Also known as: Malrotation with volvulus

1. Description of the problem

Malrotation refers to the incomplete rotation of the gastrointestinal tract during development. Malrotation itself may or may not be significant; however, malrotation can lead to volvulus or twisting of the small bowel around the superior mesenteric artery, ultimately causing vascular compromise and ischemia to a large portion of the GI tract.

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Clinical features

Infant with signs of small bowel obstruction

Vomiting (often bilious)

Abdominal pain

Hematochezia (from ischemia/necrosis)

Distended abdomen

Key management points

Fluid resuscitation

Surgical treatment with Ladd procedure

2. Emergency Management

Fluid resuscitation: Many infants can be severely dehydrated due to vomiting and poor intake

Early diagnosis: upper GI series

Early surgical intervention: Ladd procedure

3. Diagnosis

The diagnosis of malrotation with volvulus should be suspected in any infant with bilious vomiting.

Upper GI series – remains the gold standard, including an abnormal duodenum with the ligament of Trietz on the right side of the abdomen and a duodenal obstruction, often with a “bird’s beak” appearance

Abdominal X-ray – can be helpful in identifying a gas-less abdomen and a “double-bubble” sign similar to that seen in duodenal atresia

Other radiologic studies that can suggest malrotation include a barium enema, ultrasound, or CT scan.

4. Specific Treatment

Surgical correction of malrotation is necessary when symptomatic and associated with volvulus. It is unclear whether asymptomatic malrotation in older children needs to be surgically repaired; however, volvulus at any age is possible and thus surgical correction is often pursued.

5. Disease monitoring, follow-up and disposition

Prognosis depends on early identification and the degree of ischemia/necrosis that is present.

If no ischemia/necrosis, the outcome is excellent without long-term complications.

If ischemia/necrosis is present, resection of necrotic bowel is necessary. The resulting short bowel syndrome has significant long-term morbidity and mortality and depends on length of small bowel, function of small bowel, and presence of ileo-cecal valve, and requires active management of short bowel syndrome for the best possible outcome.


Because malrotation is likely due to abnormal gastrointestinal tract development and rotation, other anatomic abnormalities may be present.

Other Abnormalities Associated with Malrotation:

  • Intestinal atresia including tracheo-esophageal fistula

  • Abdominal wall defects

  • Imperforate anus

  • Situs inversus or other cardiac abnormalities

  • Polysplenia or asplenia.