Intestinal malrotation

Intestinal malrotation
Classification and external resources
Specialty	medical genetics
ICD-10	Q43.3
ICD-9-CM	751.4
OMIM	193250
eMedicine	ped/1200
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Intestinal malrotation is a congenital anomaly of rotation of the midgut (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result:

Patients (often infants) present acutely with midgut volvulus, manifested by bilious vomiting, crampy abdominal pain, abdominal distention, and the passage of blood and mucus in their stools. Patients with chronic, uncorrected malrotation can have recurrent abdominal pain and vomiting.

Malrotation can also be asymptomatic.

This can lead to a number of disease manifestations such as:

The exact causes are not known. It is not associated with a particular gene, but there is some evidence of recurrence in families.

With acutely ill patients, consider emergency surgery laparotomy if there is a high index of suspicion.

Plain radiography may demonstrate signs of duodenal obstruction with dilatation of the proximal duodenum and stomach but it is often non-specific. Upper gastrointestinal series is the modality of choice for the evaluation of malrotation as it will show an abnormal position of the duodeno-jejunal flexure (ligament of Treitz). In cases of malrotation complicated with volvulus, it demonstrates a corkscrew appearance of the distal duodenum and jejunum. In cases of obstructing Ladd bands, it will reveal a duodenal obstruction.

In equivocal cases, contrast enema, may be helpful by showing the caecum at an abnormal location.

It is usually discovered near birth, but in some cases is not discovered until adulthood. In adults, the "whirlpool sign" of the superior mesenteric artery can be useful in identifying malrotation.

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Wikipedia