Malrotation
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.
Pathophysiology
Because malrotation is likely due to abnormal gastrointestinal tract development and rotation, other anatomic abnormalities may be present.
Other Abnormalities Associated with Malrotation:
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Intestinal atresia including tracheo-esophageal fistula
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Abdominal wall defects
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Imperforate anus
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Situs inversus or other cardiac abnormalities
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Polysplenia or asplenia.
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