Intestinal malrotation in children
- Mary L Brandt, MD
Mary L Brandt, MD
- Professor of Surgery, Division of Pediatric Surgery
- Baylor College of Medicine
- Section Editors
- Jonathan I Singer, MD
Jonathan I Singer, MD
- Section Editor — Pediatric Surgical Emergencies
- Professor of Emergency Medicine and Pediatrics
- Wright State University Boonshoft School of Medicine
- Melvin B Heyman, MD, MPH
Melvin B Heyman, MD, MPH
- Section Editor — Pediatric Gastroenterology
- Professor of Pediatrics
- University of California, San Francisco
- Deputy Editor
- James F Wiley, II, MD, MPH
James F Wiley, II, MD, MPH
- Senior Deputy Editor — UpToDate
- Deputy Editor — Adult and Pediatric Emergency Medicine
- Deputy Editor — Primary Care Sports Medicine (Adolescents and Adults)
- Clinical Professor of Pediatrics and Emergency Medicine/Traumatology
- University of Connecticut School of Medicine
An overview of the presentation, diagnosis, and treatment of intestinal malrotation in children will be presented here.
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 . 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 :
●Presentation by one month of age: 30 percent
- 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.
- Aboagye J, Goldstein SD, Salazar JH, et al. Age at presentation of common pediatric surgical conditions: Reexamining dogma. J Pediatr Surg 2014; 49:995.
- Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery 2011; 149:386.
- Stewart DR, Colodny AL, Daggett WC. Malrotation of the bowel in infants and children: a 15 year review. Surgery 1976; 79:716.
- Filston HC, Kirks DR. Malrotation - the ubiquitous anomaly. J Pediatr Surg 1981; 16:614.
- KIESEWETTER WB, SMITH JW. Malrotation of the midgut in infancy and childhood. AMA Arch Surg 1958; 77:483.
- Ford EG, Senac MO Jr, Srikanth MS, Weitzman JJ. Malrotation of the intestine in children. Ann Surg 1992; 215:172.
- 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.
- Adams SD, Stanton MP. Malrotation and intestinal atresias. Early Hum Dev 2014; 90:921.
- Pachl M, Eaton S, Kiely EM, et al. Esophageal atresia and malrotation: what association? Pediatr Surg Int 2015; 31:181.
- Aslanabadi S, Ghalehgolab-Behbahan A, Jamshidi M, et al. Intestinal malrotations: a review and report of thirty cases. Folia Morphol (Warsz) 2007; 66:277.
- 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.
- Chesley PM, Melzer L, Bradford MC, Avansino JR. Association of anorectal malformation and intestinal malrotation. Am J Surg 2015; 209:907.
- 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.
- Fonkalsrud E. Rotational anomalies and volvulus. In: Principles of Pediatric Surgery, O'Neill JA et al (Ed), Mosby, St. Louis 2003. p.477.
- Diaz MC, Reichard K, Taylor AA. Intestinal nonrotation in an adolescent. Pediatr Emerg Care 2009; 25:249.
- von Flüe M, Herzog U, Ackermann C, et al. Acute and chronic presentation of intestinal nonrotation in adults. Dis Colon Rectum 1994; 37:192.
- WANG CA, WELCH CE. ANOMALIES OF INTESTINAL ROTATION IN ADOLESCENTS AND ADULTS. Surgery 1963; 54:839.
- Pickhardt PJ, Bhalla S. Intestinal malrotation in adolescents and adults: spectrum of clinical and imaging features. AJR Am J Roentgenol 2002; 179:1429.
- Rescorla FJ, Shedd FJ, Grosfeld JL, et al. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery 1990; 108:710.
- 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.
- Lin JN, Lou CC, Wang KL. Intestinal malrotation and midgut volvulus: a 15-year review. J Formos Med Assoc 1995; 94:178.
- Prasil P, Flageole H, Shaw KS, et al. Should malrotation in children be treated differently according to age? J Pediatr Surg 2000; 35:756.
- Kirby CP, Freeman JK, Ford WD, et al. Malrotation with recurrent volvulus presenting with cholestasis, pruritus, and pancreatitis. Pediatr Surg Int 2000; 16:130.
- Spigland N, Brandt ML, Yazbeck S. Malrotation presenting beyond the neonatal period. J Pediatr Surg 1990; 25:1139.
- Brandt ML, Pokorny WJ, McGill CW, Harberg FJ. Late presentations of midgut malrotation in children. Am J Surg 1985; 150:767.
- Yanez R, Spitz L. Intestinal malrotation presenting outside the neonatal period. Arch Dis Child 1986; 61:682.
- Kullendorff CM, Mikaelsson C, Ivancev K. Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Paediatr Scand 1985; 74:296.
- 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.
- Millar AJ, Rode H, Cywes S. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg 2003; 12:229.
- Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ 2013; 347:f6949.
- Applegate KE. Evidence-based diagnosis of malrotation and volvulus. Pediatr Radiol 2009; 39 Suppl 2:S161.
- 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.
- Long FR, Kramer SS, Markowitz RI, et al. Intestinal malrotation in children: tutorial on radiographic diagnosis in difficult cases. Radiology 1996; 198:775.
- Long FR, Kramer SS, Markowitz RI, Taylor GE. Radiographic patterns of intestinal malrotation in children. Radiographics 1996; 16:547.
- 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).
- Dilley AV, Pereira J, Shi EC, et al. The radiologist says malrotation: does the surgeon operate? Pediatr Surg Int 2000; 16:45.
- Berdon WE. The diagnosis of malrotation and volvulus in the older child and adult: a trap for radiologists. Pediatr Radiol 1995; 25:101.
- Taylor GA. CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation. Pediatr Radiol 2011; 41:1378.
- Kouki S, Fares A, Alard S. MRI whirpool sign in midgut volvulus with malrotation in pregnancy. JBR-BTR 2013; 96:360.
- Biyyam DR, Dighe M, Siebert JR. Antenatal diagnosis of intestinal malrotation on fetal MRI. Pediatr Radiol 2009; 39:847.
- 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.
- Zerin JM, DiPietro MA. Superior mesenteric vascular anatomy at US in patients with surgically proved malrotation of the midgut. Radiology 1992; 183:693.
- Dufour D, Delaet MH, Dassonville M, et al. Midgut malrotation, the reliability of sonographic diagnosis. Pediatr Radiol 1992; 22:21.
- Pracros JP, Sann L, Genin G, et al. Ultrasound diagnosis of midgut volvulus: the "whirlpool" sign. Pediatr Radiol 1992; 22:18.
- Ashley LM, Allen S, Teele RL. A normal sonogram does not exclude malrotation. Pediatr Radiol 2001; 31:354.
- Orzech N, Navarro OM, Langer JC. Is ultrasonography a good screening test for intestinal malrotation? J Pediatr Surg 2006; 41:1005.
- Morrison SC. Controversies in abdominal imaging. Pediatr Clin North Am 1997; 44:555.
- 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.
- Siegel MJ, Shackelford GD, McAlister WH. Small bowel volvulus in children: its appearance on the barium enema examination. Pediatr Radiol 1980; 10:91.
- Hsiao M, Langer JC. Value of laparoscopy in children with a suspected rotation abnormality on imaging. J Pediatr Surg 2011; 46:1347.
- Bass KD, Rothenberg SS, Chang JH. Laparoscopic Ladd's procedure in infants with malrotation. J Pediatr Surg 1998; 33:279.
- Waldhausen JH, Sawin RS. Laparoscopic Ladd's procedure and assessment of malrotation. J Laparoendosc Surg 1996; 6 Suppl 1:S103.
- Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg 1997; 185:172.
- Lessin MS, Luks FI. Laparoscopic appendectomy and duodenocolonic dissociation (LADD) procedure for malrotation. Pediatr Surg Int 1998; 13:184.
- Ooms N, Matthyssens LE, Draaisma JM, et al. Laparoscopic Treatment of Intestinal Malrotation in Children. Eur J Pediatr Surg 2016; 26:376.
- Seashore JH, Touloukian RJ. Midgut volvulus. An ever-present threat. Arch Pediatr Adolesc Med 1994; 148:43.
- 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.
- Andrassy RJ, Mahour GH. Malrotation of the midgut in infants and children: a 25-year review. Arch Surg 1981; 116:158.
- Associated congenital defects
- EMBRYOLOGY AND PATHOGENESIS
- CLINICAL PRESENTATION
- Clinical suspicion
- Diagnostic approach
- - Intestinal perforation with peritonitis
- - Imaging in stable patients
- Patients with equivocal findings on imaging
- DIFFERENTIAL DIAGNOSIS
- Symptomatic malrotation
- - Laparoscopy
- Asymptomatic malrotation
- - Short gut syndrome
- - Small bowel obstruction
- Resolution of symptoms
- Recurrent volvulus
- SUMMARY AND RECOMMENDATIONS