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Intestinal malrotation is a congenital condition that can have disastrous, potentially fatal sequelae. It is believed to occur in approximately 1 in 500 live births.1 Malrotation results from failure or interruption of the normal process of rotation, positioning and fixation of the embryonic gut during development, so that it is has a propensity to twist or volve about its mesentery. This interrupts its blood supply, which can lead to infarction of extensive amounts of gut. Malrotated gut is also prone to obstruction by congenital bands of fibrous tissue (Ladd’s bands) traversing the bowel externally. The majority of patients with this condition present in early infancy with bilious vomiting; however, if the diagnosis is not considered and prompt action taken to confirm or exclude malrotation, the consequences can be catastrophic.
WHAT IS THE SIGNIFICANCE OF BILIOUS VOMITING?
Bilious vomiting in childhood is a symptom that must be taken seriously because it can indicate serious gut pathology. A diagnosis of malrotation should always be considered in a child of any age with bile stained vomiting. In malrotation this is due to bowel obstruction distal to the ampulla of Vater where the biliary system joins the bowel. Other causes of high intestinal obstruction, such as duodenal stenosis, duodenal atresia or proximal jejunal atresia, are accompanied by bilious vomiting. Non-obstructive causes of bilious vomiting include intestinal ileus secondary to electrolyte imbalance and gastrointestinal infection.2
Some knowledge of the complex embryology of the gut is useful in order to understand the significance of malrotation. Formation of the embryonic gut begins by the 2nd week of gestation. The gut develops from the yolk sac and becomes internalised forming a single short tube lined with endoderm by the 3–4th weeks, joined in its mid-portion to the yolk sac by the vitellointestinal duct. The surrounding layers of mesoderm form the parietal peritoneum and …
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