Intestinal Malrotation

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Background

Many authors define intestinal malrotation as intestinal nonrotation or incomplete rotation around the superior mesenteric artery (SMA). It involves anomalies of intestinal fixation as well. 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, which can result in short-bowel syndrome or even death.[1, 2]

Malrotation was reported prior to the 1900s. During the 20th century, understanding of the embryology and anatomy of malrotation became more complete, along with changes in surgical approaches to the problems. In 1936, William E. Ladd wrote the classic article on treatment of malrotation, and his surgical approach (ie, Ladd procedure) remains the cornerstone of practice today.[3]

Pathophysiology

See Causes.

Epidemiology

Frequency

United States

Intestinal malrotation occurs at a rate of 1 in 500 live births. Most infants with gastroschisis, omphalocele, or congenital diaphragmatic hernia present with intestinal malrotation. Approximately 50% of patients with duodenal atresia and 33% of patients with jejunoileal atresia have a malrotation as well. Also, intestinal malrotation occurs in association with Hirschsprung disease, gastroesophageal reflux, intussusception, persistent cloaca, anorectal malformations (imperforate anus), and extrahepatic anomalies.

Mortality/Morbidity

Younger patients have higher rates of morbidity and mortality. In infants, the mortality rate ranges from 2-24%. The presence of necrotic bowel at surgery increases the mortality rate by 25 times for infants, and the presence of other anomalies increases the risk by 22 times. 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.[4]

Sex

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.

Age

As many as 40% of patients with malrotation present within the first week of life. This condition is diagnosed in 50% of patients by age 1 month and is diagnosed in 75% by age 1 year. The remaining 25% of patients present after age 1 year and into late adulthood; many are recognized intraoperatively during other procedures or at autopsy.

History

The history of present illness varies in patients with intestinal malrotation according to acute or chronic presentation, as well as according to type of rotational defect.

Physical

Physical examination findings may vary depending on the type of rotational defect. Acute and chronic presentations also differ.

Causes

The cause of intestinal malrotation is disruption in the normal embryological development of the bowel. Clinical features depend on the stage of disruption and are discussed as follows. A full understanding of normal development aids in understanding the etiology of malrotation.

Laboratory Studies

The following studies may be indicated in patients with intestinal malrotation:

Imaging Studies

The following imaging studies may be helpful:

Procedures

The following procedures may be helpful:

Medical Care

Medical care of intestinal malrotation is directed toward stabilizing the patient.

Surgical Care

The Ladd procedure remains the cornerstone of surgical treatment for malrotation today. Prior to William Ladd's publication in 1936,[3] surgical treatment for malrotation with or without volvulus had a mortality rate higher than 90%. In fact, at Ladd's own institution, the mortality rate was 100% before the development of his new technique. A classic Ladd procedure is described as reduction of volvulus (if present), division of mesenteric bands, placement of small bowel on the right and large bowel on the left of the abdomen, and appendectomy. Published reports for laparoscopic Ladd procedure are now appearing in the literature as well.

Consultations

Consultations with the following specialists may be helpful:

Diet

Maintain all patients NPO until bowel function is recovered after surgery.

Dopamine (Intropin)

Clinical Context:  Stimulates both adrenergic and dopaminergic receptors. Hemodynamic effect is dependent on the dose. Lower doses predominantly stimulate dopaminergic receptors that in turn produce renal and mesenteric vasodilation. Cardiac stimulation and renal vasodilation are produced by higher doses.

Class Summary

These agents are the first-line therapy because of their possible effects to increase splanchnic blood flow.

Further Inpatient Care

Immediate postoperative care in patients with intestinal malrotation includes the following:

Aspects of a return of bowel function include the following:

Aspects of nutrition include the following:

Resuming GI feedings may include the following:

Further Outpatient Care

Aspects of growth and nutrition include the following:

Development includes the following:

Transfer

Immediately transfer any patient suspected of having malrotation with or without midgut volvulus to a facility with pediatric surgical support. Furthermore, any child with bilious emesis is assumed to have a surgical problem until proven otherwise.

Complications

Complications include the following:

Prognosis

In general, older children do better than infants. The presence of midgut volvulus prolongs hospitalization, and prognosis is based on how much bowel is preserved. Because of the morbidity involved with midgut volvulus, immediate referral for pediatric surgical care can improve outcome more than any other medical intervention.

Author

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

Disclosure: Nothing to disclose.

Specialty Editors

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

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

Disclosure: Nothing to disclose.

Steven M Schwarz, MD, FAAP, FACN, AGAF, Professor of Pediatrics, Children's Hospital at Downstate, State University of New York Downstate Medical Center

Disclosure: Curemark, LLC Consulting fee Board membership; Centocor, Inc. Grant/research funds Independent contractor; Johnson & Johnson, Inc. Grant/research funds Independent contractor

Chief Editor

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

Disclosure: Nothing to disclose.

Additional Contributors

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.

References

  1. Lee HC, Pickard SS, Sridhar S, Dutta S. Intestinal malrotation and catastrophic volvulus in infancy. J Emerg Med. Jul 2012;43(1):e49-51. [View Abstract]
  2. 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. Feb 2012;129(2):e515-8. [View Abstract]
  3. [Guideline] Ladd WE. Surgical Diseases of the Alimentary Tract in Infants. N Engl J Med. 1936;215:705-8.
  4. Kouwenberg M, Severijnen RS, Kapusta L. Congenital cardiovascular defects in children with intestinal malrotation. Pediatr Surg Int. Mar 2008;24(3):257-63. [View Abstract]
  5. Wanjari AK, Deshmukh AJ, Tayde PS, Lonkar Y. Midgut malrotation with chronic abdominal pain. N Am J Med Sci. Apr 2012;4(4):196-8. [View Abstract]
  6. Spitz L, Orr JD, Harries JT. Obstructive jaundice secondary to chronic midgut volvulus. Arch Dis Child. May 1983;58(5):383-5. [View Abstract]
  7. 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. Jun 2012;14(2):164-7. [View Abstract]
  8. Draus JM Jr, Foley DS, Bond SJ. Laparoscopic Ladd procedure: a minimally invasive approach to malrotation without midgut volvulus. Am Surg. Jul 2007;73(7):693-6. [View Abstract]
  9. 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. Aug 2007;17(4):490-2. [View Abstract]
  10. 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. Mar 11 2010;[View Abstract]
  11. 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. Oct 18 2012;[View Abstract]
  12. El-Gohary Y, Alagtal M, Gillick J. Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int. Sep 16 2009;[View Abstract]
  13. El-Gohary Y, Alagtal M, Gillick J. Long-term complications following operative intervention for intestinal malrotation: a 10-year review. Pediatr Surg Int. Feb 2010;26(2):203-6. [View Abstract]
  14. Feitz R, Vos A. Malrotation: the postoperative period. J Pediatr Surg. Sep 1997;32(9):1322-4. [View Abstract]
  15. Ai VH, Lam WW, Cheng W, et al. CT appearance of midgut volvulus with malrotation in a young infant. Clin Radiol. Oct 1999;54(10):687-9. [View Abstract]
  16. Bass KD, Rothenberg SS, Chang JH. Laparoscopic Ladd's procedure in infants with malrotation. J Pediatr Surg. Feb 1998;33(2):279-81. [View Abstract]
  17. Chao HC, Kong MS, Chen JY, et al. Sonographic features related to volvulus in neonatal intestinal malrotation. J Ultrasound Med. Jun 2000;19(6):371-6. [View Abstract]
  18. Estrada RL. Anomalies of Intestinal Rotation and Fixation. Springfield, IL: Charles C Thomas; 1958.
  19. Guzzetta PC, Anderson KD, Eichelberger MR. General Surgery. In: Avery GB, Fletcher MA, MacDonald MG, eds. Neonatology: Pathophysiology and Management of the Newborn. Philadelphia, PA: Lippincott Williams & Wilkins; 1994:931-2.
  20. Howell CG, Vozza F, Shaw S, et al. Malrotation, malnutrition, and ischemic bowel disease. J Pediatr Surg. Oct 1982;17(5):469-73. [View Abstract]
  21. Irish MS, Pearl RH, Caty MG, Glick PL. The approach to common abdominal diagnosis in infants and children. Pediatr Clin North Am. Aug 1998;45(4):729-72. [View Abstract]
  22. Janik JS, Ein SH. Normal intestinal rotation with non-fixation: a cause of chronic abdominal pain. J Pediatr Surg. Dec 1979;14(6):670-4. [View Abstract]
  23. Kamal IM. Defusing the intra-abdominal ticking bomb: intestinal malrotation in children. CMAJ. May 2 2000;162(9):1315-7. [View Abstract]
  24. Kullendorff CM, Mikaelsson C, Ivancev K. Malrotation in children with symptoms of gastrointestinal allergy and psychosomatic abdominal pain. Acta Paediatr Scand. Mar 1985;74(2):296-9. [View Abstract]
  25. Kumar D, Brereton RJ, Spitz L, Hall CM. Gastro-oesophageal reflux and intestinal malrotation in children. Br J Surg. Jun 1988;75(6):533-5. [View Abstract]
  26. Ladd WE. Congenital Obstruction of the Duodenum in Children. N Engl J Med. 1932;206:277-80.
  27. Mazziotti MV, Strasberg SM, Langer JC. Intestinal rotation abnormalities without volvulus: the role of laparoscopy. J Am Coll Surg. Aug 1997;185(2):172-6. [View Abstract]
  28. Smith EI. Malrotation of the intestine. In: Welch KJ, Randolph JG, Ravitch MN, et al, eds. Pediatric Surgery. Vol 2. 4th ed. St. Louis, MO: Mosby-Year Book; 1986:882-95.
  29. Spigland N, Brandt ML, Yazbeck S. Malrotation presenting beyond the neonatal period. J Pediatr Surg. Nov 1990;25(11):1139-42. [View Abstract]

Normal rotation of the intestines during development. The superior mesenteric artery (SMA) is the axis. The duodenojejunal loop (red arrow) begins superior to the SMA, and the cecocolic loop (green arrow) begins inferior to the SMA.

In this upper GI series with abnormal results, the duodenum does not cross the midline, and the small bowel is present only in the right side of the abdomen.

These 2 lower GI series show the cecum (arrows) in the right upper quadrant, indicative of malrotation.

This patient had malrotation with midgut volvulus. The gut is darkened in color because of ischemia.

Normal rotation of the intestines during development. The superior mesenteric artery (SMA) is the axis. The duodenojejunal loop (red arrow) begins superior to the SMA, and the cecocolic loop (green arrow) begins inferior to the SMA.

In this upper GI series with abnormal results, the duodenum does not cross the midline, and the small bowel is present only in the right side of the abdomen.

These 2 lower GI series show the cecum (arrows) in the right upper quadrant, indicative of malrotation.

This patient had malrotation with midgut volvulus. The gut is darkened in color because of ischemia.