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Duodenal Atresia or Stenosis
Steven J. Kraus, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Duodenal atresia (DA), duodenal stenosis (DS)
            • Definitions

              • Atresia: Congenital occlusion of intestinal lumen
              • Stenosis: Fixed narrowing of intestinal lumen
              • Most common neonatal upper intestinal obstruction (40-50%)

            IMAGING

            • General Features

              • Radiographic Findings

                • Fluoroscopic Findings

                  • Ultrasonographic Findings

                    • MR Findings

                      • Imaging Recommendations

                        DIFFERENTIAL DIAGNOSIS

                          PATHOLOGY

                          • General Features

                            • Staging, Grading, & Classification

                              CLINICAL ISSUES

                              • Presentation

                                • Demographics

                                  • Natural History & Prognosis

                                    • Treatment

                                      DIAGNOSTIC CHECKLIST

                                      • Consider

                                        • Image Interpretation Pearls

                                          Selected References

                                          1. Nguyen HN et al: Untwisting the complexity of midgut malrotation and volvulus ultrasound. Pediatr Radiol. 51(4):658-68, 2021
                                          2. Tsitsiou Y et al: Diagnostic decision-making tool for imaging term neonatal bowel obstruction. Clin Radiol. 76(3):163-71, 2021
                                          3. Bishop JC et al: The double bubble sign: duodenal atresia and associated genetic etiologies. Fetal Diagn Ther. 47(2):98-103, 2020
                                          4. Hameed S et al: The role of sonography in differentiating congenital intrinsic duodenal anomalies from midgut malrotation: emphasizing the new signs of duodenal and gastric wall thickening and hyperechogenicity. Pediatr Radiol. 50(5):673-83, 2020
                                          5. Chandrasekaran N et al: Prenatal sonographic diagnosis of meconium peritonitis from duodenal atresia. BMJ Case Rep. 2017, 2017
                                          6. Angotti R et al: Association of duodenal atresia, malrotation, and atrial septal defect in a Down-syndrome patient. APSP J Case Rep. 7(2):16, 2016
                                          7. Rattan KN et al: Neonatal duodenal obstruction: a 15-year experience. J Neonatal Surg. 5(2):13, 2016
                                          8. Chandrasekharam V: Laparoscopic duodenoduodenostomy in neonates: report of two cases and review of the literature. J Indian Assoc Pediatr Surg. 20(3):150-2, 2015
                                          9. Fragoso AC et al: Defective upper gastrointestinal function after repair of combined esophageal and duodenal atresia. J Pediatr Surg. 50(4):531-4, 2015
                                          10. Virgone C et al: Accuracy of prenatal ultrasound in detecting jejunal and ileal atresia: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 45(5):523-9, 2015
                                          11. Adewole VA et al: Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble. Springerplus. 3:368, 2014
                                          12. Latzman JM et al: Duodenal atresia: not always a double bubble. Pediatr Radiol. 44(8):1031-4, 2014
                                          13. Maxfield CM et al: A pattern-based approach to bowel obstruction in the newborn. Pediatr Radiol. 43(3):318-29, 2013
                                          14. Mirza B et al: Multiple associated anomalies in patients of duodenal atresia: a case series. J Neonatal Surg. 1(2):23, 2012
                                          15. Akcora B et al: A newborn with duodenal atresia and a gastric perforation. Afr J Paediatr Surg. 7(1):33-5, 2010
                                          16. Choudhry MS et al: Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int. 25(8):727-30, 2009
                                          17. Gilbertson-Dahdal DL et al: Neonatal malrotation with midgut volvulus mimicking duodenal atresia. AJR Am J Roentgenol. 192(5):1269-71, 2009
                                          18. Iwai A et al: Choledochal cyst associated with duodenal atresia: case report and review of the literature. Pediatr Surg Int. 25(11):995-8, 2009
                                          19. Keckler SJ et al: The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia. Pediatr Surg Int. 24(8):921-3, 2008
                                          20. Mustafawi AR et al: Congenital duodenal obstruction in children: a decade's experience. Eur J Pediatr Surg. 18(2):93-7, 2008
                                          21. Escobar MA et al: Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg. 39(6):867-71; discussion 867-71, 2004
                                          22. Forrester MB et al: Population-based study of small intestinal atresia and stenosis, Hawaii, 1986-2000. Public Health. 118(6):434-8, 2004
                                          23. Sugimoto T et al: Choledochal cyst and duodenal atresia: a rare combination of malformations. Pediatr Surg Int. 20(9):724-6, 2004
                                          24. Doray B et al: Esophageal and duodenal atresia in a girl with a 12q24.3-qter deletion. Clin Genet. 61(6):468-71, 2002
                                          25. Haeusler MC et al: Prenatal ultrasonographic detection of gastrointestinal obstruction: results from 18 European congenital anomaly registries. Prenat Diagn. 22(7):616-23, 2002
                                          26. Mordehai J et al: Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: a case report. J Pediatr Surg. 37(4):E5, 2002
                                          27. Pumberger W et al: Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: a hereditary compound structure in duodenal atresia? Am J Med Genet. 109(1):52-5, 2002
                                          28. Rothenberg SS: Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and children. J Pediatr Surg. 37(7):1088-9, 2002
                                          29. Sencan A et al: Symptomatic annular pancreas in newborns. Med Sci Monit. 8(6):CR434-7, 2002
                                          30. Maruyama K et al: Partial monosomy 10q with partial trisomy 11q due to paternal balanced translocation. J Paediatr Child Health. 37(2):198-200, 2001
                                          31. Dalla Vecchia LK et al: Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 133(5):490-6; discussion 496-7, 1998
                                          32. Grosfeld JL: Jejunoileal atresia & stenosis. In: O'neal JA, Rowe MI, Grosfeld JL, eds. Pediatric Surgery, 3rd ed. St Louis: Mosby. 1145-58, 1998
                                          33. Buonomo C: Neonatal gastrointestinal emergencies. Radiol Clin North Am. 35(4):845-64, 1997
                                          34. Courtens W et al: Feingold syndrome: report of a new family and review. Am J Med Genet. 73(1):55-60, 1997
                                          35. Lemire EG et al: A familial disorder with duodenal atresia and tetralogy of Fallot. Am J Med Genet. 66(1):39-44, 1996
                                          36. Long FR et al: Intestinal malrotation in children: tutorial on radiographic diagnosis in difficult cases. Radiology. 198(3):775-80, 1996
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Duodenal atresia (DA), duodenal stenosis (DS)
                                                    • Definitions

                                                      • Atresia: Congenital occlusion of intestinal lumen
                                                      • Stenosis: Fixed narrowing of intestinal lumen
                                                      • Most common neonatal upper intestinal obstruction (40-50%)

                                                    IMAGING

                                                    • General Features

                                                      • Radiographic Findings

                                                        • Fluoroscopic Findings

                                                          • Ultrasonographic Findings

                                                            • MR Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      CLINICAL ISSUES

                                                                      • Presentation

                                                                        • Demographics

                                                                          • Natural History & Prognosis

                                                                            • Treatment

                                                                              DIAGNOSTIC CHECKLIST

                                                                              • Consider

                                                                                • Image Interpretation Pearls

                                                                                  Selected References

                                                                                  1. Nguyen HN et al: Untwisting the complexity of midgut malrotation and volvulus ultrasound. Pediatr Radiol. 51(4):658-68, 2021
                                                                                  2. Tsitsiou Y et al: Diagnostic decision-making tool for imaging term neonatal bowel obstruction. Clin Radiol. 76(3):163-71, 2021
                                                                                  3. Bishop JC et al: The double bubble sign: duodenal atresia and associated genetic etiologies. Fetal Diagn Ther. 47(2):98-103, 2020
                                                                                  4. Hameed S et al: The role of sonography in differentiating congenital intrinsic duodenal anomalies from midgut malrotation: emphasizing the new signs of duodenal and gastric wall thickening and hyperechogenicity. Pediatr Radiol. 50(5):673-83, 2020
                                                                                  5. Chandrasekaran N et al: Prenatal sonographic diagnosis of meconium peritonitis from duodenal atresia. BMJ Case Rep. 2017, 2017
                                                                                  6. Angotti R et al: Association of duodenal atresia, malrotation, and atrial septal defect in a Down-syndrome patient. APSP J Case Rep. 7(2):16, 2016
                                                                                  7. Rattan KN et al: Neonatal duodenal obstruction: a 15-year experience. J Neonatal Surg. 5(2):13, 2016
                                                                                  8. Chandrasekharam V: Laparoscopic duodenoduodenostomy in neonates: report of two cases and review of the literature. J Indian Assoc Pediatr Surg. 20(3):150-2, 2015
                                                                                  9. Fragoso AC et al: Defective upper gastrointestinal function after repair of combined esophageal and duodenal atresia. J Pediatr Surg. 50(4):531-4, 2015
                                                                                  10. Virgone C et al: Accuracy of prenatal ultrasound in detecting jejunal and ileal atresia: systematic review and meta-analysis. Ultrasound Obstet Gynecol. 45(5):523-9, 2015
                                                                                  11. Adewole VA et al: Antenatally detected cystic biliary atresia: differential diagnoses of a double bubble. Springerplus. 3:368, 2014
                                                                                  12. Latzman JM et al: Duodenal atresia: not always a double bubble. Pediatr Radiol. 44(8):1031-4, 2014
                                                                                  13. Maxfield CM et al: A pattern-based approach to bowel obstruction in the newborn. Pediatr Radiol. 43(3):318-29, 2013
                                                                                  14. Mirza B et al: Multiple associated anomalies in patients of duodenal atresia: a case series. J Neonatal Surg. 1(2):23, 2012
                                                                                  15. Akcora B et al: A newborn with duodenal atresia and a gastric perforation. Afr J Paediatr Surg. 7(1):33-5, 2010
                                                                                  16. Choudhry MS et al: Duodenal atresia: associated anomalies, prenatal diagnosis and outcome. Pediatr Surg Int. 25(8):727-30, 2009
                                                                                  17. Gilbertson-Dahdal DL et al: Neonatal malrotation with midgut volvulus mimicking duodenal atresia. AJR Am J Roentgenol. 192(5):1269-71, 2009
                                                                                  18. Iwai A et al: Choledochal cyst associated with duodenal atresia: case report and review of the literature. Pediatr Surg Int. 25(11):995-8, 2009
                                                                                  19. Keckler SJ et al: The influence of trisomy 21 on the incidence and severity of congenital heart defects in patients with duodenal atresia. Pediatr Surg Int. 24(8):921-3, 2008
                                                                                  20. Mustafawi AR et al: Congenital duodenal obstruction in children: a decade's experience. Eur J Pediatr Surg. 18(2):93-7, 2008
                                                                                  21. Escobar MA et al: Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg. 39(6):867-71; discussion 867-71, 2004
                                                                                  22. Forrester MB et al: Population-based study of small intestinal atresia and stenosis, Hawaii, 1986-2000. Public Health. 118(6):434-8, 2004
                                                                                  23. Sugimoto T et al: Choledochal cyst and duodenal atresia: a rare combination of malformations. Pediatr Surg Int. 20(9):724-6, 2004
                                                                                  24. Doray B et al: Esophageal and duodenal atresia in a girl with a 12q24.3-qter deletion. Clin Genet. 61(6):468-71, 2002
                                                                                  25. Haeusler MC et al: Prenatal ultrasonographic detection of gastrointestinal obstruction: results from 18 European congenital anomaly registries. Prenat Diagn. 22(7):616-23, 2002
                                                                                  26. Mordehai J et al: Preduodenal portal vein causing duodenal obstruction associated with situs inversus, intestinal malrotation, and polysplenia: a case report. J Pediatr Surg. 37(4):E5, 2002
                                                                                  27. Pumberger W et al: Duodeno-jejunal atresia with volvulus, absent dorsal mesentery, and absent superior mesenteric artery: a hereditary compound structure in duodenal atresia? Am J Med Genet. 109(1):52-5, 2002
                                                                                  28. Rothenberg SS: Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and children. J Pediatr Surg. 37(7):1088-9, 2002
                                                                                  29. Sencan A et al: Symptomatic annular pancreas in newborns. Med Sci Monit. 8(6):CR434-7, 2002
                                                                                  30. Maruyama K et al: Partial monosomy 10q with partial trisomy 11q due to paternal balanced translocation. J Paediatr Child Health. 37(2):198-200, 2001
                                                                                  31. Dalla Vecchia LK et al: Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 133(5):490-6; discussion 496-7, 1998
                                                                                  32. Grosfeld JL: Jejunoileal atresia & stenosis. In: O'neal JA, Rowe MI, Grosfeld JL, eds. Pediatric Surgery, 3rd ed. St Louis: Mosby. 1145-58, 1998
                                                                                  33. Buonomo C: Neonatal gastrointestinal emergencies. Radiol Clin North Am. 35(4):845-64, 1997
                                                                                  34. Courtens W et al: Feingold syndrome: report of a new family and review. Am J Med Genet. 73(1):55-60, 1997
                                                                                  35. Lemire EG et al: A familial disorder with duodenal atresia and tetralogy of Fallot. Am J Med Genet. 66(1):39-44, 1996
                                                                                  36. Long FR et al: Intestinal malrotation in children: tutorial on radiographic diagnosis in difficult cases. Radiology. 198(3):775-80, 1996