Congenital duodenal atresia is a common intestinal anomaly that occurs secondary to either inadequate endodermal proliferation or failure of the epithelial solid cord to recanalize. It occurs in 1 in 2500-5000 live births with 25-40% of the cases encountered in patient with trisomy 21. Definitive intervention is to correct anomaly by duodenoduodenostomy in the newborn period [eMedicine]
Duodenal atresia is typically characterized by onset of vomiting within hours of birth. While vomitus is most often bilious, it may be nonbilious because 15% of defects occur proximal to the ampulla of Vater [eMedicine].
Visualization of a fluid-filled double bubble on prenatal ultrasound scan is associated with duodenal obstruction secondary to intrinsic or extrinsic cause although it is known to have a false-positive rate. It may represent a transient finding in an otherwise healthy fetus. However it should alert the fetal medicine specialist to look for other ultrasonographic features of anomalies associated with duodenal obstruction, such as cardiac anomalies, VACTERL (vertebral, anorectal, tracheo-oesophageal, renal, limb) association, and chromosome anomalies, e.g. Trisomy 21 [M. S. Choudhry]
Proximal gut atresia is associated with polyhydramnios. However, precise incidence is unclear. Suggested aetiology is possibly due to it development in first trimester. [R.M. Kimble]
Immediate repair is recommended but contraindicated in cases of electrolyte or fluid balance disturbance, severe cardiac defect and severe respiratory insufficiency. While waiting for the surgery, infants can be maintained on OG suction and IV nutrition with aggressive repletion of fluid and electrolytes loss.
In a study done by M. S. Choudhry, Overall neonatal survival rate with duodenal atresia was 96 %
Reference:
1. Frederick Merrill Karrer, D Dean Potter & Casey M Calkins, "Duodenal Atresia", eMedicine, Mar 3, 2009.
http://emedicine.medscape.com/article/932917-overview
2. M. S. Choudhry, N. Rahman,P. Boyd et al, "Duodenal atresia: associated anomalies, prenatal diagnosis and outcome", Pediatr Surg Int (2009) 25:727–730, Springer-Verlag 2009
3. R. M. Kimble, J. E. Harding & A. Kolbe, "Does gut atresia cause polyhydramnios?", Pediatr Surg Int (1998) 13: 115-117, Springer-Verlag 1998
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