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Diseases

Intestinal malrotation

OVERVIEW

  • Highlights & Basics
  • Images

DIAGNOSIS

  • Diagnostic Approach
  • Risk Factors
  • History & Exam
  • Tests
  • Differential Diagnosis
  • Criteria
  • Screening

TREATMENT

  • Tx Approach
  • Tx Options
  • Emerging Tx
  • Prevention

FOLLOW-UP

  • Overview
  • Complications

REFERENCES

  • Citations
  • Guidelines
  • Credits

PATIENT RESOURCES

  • Patient Instructions

Highlights & Basics

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Key Highlights
  • For those with intestinal malrotation, the anatomic variant that poses the highest risk of volvulus is a narrow midgut mesenteric base accompanied by lack of retroperitoneal midgut fixation. This variant cannot be reliably determined from any radiologic studies.

  • Malrotation predisposes patients to a risk of midgut volvulus.

  • The presence of bilious (dark green) vomiting is a key factor in considering the possibility of midgut volvulus. Prompt discussion with a pediatric surgeon is advised in this scenario to ensure timely referral, investigation, and management.

  • Emergency surgical consultation is appropriate before obtaining any diagnostic studies if midgut volvulus is suspected. Upper gastrointestinal contrast studies should be ordered in patients who are stable.

  • Treatment is surgical correction, the procedure of choice being a Ladd procedure.

Malrotation with volvulus causing complete obstruction of the duodenum that does not sweep to the le
Malrotation with volvulus causing complete obstruction of the duodenum that does not sweep to the left, as seen on upper GI contrast
From the collection of Dr S.D. St Peter

Quick Reference

  • History & Exam

    • Key Factors

      • Other Factors

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      • Diagnostics Tests

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        • Treatment Options

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          Definition

          Epidemiology

          Etiology

          Pathophysiology

          content by BMJ Group
          Last updated

          Images

          • Malrotation with volvulus causing complete obstruction of the duodenum that does not sweep to the le

            Malrotation with volvulus causing complete obstruction of the duodenum that does not sweep to the left, as seen on upper GI contrast

          • Narrow base of mesentery in malrotation

            Narrow base of mesentery in malrotation

          • Malrotation with volvulus without obstruction as seen on upper GI contrast

            Malrotation with volvulus without obstruction as seen on upper GI contrast

          Citations

            Key Articles

            • Shew SB. Surgical concerns in malrotation and midgut volvulus. Pediatr Radiol. 2009;39(suppl 2):S167-71.[Abstract]

            • Expert Panel on Pediatric Imaging, Alazraki AL, Rigsby CK, et al. ACR appropriateness criteria® vomiting in infants. J Am Coll Radiol. 2020 Nov;17(11s):S505-15.[Abstract][Full Text]

            • 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 Oct;50(10):1783-90.[Abstract]

            • Ooms N, Matthyssens LE, Draaisma JM, et al. Laparoscopic treatment of intestinal malrotation in children. Eur J Pediatr Surg. 2016 Aug;26(4):376-81.[Abstract][Full Text]

            Referenced Articles

            • 1. Flynn-O'Brien KT, Rice-Townsend SE. Structural anomalies of the gastrointestinal tract. In: Gleason CA, Juul SE, eds. Avery's Diseases of the Newborn. 11th ed. Philadelphia, PA: Elsevier;2024:897-912.

            • 2. Gross RE. Malrotation of the intestine and colon. In: Gros RE, ed. The surgery of infancy and childhood. Philadelphia, PA: WB Saunders; 1953:192.

            • 3. Forrester MB, Merz RD. Epidemiology of intestinal malrotation, Hawaii, 1986-99. Paediatr Perinat Epidemiol. 2003 Apr;17(2):195-200.[Abstract]

            • 4. von Flüe M, Herzog U, Ackermann C, et al. Acute and chronic presentation of intestinal nonrotation in adults. Dis Colon Rectum. 1994 Feb;37(2):192-8.[Abstract]

            • 5. Schulman J, Edmonds LD, McClearn AB, et al. Surveillance for and comparison of birth defect prevalences in two geographic areas-United States, 1983-88. MMWR CDC Surveill Summ. 1993 Mar 19;42(1):1-7.[Abstract][Full Text]

            • 6. Kantor JL. Anomalies of the colon: their roentgen diagnosis and clinical significance: Resume of 10 years study. Radiology. 1934;23:651-662.

            • 7. Skandalakis JE, Gray SW, Ricketts R, et al. The small intestines. In: Skandalakis JE, Gray SW, eds. Embryology for surgeons, 2nd ed. Baltimore, MD: Williams & Wilkins; 1994:184.

            • 8. Gray SW, Skandalakis JE. Embryology for surgeons. Philadelphia, PA: WB Saunders; 1972:129-141.

            • 9. Sadler TW. Langman's medical embryology, 7th Ed. Philadelphia, PA: Lippincott Williams & Wilkins; 1995:258-267.

            • 10. Shew SB. Surgical concerns in malrotation and midgut volvulus. Pediatr Radiol. 2009;39(suppl 2):S167-71.[Abstract]

            • 11. Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ. 2013 Nov 26;347:f6949.

            • 12. Expert Panel on Pediatric Imaging, Alazraki AL, Rigsby CK, et al. ACR appropriateness criteria® vomiting in infants. J Am Coll Radiol. 2020 Nov;17(11s):S505-15.[Abstract][Full Text]

            • 13. Sizemore AW, Rabbani KZ, Ladd A, et al. Diagnostic performance of the upper gastrointestinal series in the evaluation of children with clinically suspected malrotation. Pediatr Radiol. 2008 May;38(5):518-28.[Abstract]

            • 14. Applegate KE. Evidence-based diagnosis of malrotation and volvulus. Pediatr Radiol. 2009;39(suppl 2):S161-3.[Abstract]

            • 15. Hsiao M, Langer JC. Value of laparoscopy in children with a suspected rotation abnormality on imaging. J Pediatr Surg. 2011 Jul;46(7):1347-52.[Abstract]

            • 16. Kirks DR, Caron KJ. Gastrointestinal tract. In: Kirks DR, ed. Practical pediatric imaging, 2nd ed. Philadelphia, PA: JB Lippincott; 1991:710.

            • 17. Daneman A. Malrotation: the balance of evidence. Pediatr Radiol. 2009;39(suppl 2):S164-S166.[Abstract]

            • 18. Nguyen HN, Navarro OM, Bloom DA, et al. Ultrasound for midgut malrotation and midgut volvulus: AJR expert panel narrative review. AJR Am J Roentgenol. 2022 Jun;218(6):931-9.[Abstract][Full Text]

            • 19. Nguyen HN, Kulkarni M, Jose J, et al. Ultrasound for the diagnosis of malrotation and volvulus in children and adolescents: a systematic review and meta-analysis. Arch Dis Child. 2021 Dec;106(12):1171-8.[Abstract][Full Text]

            • 20. Xiong Z, Shen Y, Morelli JN, et al. CT facilitates improved diagnosis of adult intestinal malrotation: a 7-year retrospective study based on 332 cases. Insights Imaging. 2021 Apr 30;12(1):58.[Abstract][Full Text]

            • 21. Royal College of Surgeons of England. Emergency general surgery - commissioning guide​. Apr 2014 [internet publication].[Full Text]

            • 22. Lin YP, Lee J, Chao HC, et al. Risk factors for intestinal gangrene in children with small-bowel volvulus. J Pediatr Gastroenterol Nutr. 2011 Oct;53(4):417-22.[Abstract]

            • 23. Guan X, Wang Z, He Q, et al. Nomogram for estimating the risks of intestinal ischemia and necrosis in neonates with midgut volvulus: a retrospective study. Front Pediatr. 2022 Jun 20;10:888594.[Abstract][Full Text]

            • 24. Fo Y, Kang X, Tang Y, et al. Analysis of clinical diagnosis and treatment of intestinal volvulus. BMC Gastroenterol. 2023 Mar 28;23(1):93.[Abstract][Full Text]

            • 25. Powell D, Othersen JB, Smith CD. Malrotation of the intestines in children: the effect of age on presentation and therapy. J Pediatr Surg. 1989;24:777-780.[Abstract]

            • 26. 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 Oct;50(10):1783-90.[Abstract]

            • 27. Yu DC, Thiagarajan RR, Laussen PC, et al. Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg. 2009;44:1089-1095.[Abstract]

            • 28. Tsao KJ, St Peter SD, Valusek PA, et al. Adhesive small bowel obstruction after appendectomy in children: comparison between the laparoscopic and open approach. J Pediatr Surg. 2007 Jun;42(6):939-42;discussion 942.[Abstract]

            • 29. Zhang Z, Chen Y, Yan J. Laparoscopic versus open Ladd's procedure for intestinal malrotation in infants and children: a systematic review and meta-analysis. J Laparoendosc Adv Surg Tech A. 2022 Feb;32(2):204-12.[Abstract]

            • 30. Ooms N, Matthyssens LE, Draaisma JM, et al. Laparoscopic treatment of intestinal malrotation in children. Eur J Pediatr Surg. 2016 Aug;26(4):376-81.[Abstract][Full Text]

            • 31. Fraser JD, Aguayo P, Sharp SW, et al. The role of laparoscopy in the management of malrotation. J Surg Res. 2009;156:80-82.[Abstract]

            • 32. Bratzler DW, Dellinger EP, Olsen KM, et al. Clinical practice guidelines for antimicrobial prophylaxis in surgery. Am J Health Syst Pharm. 2013 Feb 1;70(3):195-283.[Full Text]

            • 33. Mazuski JE, Tessier JM, May AK, et al. The Surgical Infection Society revised guidelines on the management of intra-abdominal infection. Surg Infect (Larchmt). 2017 Jan;18(1):1-76.[Abstract][Full Text]

            • 34. Lessin MS, Luks FI. Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int. 1998;13:184-185.[Abstract]

            • 35. Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg. 1997;185:172-176.[Abstract]

            • 36. Draus JM Jr, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg. 2007;73:693-696.[Abstract]

            • 37. NHS Improvement. Patient Safety review and response: April to September 2018. March 2019 [internet publication].[Full Text]

            • 38. NHS England. Patient safety alert on placement devices for nasogastric tube insertion. December 2019 [internet publication].[Full Text]

            • 39. Warner BW. Malrotation. In: Oldham KT, Colambani PM, Foglia RP, eds. Surgery of infants and children: scientific principles and practice. Philadelphia, PA: Lippincott-Raven; 1997:1229-1240.

            • 40. Stewart DR, Colodny AL, Daggett WC. Malrotation of the bowel in infants and children: a 15 year review. Surgery. 1976;19:716-720.[Abstract]

            • 41. Ferreira MS, Simões J, Folgado A, et al. Recurrent midgut volvulus in an adult patient - the case for pexy? a case report and review of the literature. Int J Surg Case Rep. 2020;66:91-5.[Abstract][Full Text]

            • 42. Jen HC, Shew SB. Laparoscopic versus open appendectomy in children: outcomes comparison based on a statewide analysis. J Surg Res. 2010;161:13-17.[Abstract]

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