- the small intestine is found predominantly on the right side of the abdomen
- the cecum is displaced (from its usual position in the right lower quadrant) into the 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 has an unusually narrow base, and therefore the midgut is prone to volvulus (a twisting that can obstruct the mesenteric blood vessels 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.
This can lead to a number of disease manifestations such as:
- Acute midgut volvulus
- Chronic midgut volvulus
- Acute duodenal obstruction
- Chronic duodenal obstruction
- Internal herniation
- Superior mesenteric artery syndrome
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.
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.
Treatment is possible and these are the steps taken: Resuscitate the patient with fluids to stabilize them before surgically
- correcting the malrotation (counterclockwise rotation of the bowel),
- cutting the fibrous bands over the duodenum,
- widening the mesenteric pedicle by separation of the duodenum and cecum.
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.
One surgical technique is known as "Ladd's procedure", after Dr. William Ladd. Long term research on the Ladd procedure shows that even after the procedure, patients are susceptible to have complaints and might need further surgery.
- Situs inversus, a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions.
- Stalker HJ, Chitayat D (1992). "Familial intestinal malrotation with midgut volvulus and facial anomalies: a disorder involving a gene controlling the normal gut rotation?". Am. J. Med. Genet. 44 (1): 46–7. doi:10.1002/ajmg.1320440111. PMID 1519649.
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- Ladd WE (1936). "Surgical Diseases of the Alimentary Tract in Infants". N Engl J Med. 215 (16): 705–8. doi:10.1056/NEJM193610152151604.
- Bass KD, Rothenberg SS, Chang JH (1998). "Laparoscopic Ladd's procedure in infants with malrotation". J. Pediatr. Surg. 33 (2): 279–81. doi:10.1016/S0022-3468(98)90447-X. PMID 9498402.
- Murphy FL, Sparnon AL (2006-04-01). "Long-term complications following intestinal malrotation and the Ladd's procedure: a 15 year review". Pediatric Surgery International. 22 (4): 326–329. doi:10.1007/s00383-006-1653-4. PMID 16518597.