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Duodenal atresia and stenosis

Last updated: August 31, 2021

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Duodenal atresia is a complete occlusion or absence of the duodenal lumen, whereas duodenal stenosis refers to an incomplete obstruction caused by narrowing. Both conditions present with signs of obstruction, including a distended upper abdomen, delayed meconium passage, and vomiting. If the defect is located proximal to the major duodenal papilla, nonbilious vomiting occurs, whereas a defect occurring distal to the papilla causes bilious vomiting. Evidence of polyhydramnios on prenatal ultrasonography may lead to an early diagnosis of duodenal obstruction. An abdominal x-ray typically shows the double bubble sign. Duodenal atresia and stenosis generally have a favorable prognosis and may be surgically treated through duodenoduodenostomy.

Epidemiological data refers to the US, unless otherwise specified.

Duodenal atresia occurs when recanalization of the closed duodenum fails to occur or occurs only partially during the embryonic period (usually between the 8th–10th week of gestation). Because the development of the duodenum is connected to the growth of the pancreas and the hepatobiliary system, duodenal atresia is commonly associated with anomalies of these organs as well. [1]





Jejunal atresia and Ileal atresia

Other differential diagnoses

The differential diagnoses listed here are not exhaustive.

  • Preoperative management
  • Surgery: bypass the atresia or stenosis
    • The exact procedure depends on the anatomic findings and associated anomalies
    • Common procedure: duodenoduodenostomy or duodenojejunostomy with a proximal transverse-to-distal longitudinal (diamond-shaped) anastomosis
  • If diagnosis and treatment occur quickly, the survival rate of isolated duodenal atresia (without further anomalies) is 95%.
  • The presence of other anomalies worsens the prognosis.


  1. Kimura K, Loening-Baucke V. Bilious Vomiting in the Newborn: Rapid Diagnosis of Intestinal Obstruction. Am Fam Physician. 2000; 61 (9): p.2791-2798.
  2. Choudhry MS, Rahman N, Boyd P, Lakhoo K. Duodenal atresia: associated anomalies, prenatal diagnosis and outcome.. Pediatr Surg Int. 2009 . doi: 10.1007/s00383-009-2406-y . | Open in Read by QxMD
  3. Editors: Coppola CP, Kennedy Jr. AP, Scorpio RJ. Pediatric Surgery: Diagnosis and Treatment. Springer International Publishing ; 2014
  4. Brantberg A, Blaas H-G K, Salvesen KA, Haugen SE, Mollerlokken G, Eik-Nes SH. Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound Obstet Gynecol. 2002; 20 : p.439-446. doi: 10.1046/j.1469-0705.2002.00831.x . | Open in Read by QxMD