with duodenal dilatation in multiple intestinal atresia syndrome (MIAS)
References
Galan-Gomez E, Sanchez EB, Arias-Castro S, Cardesa-Garcia JJ. Intrauterine growth retardation, duodenal and extrahepatic biliary atresia, hypoplastic pancreas and other intestinal anomalies: further evidence of the Martinez-Frias syndrome. Eur J Med Genet. 2007 Mar-Apr;50(2):144-8. PMID: #17321227#
Maegawa GH, Chitayat D, Blaser S, Whyte H, Thomas M, Kim P, Kim J, Taylor G, McNamara PJ. Duodenal and biliary atresia associated with facial, thyroid and auditory apparatus abnormalities: a new mandibulofacial dysostosis syndrome? Clin Dysmorphol. 2006 Oct;15(4):191-6. PMID: #16957470#
Holder-Espinasse M, Ahmad Z, Hamill J, Pahari A, Misra D, Drake D, Winter RM, Wilson LC. Familial syndromic duodenal atresia: Feingold syndrome. Eur J Pediatr Surg. 2004 Apr;14(2):112-6. PMID: #15185158#
Keegan CE, Vilain E, Mohammed M, Lehoczky J, Dobyns WB, Archer SM, Innis JW. Microcephaly, jejunal atresia, aberrant right bronchus, ocular anomalies, and XY sex reversal. Am J Med Genet A. 2004 Mar 15;125(3):293-8. PMID: #14994240#
Sajja SB, Middlesworth W, Niazi M, Schein M, Gerst PH. Duodenal atresia associated with proximal jejunal perforations: a case report and review of the literature. J Pediatr Surg. 2003 Sep;38(9):1396-8. PMID: #14523829#