Fig. 1
Classification of duodenal atresia (Source: Christopher Coppola)
(i)
Type I: This type is most common, with a luminal web or membrane, consisting of mucosa and submucosa.
(ii)
Type II: Duodenum is reduced to a fibrous cord connecting the proximal and distal segments
(iii)
Type III: There is discontinuity of the duodenal segments with no intervening tissue.
2.
Epidemiology: intrinsic duodenal obstruction occurs in 1:7,000 live births. It represents 49 % of small bowel atresia.
(a)
There is a strong association with trisomy 21; if duodenal atresia is detected, a karyotype should be performed.
(b)
Other associated conditions are Hirschsprung’s disease, Meckel’s diverticulum, esophageal atresia, imperforate anus, congenital heart disease, renal abnormalities and neurologic disorders.
3.
Clinical features:
(a)
One third to one half of fetuses with duodenal atresia will have polyhydramnios from inability to pass amniotic fluid through the gastrointestinal tract.
(b)
After birth, newborns with duodenal atresia experience feeding intolerance, distention, discomfort, and vomiting, which is usually bilious.
(c)
Children may pass meconium and have a normal rectal exam.
(d)
Features of trisomy 21 will frequently be present.
4.
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Assessment:
(a)
Abdominal x-ray will reveal a double bubble sign formed by distention of the stomach proximal to the pylorus, and distention of the obstructed first and second portion of the duodenum, proximal to the duodenal atresia. Distal bowel gas will be present in cases of partial obstruction and rare cases of complete atresia when air can pass from proximal to distal via the papilla of Vater and the secondary papilla.
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