Malrotation - Quick Consult
Last Updated / Reviewed: June 2022

Definition
Key History
Key Physical Exam
Risk Factors For Malrotation
Differential Diagnosis
Diagnostic Testing
Clinical Risk and Safety Pearls
Treatment
Complications

Definition

Intestinal Malrotation occurs when there is an abnormal or incomplete rotation of the embryonic gut around the superior mesenteric artery. This malrotation will then predispose the patient to developing a volvulus, which is when a bowel loop abnormally twists onto itself. This can lead to intestinal obstruction, bowel necrosis, sepsis and death.

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Key History

  • Vomiting - most frequent sign in infants
  • Bilious emesis in the infant is key; may be non-bilious as well
  • Baby presenting with a bilious stained bib/“onesie” should put the practitioner on high alert
  • Possible history of formula intolerance or spitting up
  • Abdominal pain - diffuse, acute or chronic - most frequent sign in older children and adults
  • Patient may be acting normally early in addition to vomiting; later may be irritable or lethargic
  • Abdomen reported as appearing large or full
  • Bloody stools
  • +/- diarrhea
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Key Physical Exam

  • May appear well early; later will likely appear ill or even “shocky”
  • Older children may have “wasted” appearance
  • Likely will have tachycardia and/or tachypnea, but VS could be WNL early
  • Abdominal tenderness
  • Abdominal rigidity/peritonitis
  • Abdominal distension
  • Guaiac + stools or currant jelly stools
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Risk Factors for Malrotation

  • Approximately 1/3 of patients under 1 year of age
  • Seen in adolescents and adults, either asymptomatic or with a chronic abdominal pain history
  • Up to 60%-70% of patients will have an associated gastrointestinal congenital anomaly
  • Concurrent gastrointestinal anomalies, including: gastroschisis/omphalocele, congenital diaphragmatic hernia, Hirschsprung’s disease, intestinal atresia, GERD, and other intestinal and anorectal malformations
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Differential Diagnosis

  • Feeding Intolerance
  • Gastroenteritis
  • Necrotizing enterocolitis (neonates)
  • Appendicitis
  • Intussusception
  • Recurrent or functional abdominal pain (older children)
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Diagnostic Testing

  1. Imaging
  • Upper GI Series: Gold standard to rule in a malrotation/volvulus. Reveals abnormal anatomic position of the ligament of Trietz and likely reveals a coiled spring or corkscrew appearance of the small bowel.
  • Abdominal X-Rays: Will often yield nonspecific findings; should not be used to rule in or rule out a malrotation. May be helpful in diagnosing a bowel obstruction. It is rare, but you may see a “double bubble sign,” indicating an obstruction in the duodenum; you may possibly see bowel loops obscuring the liver border.
  • Ultrasound: Can aid in ruling in a malrotation, but does not rule out a malrotation.
  • Barium Enema: Can be misleading because the cecum in neonates can be mobile and may appear to be in the normal RLQ position. This is not a reliable test to rule in a malrotation; may see higher false + results.
  1. Lab
  • Complete Blood Count: Not diagnostic for malrotation. May aid the practitioner in determining the degree of stress and/or infectious component (peritonitis, sepsis, etc.) that may be seen later in malrotation with midgut volvulus.
  • Complete Metabolic Panel: Not diagnostic for malrotation. May aid the practitioner in assessing degree of dehydration/acidosis that may be seen later in a patient with a malrotation and midgut volvulus.
  • Stool Guaiac
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Clinical Risk and Safety Pearls

  • The practitioner should be on high alert for a malrotation with midgut volvulus on ANY infant with bilious emesis.
  • Maintain an index of suspicion even with older children, as their presentation may be more chronic or sub-acute with waxing and waning episodes of belly pain and vomiting.
  • Once you entertain this diagnosis, early mobilization of radiology for an upper GI series and preparation for surgery should be immediate priorities.
  • These patients can become quite ill and septic from bowel necrosis. Maintaining hemodynamic stability is crucial. If sepsis is considered, early administration of antibiotics is necessary.

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Treatment

  • Stabilize the patient with IVF, NG tube for bowel decompression, hemodynamic support, antibiotics if sepsis or peritonitis is suggested. Type and cross match, blood replacement for symptomatic/significant anemia.
  • Immediate mobilization of radiology and surgery.
  • The definitive treatment for malrotation is surgical intervention, specifically the Ladd’s procedure. This will reposition the intestines within the abdomen and decrease the incidence of further torsing of the intestinal base.

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Complications

  • Bowel wall necrosis from torsing of the gut around the superior mesenteric artery
  • Sepsis due to the loss of bowel wall integrity and peritonitis
  • Anemia from blood loss from the gut and possibly overwhelming infection
  • Short Gut Syndrome occurs after loss of bowel and ensuing bowel resection
  • Bowel obstruction, which is possible after any intra-abdominal surgery
  • Reoccurrence of developing a volvulus after undergoing Ladd’s procedure has been reported to be up to 7%-8%
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  1. Aboagye J, Goldstein SD, Salazar JH, et al. Age at presentation of common pediatric surgical conditions: Reexamining dogma. J Pediatr Surg. 2014;49(6):995-999.
  2. 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(1):1783-1790.
  3. Nehra D, Goldstein AM. Intestinal malrotation: Varied clinical presentation from infancy through adulthood. Surgery. 2011;149(3):386-393.
  4. Shalaby MS, Kuti K, Walker G. Intestinal malrotation and volvulus in infants and children. BMJ. 2013;347:f6949.

This is intended solely as reference material and is not a recommendation for any specific patient. The practitioner must rely upon his or her own professional judgment and medical decision-making to determine whether it is relevant in a particular case. Materials are derived from medical and nursing texts, medical literature and national guidelines and should not be considered complete or authoritative. Users must rely on specific patient presentation, experience and judgment when utilizing any of the information contained herein relative to an actual patient.
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