Intestinal malrotation comprises a spectrum of rotational abnormalities that may lead to a variety of clinical symptoms in neonates, infants, and older children .Intestinal malrotation is a congenital anomaly of rotation of the midgut (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result of which the

• Small intestine found predominantly on the right side of the abdomen
• The cecum is displaced into the right hypochondrium
• Fibrous bands (of Ladd)course over the vertical portion of the duodenum (DII), causing intestinal obstruction.
• The small intestinehas an unusually narrow base, and therefore the midgut is prone to volvulus.

Malrotation can be asymptomatic. 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.

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 [1]. In adults, the “whirlpool sign” of the superior mesenteric artery can be useful in identifying malrotation [2]. 

Treatment involves resuscitation of the patient with fluids to stabilize them before surgery. Surgical technique is known as “Ladd’s procedure”  [3].

• Correcting the volvulus (counterclockwise rotation of the bowel)
• Dividing the fibrous LADD’s bands over the duodenum.
• Straightening of the duodenum.
• Widening the mesenteric pedicle by separation of the duodenum and cecum (Duodeno-colic isthumus)

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, to avoid confusion in the future.

The excellent feasibility of laparoscopy in pediatric gastrointestinal surgery has led to a dramatic increase in the scope of procedures performed laparoscopically. There is evidence that laparoscopic Ladd’s procedure in pediatric age group is safe and gives similar results as in open procedure [4].

References:

1. Dietz DW, Walsh RM, Grundfest-Broniatowski S, Lavery IC, Fazio VW, Vogt DP (2002). “Intestinal malrotation: a rare but important cause of bowel obstruction in adults”. Dis. Colon Rectum. 45 (10): 1381–6.
2. Yeh WC, Wang HP, Chen C, Wang HH, Wu MS, Lin JT (1999). “Preoperative sonographic diagnosis of midgut malrotation with volvulus in adults: the “whirlpool” sign”. Journal of Clinical Ultrasound. 27(5): 279–83.
3.  Ladd WE (1936). “Surgical Diseases of the Alimentary Tract in Infants”. N Engl J Med. 215: 705–8.
4. Bass  KD, Rothenberg SS, Chang JH (1998). “Laparoscopic Ladd’s procedure in infants with malrotation”. J. Pediatr. Surg. 33 (2): 279–81.