Intestinal malrotation

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Intestinal malrotation
ICD-10 Q43.3
ICD-9 751.4
OMIM 193250
eMedicine ped/1200 

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]


Overview

Intestinal malrotation is a congenital anomaly of rotation of the midgut (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result:

Associated conditions

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

Causes

The exact causes are not known. It is not associated with a particular gene, but there is some evidence of recurrence in families.[1]

Presentation

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 stool. Patients with chronic, uncorrected malrotation can have recurrent abdominal pain and vomiting.

Malrotation can also be entirely asymptomatic.

Diagnosis

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 is not discovered until adulthood.[2] In adults, the "whirlpool sign" of the superior mesenteric artery can be useful in identifying malrotation.[3]

Patient#1

Patient#2

Treatment

Resuscitate the patient with fluids to stabilize them before surgically

  • correcting the malrotation
  • cutting the fibrous bands over the duodenum.

One surgical technique is known as "Ladd's procedure", after Dr. William Ladd.[4][5]

References

  1. 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. PMID 1519649. doi:10.1002/ajmg.1320440111. 
  2. 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. PMID 12394439. doi:10.1097/01.DCR.0000029637.48119.8D. 
  3. 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 : JCU. 27 (5): 279–83. PMID 10355892. 
  4. Ladd WE: Surgical Diseases of the Alimentary Tract in Infants . N Engl J Med 1936; 215: 705-8.
  5. Bass KD, Rothenberg SS, Chang JH (1998). "Laparoscopic Ladd's procedure in infants with malrotation". J. Pediatr. Surg. 33 (2): 279–81. PMID 9498402. 



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