- 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
- George D Ferry, MD
George D Ferry, MD
- Section Editor — Pediatric Gastroenterology
- Professor of Pediatrics
- Baylor College of Medicine
- Deputy Editor
- James F Wiley, II, MD, MPH
James F Wiley, II, MD, MPH
- Senior Deputy Editor — Adult and Pediatric Emergency Medicine
- Senior Deputy Editor — Primary Care Sports Medicine (Adolescents and Adults)
- Professor of Pediatrics and Emergency Medicine/Traumatology
- University of Connecticut School of Medicine
Rotational anomalies occur as a result of an arrest of normal rotation of the embryonic gut. They are often associated with other gastrointestinal abnormalities, particularly those in which the intestines are located outside the coelomic cavity (eg, congenital diaphragmatic hernia or abdominal wall defects). However, intestinal malrotation can also occur in children and adults who have no associated anomalies.
An overview of the presentation, diagnosis, and treatment of intestinal malrotation will be presented here. Congenital diaphragmatic hernia and abdominal wall defects are discussed separately. (See "Omphalocele".)
Rotational anomalies, which may or may not be symptomatic, are estimated to occur in between 1 in 200 and 1 in 500 live births [1,2]. Symptomatic malrotation is estimated to occur in 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. 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
●Presentation before one year of age: 58 percent
- Warner B. Malrotation. In: Surgery of Infants and Children: Scientific Principles and Practice, Oldham KT, Colombani PM, Foglia RP (Eds), Lippincott Williams & Wilkins, Philadelphia 1997. p.1229.
- Dilley AV, Pereira J, Shi EC, et al. The radiologist says malrotation: does the surgeon operate? Pediatr Surg Int 2000; 16:45.
- Berseth CL. Disorders of the intestines and pancreas. In: Avery's Diseases of the Newborn, 7th, Taeusch WH, Ballard RA (Eds), WB Saunders, Philadelphia 1998. p.918.
- 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.
- Smith EI. Malrotation of the intestine. In: Pediatric Surgery, 4th, Welch KJ, Randolph JG, Ravich MR, et al (Eds), Year Book Medical, Chicago 1986. p.882.
- GLOVER DM, BARRY FM. Intestinal obstruction in the newborn. Ann Surg 1949; 130:480.
- Fonkalsrud E. Rotational anomalies and volvulus. In: Principles of Pediatric Surgery, O'Neill JA et al (Ed), Mosby, St. Louis 2003. p.477.
- Diaz, MCG, Reichard, K, Taylor, AA. Intestinal nonrotation in an adolescent. Pediatr Emer Care 2009; 25: 249.
- Bachur RG. Abdominal emergencies. In: Textbook of Pediatric Emergency Medicine, 5th, Fleisher GR, Ludwig S, Henretig FM (Eds), Lippincott Williams & Wilkins, Philadelphia 2006. p.1605.
- Millar AJ, Rode H, Cywes S. Malrotation and volvulus in infancy and childhood. Semin Pediatr Surg 2003; 12:229.
- 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.
- 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. Available at http://www.acr.org/~/media/ACR/Documents/AppCriteria/Diagnostic/VomitingInInfantsUpTo3MonthsOfAge.pdf. Accessed August 6, 2012.
- Berdon WE. The diagnosis of malrotation and volvulus in the older child and adult: a trap for radiologists. Pediatr Radiol 1995; 25:101.
- 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.
- 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.
- Taylor GA. CT appearance of the duodenum and mesenteric vessels in children with normal and abnormal bowel rotation. Pediatr Radiol 2011; 41:1378.
- 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.
- 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.
- Normal gut development
- Abnormal gut development
- CLINICAL PRESENTATION
- General considerations
- Duodenal obstruction
- Other presentations
- CONFIRMATORY STUDIES
- Plain radiographs
- Upper GI series
- Barium enema
- Computed tomography
- DIFFERENTIAL DIAGNOSIS
- Symptomatic malrotation
- - Laparoscopy
- Asymptomatic malrotation
- - Short gut syndrome
- - Small bowel obstruction
- Resolution of symptoms
- Recurrent volvulus
- SUMMARY AND RECOMMENDATIONS