Official reprint from UpToDate®
www.uptodate.com ©2017 UpToDate, Inc. and/or its affiliates. All Rights Reserved.

Intestinal malrotation in children

Mary L Brandt, MD
Section Editors
Jonathan I Singer, MD
Melvin B Heyman, MD, MPH
Deputy Editor
James F Wiley, II, MD, MPH


An overview of the presentation, diagnosis, and treatment of intestinal malrotation in children will be presented here.

Intestinal malrotation in adults and congenital diaphragmatic hernia and abdominal wall defects are discussed separately. (See "Intestinal malrotation in adults" and "Omphalocele".)


Rotational anomalies occur as a result of an arrest of normal rotation of the embryonic gut. Because rotational anomalies may remain asymptomatic throughout a person’s life span, the true incidence is not known. Nonrotation, a type of malrotation is an incidental finding on approximately 2 out of 1000 upper gastrointestinal contrast studies [1]. Symptomatic malrotation in neonates occurs with a frequency of about 1 in 6000 live births.

Traditionally, intestinal malrotation has been considered primarily a disease of infancy with infrequent occurrence beyond the first year of life [2,3]. However, analysis of 2744 cases of intestinal rotation in children up to 17 years of age obtained from a national hospital discharge database found the following [2]:

Presentation by one month of age: 30 percent

To continue reading this article, you must log in with your personal, hospital, or group practice subscription. For more information on subscription options, click below on the option that best describes you:

Subscribers log in here

Literature review current through: Nov 2017. | This topic last updated: Jan 03, 2017.
The content on the UpToDate website is not intended nor recommended as a substitute for medical advice, diagnosis, or treatment. Always seek the advice of your own physician or other qualified health care professional regarding any medical questions or conditions. The use of this website is governed by the UpToDate Terms of Use ©2017 UpToDate, Inc.
  1. Stockmann PT. Malrotation. In: Principles and Practice of Pediatric Surgery, 2nd ed, Oldham KT, Colombani PM, Foglia RP, Skinner MA (Eds), Lippincott Williams & Wilkings, Philadelphia 2005. Vol 2, p.1283.
  2. Aboagye J, Goldstein SD, Salazar JH, et al. Age at presentation of common pediatric surgical conditions: Reexamining dogma. J Pediatr Surg 2014; 49:995.
  3. Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery 2011; 149:386.
  4. Stewart DR, Colodny AL, Daggett WC. Malrotation of the bowel in infants and children: a 15 year review. Surgery 1976; 79:716.
  5. Filston HC, Kirks DR. Malrotation - the ubiquitous anomaly. J Pediatr Surg 1981; 16:614.
  6. KIESEWETTER WB, SMITH JW. Malrotation of the midgut in infancy and childhood. AMA Arch Surg 1958; 77:483.
  7. Ford EG, Senac MO Jr, Srikanth MS, Weitzman JJ. Malrotation of the intestine in children. Ann Surg 1992; 215:172.
  8. Graziano K, Islam S, Dasgupta R, et al. Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. J Pediatr Surg 2015; 50:1783.
  9. Adams SD, Stanton MP. Malrotation and intestinal atresias. Early Hum Dev 2014; 90:921.
  10. Pachl M, Eaton S, Kiely EM, et al. Esophageal atresia and malrotation: what association? Pediatr Surg Int 2015; 31:181.
  11. Aslanabadi S, Ghalehgolab-Behbahan A, Jamshidi M, et al. Intestinal malrotations: a review and report of thirty cases. Folia Morphol (Warsz) 2007; 66:277.
  12. Martinez-Leo B, Chesley P, Alam S, et al. The association of the severity of anorectal malformations and intestinal malrotation. J Pediatr Surg 2016; 51:1241.
  13. Chesley PM, Melzer L, Bradford MC, Avansino JR. Association of anorectal malformation and intestinal malrotation. Am J Surg 2015; 209:907.
  14. Deardorff MA, Noon SE, Krantz ID. Cornelia de Lange Syndrome. In: Gene Reviews, Pagon RA, Adam MP, Ardinger HH, et al (Eds), University of Washington, Seattle 2016.
  15. Fonkalsrud E. Rotational anomalies and volvulus. In: Principles of Pediatric Surgery, O'Neill JA et al (Ed), Mosby, St. Louis 2003. p.477.
  16. Diaz MC, Reichard K, Taylor AA. Intestinal nonrotation in an adolescent. Pediatr Emerg Care 2009; 25:249.
  17. von Flüe M, Herzog U, Ackermann C, et al. Acute and chronic presentation of intestinal nonrotation in adults. Dis Colon Rectum 1994; 37:192.
  19. Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and adults: spectrum of clinical and imaging features. AJR Am J Roentgenol 2002; 179:1429.
  20. Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990; 108:710.
  21. Powell DM, Othersen HB, Smith CD. Malrotation of the intestines in children: the effect of age on presentation and therapy. J Pediatr Surg 1989; 24:777.
  22. Lin JN, Lou CC, Wang KL. Intestinal malrotation and midgut volvulus: a 15-year review. J Formos Med Assoc 1995; 94:178.
  23. Prasil P, Flageole H, Shaw KS, et al. Should malrotation in children be treated differently according to age? J Pediatr Surg 2000; 35:756.
  24. Kirby CP, Freeman JK, Ford WD, et al. Malrotation with recurrent volvulus presenting with cholestasis, pruritus, and pancreatitis. Pediatr Surg Int 2000; 16:130.
  25. Spigland N, Brandt ML, Yazbeck S. Malrotation presenting beyond the neonatal period. J Pediatr Surg 1990; 25:1139.
  26. Brandt ML, Pokorny WJ, McGill CW, Harberg FJ. Late presentations of midgut malrotation in children. Am J Surg 1985; 150:767.
  27. Yanez R, Spitz L. Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 1986; 61:682.
  28. Kullendorff CM, Mikaelsson C, Ivancev K. Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Paediatr Scand 1985; 74:296.
  29. Gardner CE, Hart D. Anomalies of intestinal rotation as a cause of intestinal obstructions: report of two personal observations: review of one hundred and three reported cases. Arch Surg 1934; 29:942.
  30. Millar AJ, Rode H, Cywes S. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg 2003; 12:229.
  31. Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ 2013; 347:f6949.
  32. Applegate KE. Evidence-based diagnosis of malrotation and volvulus. Pediatr Radiol 2009; 39 Suppl 2:S161.
  33. Sizemore AW, Rabbani KZ, Ladd A, Applegate KE. Diagnostic performance of the upper gastrointestinal series in the evaluation of children with clinically suspected malrotation. Pediatr Radiol 2008; 38:518.
  34. Long FR, Kramer SS, Markowitz RI, et al. Intestinal malrotation in children: tutorial on radiographic diagnosis in difficult cases. Radiology 1996; 198:775.
  35. Long FR, Kramer SS, Markowitz RI, Taylor GE. Radiographic patterns of intestinal malrotation in children. Radiographics 1996; 16:547.
  36. American College of Radiology. ACR appropriateness criteria. Vomiting in infants up to 3 months of age. http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/VomitingInInfantsUpTo3MonthsOfAge.pdf (Accessed on August 06, 2012).
  37. Dilley AV, Pereira J, Shi EC, et al. The radiologist says malrotation: does the surgeon operate? Pediatr Surg Int 2000; 16:45.
  38. Berdon WE. The diagnosis of malrotation and volvulus in the older child and adult: a trap for radiologists. Pediatr Radiol 1995; 25:101.
  39. Taylor GA. CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation. Pediatr Radiol 2011; 41:1378.
  40. Kouki S, Fares A, Alard S. MRI whirpool sign in midgut volvulus with malrotation in pregnancy. JBR-BTR 2013; 96:360.
  41. Biyyam DR, Dighe M, Siebert JR. Antenatal diagnosis of intestinal malrotation on fetal MRI. Pediatr Radiol 2009; 39:847.
  42. Yousefzadeh DK. The position of the duodenojejunal junction: the wrong horse to bet on in diagnosing or excluding malrotation. Pediatr Radiol 2009; 39 Suppl 2:S172.
  43. Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy at US in patients with surgically proved malrotation of the midgut. Radiology 1992; 183:693.
  44. Dufour D, Delaet MH, Dassonville M, et al. Midgut malrotation, the reliability of sonographic diagnosis. Pediatr Radiol 1992; 22:21.
  45. Pracros JP, Sann L, Genin G, et al. Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign. Pediatr Radiol 1992; 22:18.
  46. Ashley LM, Allen S, Teele RL. A normal sonogram does not exclude malrotation. Pediatr Radiol 2001; 31:354.
  47. Orzech N, Navarro OM, Langer JC. Is ultrasonography a good screening test for intestinal malrotation? J Pediatr Surg 2006; 41:1005.
  48. Morrison SC. Controversies in abdominal imaging. Pediatr Clin North Am 1997; 44:555.
  49. Firor HV, Harris VJ. Rotational abnormalities of the gut. Re-emphasis of a neglected facet, isolated incomplete rotation of the duodenum. Am J Roentgenol Radium Ther Nucl Med 1974; 120:315.
  50. Siegel MJ, Shackelford GD, McAlister WH. Small bowel volvulus in children: its appearance on the barium enema examination. Pediatr Radiol 1980; 10:91.
  51. Hsiao M, Langer JC. Value of laparoscopy in children with a suspected rotation abnormality on imaging. J Pediatr Surg 2011; 46:1347.
  52. Bass KD, Rothenberg SS, Chang JH. Laparoscopic Ladd's procedure in infants with malrotation. J Pediatr Surg 1998; 33:279.
  53. Waldhausen JH, Sawin RS. Laparoscopic Ladd's procedure and assessment of malrotation. J Laparoendosc Surg 1996; 6 Suppl 1:S103.
  54. Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg 1997; 185:172.
  55. Lessin MS, Luks FI. Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int 1998; 13:184.
  56. Ooms N, Matthyssens LE, Draaisma JM, et al. Laparoscopic Treatment of Intestinal Malrotation in Children. Eur J Pediatr Surg 2016; 26:376.
  57. Seashore JH, Touloukian RJ. Midgut volvulus. An ever-present threat. Arch Pediatr Adolesc Med 1994; 148:43.
  58. Messineo A, MacMillan JH, Palder SB, Filler RM. Clinical factors affecting mortality in children with malrotation of the intestine. J Pediatr Surg 1992; 27:1343.
  59. Andrassy RJ, Mahour GH. Malrotation of the midgut in infants and children: a 25-year review. Arch Surg 1981; 116:158.