Intestinal malrotation

Intestinal malrotation is a congenital anomaly . The children are born with abnormal position of gut as a result of incomplete or non rotation of gut during embryogenesis.This can predispose to midgut volvulus(strangulation of malrotated gut) and internal hernias, and can result in life-threatening complications. Although some individuals live their entire life with malrotated bowel without symptoms.

The abnormal rotation of gut is charecterised with:

  • The small bowel is present on the right side of the abdomen
  • The cecum is displaced to epigastrium – right hypochondrium
  • The ligament of Treitz is displaced inferiorly and rightward
  • Fibrous bands (of Ladd) course over the vertical portion of the duodenum (DII), causing intestinal obstruction.
  • The small intestine have an unusually narrow base, and therefore the midgut is prone to volvulus and cause intestinal ischemia.

Signs and symptoms

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.

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

  • Acute midgut volvulus
  • Chronic midgut volvulus
  • Acute duodenal obstruction
  • Chronic duodenal osbstruction
  • Internal herniation
  • Superior mesenteric artery syndrome

Diagnosis

Children(often infants) presenting with clinical sign and symptoms should undergo following tests:

Plain radiography may demonstrate signs of duodenal obstruction with dilatation of the proximal duodenum and stomach.

Ultrasound abdomen can define vascular flow through the superior mesenteric vessels. Whirlpool sign and reversal of position of superior messenteric vessels can be useful in identifying malrotation.

Upper gastrointestinal contrast study 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.

  

Contrast enema, may be helpful by showing the caecum at an abnormal location.

Treatment

Treatment is surgical and done after resuscitating the patient with fluids .
With this condition the appendix is often on the wrong side of the body and therefore removed as a precautionary measure during the surgical procedure.
Surgical technique is known as “Ladd’s procedure