Intestinal Malrotation

Intestinal Malrotation

No Results

No Results


During normal abdominal development, the 3 divisions of the GI tract (ie, foregut, midgut, hindgut) herniate out from the abdominal cavity, where they then undergo a 270º counterclockwise rotation around the superior mesenteric vessels. Following this rotation, the bowels return to the abdominal cavity, with fixation of the duodenojejunal loop to the left of the midline and the cecum in the right lower quadrant.

Intestinal malrotation, also known as intestinal nonrotation or incomplete rotation, refers to any variation in this rotation and fixation of the GI tract during development. Interruption of typical intestinal rotation and fixation during fetal development can occur at a wide range of locations; this leads to various acute and chronic presentations of disease. The most common type found in pediatric patients is incomplete rotation predisposing to midgut volvulus, requiring emergent operative intervention. [1, 2]

The first reports of intestinal malrotation were based on surgical and autopsy findings and occurred prior to 1900; however, the first description of the embryologic process of intestinal rotation and fixation was not published until 1898. [3] In 1923, Dott was the first to describe the relationship between embryologic intestinal rotation and surgical treatment. [4] In 1936, William E. Ladd wrote the classic article on treatment of malrotation. His surgical approach, now known as the Ladd procedure, remains the cornerstone of practice today. [5]

Intestinal malrotation occurs due to disruption of the normal embryologic development of the bowel. Understanding of normal abdominal development aids in the understanding of the etiology of the clinical findings seen with malrotation.


See the list below:


Incomplete rotation:

Incomplete fixation:

United States

Intestinal malrotation occurs in between 1 in 200 and 1 in 500 live births. [6, 7] However, most patients with malrotation are asymptomatic, with symptomatic malrotation occurring in only 1 in 6000 live births. [8] Symptoms and diagnosis may occur at any age, with some reports of prenatal diagnosis of intestinal malrotation. [9]

Malrotation may occur as an isolated anomaly or in association with other congenital anomalies; 30-62% of children with malrotation have an associated congenital anomaly. All children with diaphragmatic hernia, gastroschisis, and omphalocele have intestinal malrotation by definition. Additionally, malrotation is seen in approximately 17% of patients with duodenal atresia and 33% of patients with jejunoileal atresia. [10, 11]

Data from recent series reveal that mortality rates in adults and children operated on for intestinal malrotation range from 0-14%. Higher mortality rates are seen in cases with acute onset of midgut volvulus, delayed diagnosis, or the presence of intestinal necrosis. [12, 13, 14, 15, 1, 16] Children with other associated anomalies also have higher overall mortality rates. A report of 25 years’ experience demonstrated congenital cardiovascular disease in 27.1% of patients with intestinal malrotation; those patients had a morbidity rate of 61.1% after intestinal malrotation surgery. [17]

Male predominance is observed in neonatal presentations at a male-to-female ratio of 2:1. No sexual predilection is observed in patients older than 1 year.

Traditional teaching suggests that as many as 40% of patients with malrotation present within the first week of life, 50% in the first month, and 75% in the first year. However, more recent series have shown that malrotation is increasingly identified in adults. A series of 170 patients with intestinal malrotation diagnosed at a single institution between 1992-2009 found that 31% were infants, 21% were aged 1-18 years, and the remaining 48% were adults. [18] Although unusual, there are reports of adults presenting with total small bowel volvulus due to malrotation in adults. [19] A second series found that 42% of patients with a new diagnosis of malrotation were adults. [20]  Reports have even documented congenital malrotation presenting during pregnancy. [21, 22, 23]

Lee HC, Pickard SS, Sridhar S, Dutta S. Intestinal malrotation and catastrophic volvulus in infancy. J Emerg Med. 2012 Jul. 43(1):e49-51. [Medline]. [Full Text].

Zellos A, Zarganis D, Ypsiladis S, Chatzis D, Papaioannou G, Bartsocas C. Malrotation of the intestine and chronic volvulus as a cause of protein-losing enteropathy in infancy. Pediatrics. 2012 Feb. 129(2):e515-8. [Medline].

Mall FP. Development of the human intestine and its position in the adult. 1898. 9:197-208.

Dott NM. Anomalies of intestinal rotation: their embryology and surgical aspects: with report of 5 cases. Br J Surg. 1923. 24:251-286.

Ladd WE. Congenital Obstruction of the Duodenum in Children. N Engl J Med. 1932. 206:277-80.

Warner B. Malrotation. Oldham KT, Colombani PM, Foglia RP, eds. Surgery of Infants and Children: Scientific Principles and Practice. Philadelphia: Lippincott Williams & Wilkins; 1997. 1229.

Dilley AV, Pereira J, Shi EC, Adams S, Kern IB, Currie B. The radiologist says malrotation: does the surgeon operate?. Pediatr Surg Int. 2000. 16(1-2):45-9. [Medline].

Berseth CL. Disorders of the intestines and pancreas. Taeusch WH, Ballard RA, eds. Avery’s Diseases of the Newborn. 7th ed. Philadelphia: WB Saunders; 1998. 918.

Varetti C, Meucci D, Severi F, Di Maggio G, Bocchi C, Petraglia F, et al. Intrauterine volvulus with malrotation: prenatal diagnosis. Minerva Pediatr. 2013 Apr. 65(2):219-23. [Medline].

Smith EI. Malrotation of the intestine. Welch KJ, Randolph JG, Ravitch MN, eds. Pediatric Surgery. 4th ed. St. Louis: MO: Mosby-Year Book; 1986. Vol 2: 882-95.

Glover DM, Barry FM. Intestinal obstruction in the newborn. Ann Surg. 1949 Sep. 130(3):480-511. [Medline].

Messineo A, MacMillan JH, Palder SB, Filler RM. Clinical factors affecting mortality in children with malrotation of the intestine. J Pediatr Surg. 1992 Oct. 27(10):1343-5. [Medline].

Rescorla FJ, Shedd FJ, Grosfeld JL, Vane DW, West KW. Anomalies of intestinal rotation in childhood: analysis of 447 cases. Surgery. 1990 Oct. 108(4):710-5; discussion 715-6. [Medline].

Wallberg SV, Qvist N. Increased risk of complication in acute onset intestinal malrotation. Dan Med J. 2013. 60:A4744.

Nagdeve NG, Qureshi AM, Bhingare PD, Shinde SK. Malrotation beyond infancy. J Pediatr Surg. 2012 Nov. 47(11):2026-32. [Medline].

El-Gohary Y, Alagtal M, Gillick J. Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int. 2010 Feb. 26(2):203-6. [Medline].

Kouwenberg M, Severijnen RS, Kapusta L. Congenital cardiovascular defects in children with intestinal malrotation. Pediatr Surg Int. 2008 Mar. 24(3):257-63. [Medline]. [Full Text].

Nehra D, Goldstein AM. Intestinal malrotation: varied clinical presentation from infancy through adulthood. Surgery. 2011 Mar. 149(3):386-93. [Medline].

Kotobi H, Tan V, Lefèvre J, Duramé F, Audry G, Parc Y. Total midgut volvulus in adults with intestinal malrotation. Report of eleven patients. J Visc Surg. 2016 Nov 22. [Medline].

Durkin ET, Lund DP, Shaaban AF, Schurr MJ, Weber SM. Age-related difference in diagnosis and morbidity of intestinal malrotation. J Am Coll Surg. 2008. 206:658-663.

Yin Y, Li C, Xu C, Wu L, Deng N, Hou H, et al. Intestinal obstruction due to congenital malrotation complicating a multiple pregnancy: A rare case report. J Pak Med Assoc. 2017 Feb. 67 (2):308-310. [Medline].

Gião Antunes AS, Peixe B, Guerreiro H. Midgut Volvulus as a Complication of Intestinal Malrotation in Pregnancy. ACG Case Rep J. 2017 Jan 18. 4:e9. [Medline].

Esterson YB, Villani R, Dela Cruz RA, Friedman B, Grimaldi GM. Small bowel volvulus in pregnancy with associated superior mesenteric artery occlusion. Clin Imaging. 2017 Mar – Apr. 42:228-231. [Medline].

Wanjari AK, Deshmukh AJ, Tayde PS, Lonkar Y. Midgut malrotation with chronic abdominal pain. N Am J Med Sci. 2012 Apr. 4(4):196-8. [Medline]. [Full Text].

Spitz L, Orr JD, Harries JT. Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child. 1983 May. 58(5):383-5. [Medline].

Applegate KE, Anderson JM, Klatte EC. Intestinal malrotation in children: a problem-solving approach to the upper gastrointestinal series. Radiographics. 2006 Sep-Oct. 26(5):1485-500. [Medline].

Lin JN, Lou CC, Wang KL. Intestinal malrotation and midgut volvulus: a 15-year review. J Formos Med Assoc. 1995 Apr. 94(4):178-81. [Medline].

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 May. 38(5):518-28. [Medline].

Fonio P, Coppolino F, Russo A, D’Andrea A, Giannattasio A, Reginelli A. Ultrasonography (US) in the assessment of pediatric non traumatic gastrointestinal emergencies. Crit Ultrasound J. 2013 Jul 15. 5 Suppl 1:S12. [Medline].

Alehossein M, Abdi S, Pourgholami M, Naseri M, Salamati P. Diagnostic accuracy of ultrasound in determining the cause of bilious vomiting in neonates. Iran J Radiol. 2012 Nov. 9(4):190-4. [Medline].

Zhang W, Sun H, Luo F. The efficiency of sonography in diagnosing volvulus in neonates with suspected intestinal malrotation. Medicine (Baltimore). 2017 Oct. 96 (42):e8287. [Medline].

Hennessey I, John R, Gent R, Goh DW. Utility of sonographic assessment of the position of the third part of the duodenum using water instillation in intestinal malrotation: a single-center retrospective audit. Pediatr Radiol. 2014 Apr. 44(4):387-91. [Medline].

Quail MA. Question 2. Is Doppler ultrasound superior to upper gastrointestinal contrast study for the diagnosis of malrotation?. Arch Dis Child. 2011 Mar. 96(3):317-8. [Medline].

Shahverdi E, Morshedi M, Allahverdi Khani M, Baradaran Jamili M, Shafizadeh Barmi F. Utility of the CT Scan in Diagnosing Midgut Volvulus in Patients with Chronic Abdominal Pain. Case Rep Surg. 2017. 2017:1079192. [Medline].

Catania VD, Lauriti G, Pierro A, Zani A. Open versus laparoscopic approach for intestinal malrotation in infants and children: a systematic review and meta-analysis. Pediatr Surg Int. 2016 Dec. 32 (12):1157-1164. [Medline].

Arnaud AP, Suply E, Eaton S, Blackburn SC, Giuliani S, Curry JI, et al. Laparoscopic Ladd’s procedure for malrotation in infants and children is still a controversial approach. J Pediatr Surg. 2018 Oct 28. [Medline].

Dassinger MS, Smith SD. Chapter 86. Disorders of Intestinal Rotation and Fixation. Coran A, Adzick NS, Krummel TM, et al, eds. Pediatric Surgery. 7th ed. Elsevier; 837-51.

Badea R, Al Hajjar N, Andreica V, Procopet B, Caraiani C, Tamas-Szora A. Appendicitis associated with intestinal malrotation: imaging diagnosis features. Case report. Med Ultrason. 2012 Jun. 14(2):164-7. [Medline].

Tsao KJ, St Peter SD, Valusek PA, Keckler SJ, Sharp S, Holcomb GW 3rd. 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. [Medline].

Kinlin C, Shawyer AC. The surgical management of malrotation: A Canadian Association of Pediatric Surgeons survey. J Pediatr Surg. 2017 Jan 28. [Medline].

Huntington JT, Lopez JJ, Mahida JB, Ambeba EJ, Asti L, Deans KJ, et al. Comparing laparoscopic versus open Ladd’s procedure in pediatric patients. J Pediatr Surg. 2016 Oct 30. [Medline].

Draus JM Jr, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg. 2007 Jul. 73(7):693-6. [Medline].

Palanivelu C, Rangarajan M, Shetty AR, Jani K. Intestinal malrotation with midgut volvulus presenting as acute abdomen in children: value of diagnostic and therapeutic laparoscopy. J Laparoendosc Adv Surg Tech A. 2007 Aug. 17(4):490-2. [Medline].

Stanfill AB, Pearl RH, Kalvakuri K, Wallace LJ, Vegunta RK. Laparoscopic Ladd’s procedure: treatment of choice for midgut malrotation in infants and children. J Laparoendosc Adv Surg Tech A. 2010 May. 20(4):369-72. [Medline].

Ferrero L, Ben Ahmed Y, Philippe P, Reinberg O, Lacreuse I, Schneider A, et al. Intestinal Malrotation and Volvulus in Neonates: Laparoscopy Versus Open Laparotomy. J Laparoendosc Adv Surg Tech A. 2017 Jan 5. [Medline].

Vassaur J, Vassaur H, Buckley FP 3rd. Single-incision laparoscopic Ladd’s procedure for intestinal malrotation. JSLS. 2014 Jan-Mar. 18(1):132-5. [Medline].

Ooms N, Matthyssens LE, Draaisma JM, de Blaauw I, Wijnen MH. Laparoscopic Treatment of Intestinal Malrotation in Children. Eur J Pediatr Surg. 2015 Jun 18. [Medline].

Newman B, Koppolu R, Murphy D, Sylvester K. Heterotaxy syndromes and abnormal bowel rotation. Pediatr Radiol. 2014 May. 44(5):542-51. [Medline].

Pockett CR, Dicken B, Rebeyka IM, Ross DB, Ryerson LM. Heterotaxy syndrome: is a prophylactic Ladd procedure necessary in asymptomatic patients?. Pediatr Cardiol. 2013 Jan. 34(1):59-63. [Medline].

Cullis PS, Siminas S, Losty PD. Is Screening of Intestinal Foregut Anatomy in Heterotaxy Patients Really Necessary?: A Systematic Review in Search of the Evidence. Ann Surg. 2016 Dec. 264 (6):1156-1161. [Medline].

Elsinga RM, Roze E, Van Braeckel KN, Hulscher JB, Bos AF. Motor and cognitive outcome at school age of children with surgically treated intestinal obstructions in the neonatal period. Early Hum Dev. 2013 Mar. 89(3):181-5. [Medline].

El-Gohary Y, Alagtal M, Gillick J. Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int. 2010 Feb. 26(2):203-6. [Medline].

Feitz R, Vos A. Malrotation: the postoperative period. J Pediatr Surg. 1997 Sep. 32(9):1322-4. [Medline].

Ai VH, Lam WW, Cheng W. CT appearance of midgut volvulus with malrotation in a young infant. ClinRadiol. Oct 1999. 54(10):687-9.

Bass KD, Rothenberg SS, Chang JH. Laparoscopic Ladd’s procedure in infants with malrotation. J Pediatr Surg. 1998 Feb. 33(2):279-81. [Medline].

Chao HC, Kong MS, Chen JY, Lin SJ, Lin JN. Sonographic features related to volvulus in neonatal intestinal malrotation. J Ultrasound Med. 2000 Jun. 19(6):371-6. [Medline].

Estrada RL. Thomas CC, ed. Anomalies of Intestinal Rotation and Fixation. Springfield, IL: 1958.

Guzzetta PC, Anderson KD, Eichelberger MR. General Surgery. Avery GB, Fletcher MA, MacDonald MG, eds. Neonatology: Pathophysiology and Management of the Newborn. Philadelphia, PA: Lippincott Williams & Wilkins; 1994. 931-2.

Howell CG, Vozza F, Shaw S, Robinson M, Srouji MN, Krasna I. Malrotation, malnutrition, and ischemic bowel disease. J Pediatr Surg. 1982 Oct. 17(5):469-73. [Medline].

Irish MS, Pearl RH, Caty MG, Glick PL. The approach to common abdominal diagnosis in infants and children. Pediatr Clin North Am. 1998 Aug. 45(4):729-72. [Medline].

Janik JS, Ein SH. Normal intestinal rotation with non-fixation: a cause of chronic abdominal pain. J Pediatr Surg. 1979 Dec. 14(6):670-4. [Medline].

Kamal IM. Defusing the intra-abdominal ticking bomb: intestinal malrotation in children. CMAJ. 2000 May 2. 162(9):1315-7. [Medline].

Kullendorff CM, Mikaelsson C, Ivancev K. Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Paediatr Scand. 1985 Mar. 74(2):296-9. [Medline].

Kumar D, Brereton RJ, Spitz L, Hall CM. Gastro-oesophageal reflux and intestinal malrotation in children. Br J Surg. 1988 Jun. 75(6):533-5. [Medline].

[Guideline] Ladd WE. Surgical Diseases of the Alimentary Tract in Infants. N Engl J Med. 1936. 215:705-8.

Lee HC, Pickard SS, Sridhar S, Dutta S. Intestinal malrotation and catastrophic volvulus in infancy. J Emerg Med. 2012 Jul. 43(1):e49-51. [Medline]. [Full Text].

Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg. 1997 Aug. 185(2):172-6. [Medline].

Spigland N, Brandt ML, Yazbeck S. Malrotation presenting beyond the neonatal period. J Pediatr Surg. 1990 Nov. 25(11):1139-42. [Medline].

Denis D Bensard, MD, FACS, FAAP Director of Pediatric Surgery and Trauma, Attending Surgeon in Adult and Pediatric Acute Care Surgery, Attending Surgeon in Adult and Pediatric Surgical Critical Care, Denver Health Medical Center; Professor of Surgery, University of Colorado School of Medicine; Associate Program Director, General Surgery Residency, Attending Surgeon, Children’s Hospital Colorado

Denis D Bensard, MD, FACS, FAAP is a member of the following medical societies: American Association for the Surgery of Trauma, Alpha Omega Alpha, Society of American Gastrointestinal and Endoscopic Surgeons, Southwestern Surgical Congress, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, Association for Academic Surgery, Society of University Surgeons

Disclosure: Nothing to disclose.

Shannon N Acker, MD Resident Physician, Department of Surgery, University of Colorado School of Medicine

Disclosure: Nothing to disclose.

Ann M Kulungowski, MD Assistant Professor of Pediatric Surgery, University of Colorado School of Medicine

Ann M Kulungowski, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Mary L Windle, PharmD Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

David A Piccoli, MD Chief of Pediatric Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia; Professor, University of Pennsylvania School of Medicine

David A Piccoli, MD is a member of the following medical societies: American Association for the Study of Liver Diseases, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition

Disclosure: Nothing to disclose.

Carmen Cuffari, MD Associate Professor, Department of Pediatrics, Division of Gastroenterology/Nutrition, Johns Hopkins University School of Medicine

Carmen Cuffari, MD is a member of the following medical societies: American College of Gastroenterology, American Gastroenterological Association, North American Society for Pediatric Gastroenterology, Hepatology and Nutrition, Royal College of Physicians and Surgeons of Canada

Disclosure: Received honoraria from Prometheus Laboratories for speaking and teaching; Received honoraria from Abbott Nutritionals for speaking and teaching. for: Abbott Nutritional, Abbvie, speakers’ bureau.

Jeffrey J Du Bois, MD Chief of Children’s Surgical Services, Division of Pediatric Surgery, Kaiser Permanente, Women and Children’s Center, Roseville Medical Center

Jeffrey J Du Bois, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American College of Surgeons, American Pediatric Surgical Association, California Medical Association

Disclosure: Nothing to disclose.

Robyn Hatley, MD Professor, Departments of Surgery and Pediatrics, Medical College of Georgia

Robyn Hatley, MD is a member of the following medical societies: American Academy of Pediatrics, American College of Surgeons, American Medical Association, and American Pediatric Surgical Association

Disclosure: Nothing to disclose.

Anjali Parish, MD Assistant Professor of Pediatrics, Department of Neonatology, Medical College of Georgia

Anjali Parish, MD is a member of the following medical societies: American Academy of Pediatrics and American Medical Association

Disclosure: Nothing to disclose.

Intestinal Malrotation

Research & References of Intestinal Malrotation|A&C Accounting And Tax Services

Leave a Reply