Intestinal malrotation
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223725844
2008-07-05T13:41:34Z
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{{Infobox_Disease
| Name = {{PAGENAME}}
| Image =
| Caption =
| DiseasesDB =
| ICD10 = {{ICD10|Q|43|3|q|38}}
| ICD9 = {{ICD9|751.4}}
| ICDO =
| OMIM = 193250
| MedlinePlus =
| eMedicineSubj = ped
| eMedicineTopic = 1200
| MeshID =
}}
'''Intestinal malrotation''' is a [[congenital]] anomaly of rotation of the [[midgut]] (embryologically, the gut undergoes a complex rotation outside the abdomen). As a result:
* the [[small bowel]] 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 [[Ladd's bands|bands (of Ladd)]] course over the horizontal part 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]]).
==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.<ref name="pmid1519649">{{cite journal |author=Stalker HJ, Chitayat D |title=Familial intestinal malrotation with midgut volvulus and facial anomalies: a disorder involving a gene controlling the normal gut rotation? |journal=Am. J. Med. Genet. |volume=44 |issue=1 |pages=46–7 |year=1992 |pmid=1519649 |doi=10.1002/ajmg.1320440111}}</ref>
==Presentation==
Patients (often infants) present acutely with [[midgut volvulus]], manifested by [[bilious]] [[vomit]]ing, crampy abdominal pain, abdominal [[distention]], and the passage of blood and [[mucus]] in their [[stool]]. Patients with [[chronic (medicine)|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, [[Radiocontrast|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.<ref name="pmid12394439">{{cite journal |author=Dietz DW, Walsh RM, Grundfest-Broniatowski S, Lavery IC, Fazio VW, Vogt DP |title=Intestinal malrotation: a rare but important cause of bowel obstruction in adults |journal=Dis. Colon Rectum |volume=45 |issue=10 |pages=1381–6 |year=2002 |pmid=12394439 |doi=10.1097/01.DCR.0000029637.48119.8D |doi_brokendate=2008-07-05}}</ref> In adults, the "whirlpool sign" of the [[superior mesenteric artery]] can be useful in identifying malrotation.<ref name="pmid10355892">{{cite journal |author=Yeh WC, Wang HP, Chen C, Wang HH, Wu MS, Lin JT |title=Preoperative sonographic diagnosis of midgut malrotation with volvulus in adults: the "whirlpool" sign |journal=Journal of clinical ultrasound : JCU |volume=27 |issue=5 |pages=279–83 |year=1999 |pmid=10355892 |doi=10.1002/(SICI)1097-0096(199906)27:5<279::AID-JCU8>3.0.CO;2-G}}</ref>
==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.<ref>Ladd WE: Surgical Diseases of the Alimentary Tract in Infants . N Engl J Med 1936; 215: 705-8.</ref><ref name="pmid9498402">{{cite journal |author=Bass KD, Rothenberg SS, Chang JH |title=Laparoscopic Ladd's procedure in infants with malrotation |journal=J. Pediatr. Surg. |volume=33 |issue=2 |pages=279–81 |year=1998 |pmid=9498402 |doi=10.1016/S0022-3468(98)90447-X}}</ref>
==See also==
*[http://www.vesalius.com/cfoli_frms.asp?VID=628&StartFrame=13&tnVID=629#n15 Normal rotation of the gut]
==References==
{{reflist}}
{{disease-stub}}
{{Congenital malformations and deformations of digestive system}}
[[Category:Surgery]]
[[de:Malrotation]]
[[it:Malrotazione intestinale]]
[[pl:Malrotacja jelit]]