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Intestinal atresia

David E Wesson, MD
Section Editors
Melvin B Heyman, MD, MPH
Joseph A Garcia-Prats, MD
Deputy Editor
Alison G Hoppin, MD


An atresia is a congenital defect of a hollow viscus that results in complete obstruction of the lumen. Intestinal atresia is one of the most frequent causes of bowel obstruction in the newborn and can occur at any point in the gastrointestinal tract. The ileum is the most commonly affected site.  

The outcome of intestinal atresia following surgical repair is very good. In general, morbidity and mortality depend upon associated medical conditions such as prematurity or cystic fibrosis, other congenital anomalies, the complexity of the lesion, and surgical complications.

Atresia and stenosis of the small and large intestine are reviewed here. Esophageal atresia and anorectal malformations, including rectal atresia, are discussed separately. (See "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula", section on 'Tracheoesophageal fistula and esophageal atresia' and "Functional constipation in infants and children: Clinical features and differential diagnosis", section on 'Other causes'.)


The most common site of intestinal atresia is the small intestine (jejunum and ileum). The incidence of jejunal and ileal atresia ranges from one in 1500 to one in 12,000 births [1].

Duodenal atresia occurs in one in 10,000 to 40,000 births and represents up to 60 percent of intestinal atresias [1]. Approximately 30 percent of infants with duodenal atresia have a chromosomal anomaly, primarily Down syndrome. (See "Down syndrome: Clinical features and diagnosis".)


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Literature review current through: Sep 2016. | This topic last updated: Aug 15, 2016.
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  1. Best KE, Tennant PW, Addor MC, et al. Epidemiology of small intestinal atresia in Europe: a register-based study. Arch Dis Child Fetal Neonatal Ed 2012; 97:F353.
  2. Dalla Vecchia LK, Grosfeld JL, West KW, et al. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133:490.
  3. Louw JH. Resection and end-to-end anastomosis in the management of atresia and stenosis of the small bowel. Surgery 1967; 62:940.
  4. Martin LW, Zerella JT. Jejunoileal atresia: a proposed classification. J Pediatr Surg 1976; 11:399.
  5. Grosfeld JL, Ballantine TV, Shoemaker R. Operative mangement of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg 1979; 14:368.
  6. Kao KJ, Fleischer R, Bradford WD, Woodard BH. Multiple congenital septal atresias of the intestine: histomorphologic and pathogenetic implications. Pediatr Pathol 1983; 1:443.
  7. Tibboel D, van der Kamp AW, Molenaar JC. The effect of experimentally induced intestinal perforation at an early developmental stage. J Pediatr Surg 1981; 16:1017.
  8. Lopez de Torre B, Tovar JA, Uriarte S, Aldazabal P. The nutrition of the fetus with intestinal atresia: studies in the chick embryo model. J Pediatr Surg 1992; 27:1325.
  9. Kilic N, Kiristioglu I, Kirkpinar A, Dogruyol H. A very rare cause of intestinal atresia: intrauterine intussusception due to Meckel's diverticulum. Acta Paediatr 2003; 92:756.
  10. Werler MM, Sheehan JE, Mitchell AA. Association of vasoconstrictive exposures with risks of gastroschisis and small intestinal atresia. Epidemiology 2003; 14:349.
  11. Johnson SM, Meyers RL. Inherited thrombophilia: a possible cause of in utero vascular thrombosis in children with intestinal atresia. J Pediatr Surg 2001; 36:1146.
  12. Nichol PF, Reeder A, Botham R. Humans, mice, and mechanisms of intestinal atresias: a window into understanding early intestinal development. J Gastrointest Surg 2011; 15:694.
  13. Baerg J, Kaban G, Tonita J, et al. Gastroschisis: A sixteen-year review. J Pediatr Surg 2003; 38:771.
  14. Kimble RM, Blakelock R, Cass D. Vanishing gut in infants with gastroschisis. Pediatr Surg Int 1999; 15:483.
  15. Ogunyemi D. Gastroschisis complicated by midgut atresia, absorption of bowel, and closure of the abdominal wall defect. Fetal Diagn Ther 2001; 16:227.
  16. Adams SD, Stanton MP. Malrotation and intestinal atresias. Early Hum Dev 2014; 90:921.
  17. Seashore JH, Collins FS, Markowitz RI, Seashore MR. Familial apple peel jejunal atresia: surgical, genetic, and radiographic aspects. Pediatrics 1987; 80:540.
  18. Moreno LA, Gottrand F, Turck D, et al. Severe combined immunodeficiency syndrome associated with autosomal recessive familial multiple gastrointestinal atresias: study of a family. Am J Med Genet 1990; 37:143.
  19. Bilodeau A, Prasil P, Cloutier R, et al. Hereditary multiple intestinal atresia: thirty years later. J Pediatr Surg 2004; 39:726.
  20. Chen R, Giliani S, Lanzi G, et al. Whole-exome sequencing identifies tetratricopeptide repeat domain 7A (TTC7A) mutations for combined immunodeficiency with intestinal atresias. J Allergy Clin Immunol 2013; 132:656.
  21. Filges I, Bruder E, Brandal K, et al. Strømme Syndrome Is a Ciliary Disorder Caused by Mutations in CENPF. Hum Mutat 2016; 37:359.
  22. Seo T, Ando H, Watanabe Y, et al. Colonic atresia and Hirschsprung's disease: importance of histologic examination of the distal bowel. J Pediatr Surg 2002; 37:E19.
  23. Cozzi F, Wilkinson AW. Intrauterine growth rate in relation to anorectal and oesophageal anomalies. Arch Dis Child 1969; 44:59.
  24. Tulloh RM, Tansey SP, Parashar K, et al. Echocardiographic screening in neonates undergoing surgery for selected gastrointestinal malformations. Arch Dis Child Fetal Neonatal Ed 1994; 70:F206.
  25. Epstein CJ. Down syndrome (Trisomy 21). In: The Metabolic and Molecular Bases of Inherited Disease, 8th, Scriver CR, Beaudet AL, Sly WS, Valle D (Eds), McGraw-Hill, New York 2001. p.1223.
  26. Sweeney B, Surana R, Puri P. Jejunoileal atresia and associated malformations: correlation with the timing of in utero insult. J Pediatr Surg 2001; 36:774.
  27. Azzie G, Craw S, Beasley SW. Colonic atresia: From suspicion to confirmation on pre-operative radiology. J Paediatr Child Health 2002; 38:518.
  28. Sauve RS, Leung AK. Congenital varicella syndrome with colonic atresias. Clin Pediatr (Phila) 2003; 42:451.
  29. Brantberg A, Blaas HG, Salvesen KA, et al. Fetal duodenal obstructions: increased risk of prenatal sudden death. Ultrasound Obstet Gynecol 2002; 20:439.
  30. Phelps S, Fisher R, Partington A, Dykes E. Prenatal ultrasound diagnosis of gastrointestinal malformations. J Pediatr Surg 1997; 32:438.
  31. Ghose I, Mason GC, Martinez D, et al. Hyperechogenic fetal bowel: a prospective analysis of sixty consecutive cases. BJOG 2000; 107:426.
  32. Kumaran N, Shankar KR, Lloyd DA, Losty PD. Trends in the management and outcome of jejuno-ileal atresia. Eur J Pediatr Surg 2002; 12:163.
  33. Tam PK, Nicholls G. Implications of antenatal diagnosis of small-intestinal atresia in the 1990s. Pediatr Surg Int 1999; 15:486.
  34. Corteville JE, Gray DL, Langer JC. Bowel abnormalities in the fetus--correlation of prenatal ultrasonographic findings with outcome. Am J Obstet Gynecol 1996; 175:724.
  35. Gilbertson-Dahdal DL, Dutta S, Varich LJ, Barth RA. Neonatal malrotation with midgut volvulus mimicking duodenal atresia. AJR Am J Roentgenol 2009; 192:1269.
  36. Francisco J RP, Cintrón Diaz E, Idelissa L. Colonic duplication: another suspected diagnosis in a prenatal ultrasound with double bubble sign. Bol Asoc Med P R 2012; 104:55.
  37. Schwartzberg D, Burjonrappa SC. Pseudo double bubble: jejunal duplication mimicking duodenal atresia on prenatal ultrasound. J Neonatal Surg 2013; 2:42.
  38. Kimble RM, Harding J, Kolbe A. Additional congenital anomalies in babies with gut atresia or stenosis: when to investigate, and which investigation. Pediatr Surg Int 1997; 12:565.
  39. Patil VK, Kulkarni BK, Jiwane A, et al. Intestinal atresia: an end-to-end linear anastomotic technique. Pediatr Surg Int 2001; 17:661.
  40. Watts AC, Sabharwal AJ, MacKinlay GA, Munro FD. Congenital colonic atresia: should primary anastomosis always be the goal? Pediatr Surg Int 2003; 19:14.
  41. Escobar MA, Ladd AP, Grosfeld JL, et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg 2004; 39:867.
  42. Festen S, Brevoord JC, Goldhoorn GA, et al. Excellent long-term outcome for survivors of apple peel atresia. J Pediatr Surg 2002; 37:61.