Intestinal malrotation
Encyclopedia
Intestinal malrotation is a congenital anomaly of rotation of the midgut
(embryologically, the gut undergoes a complex rotation outside the abdomen). As a result:
in their stool
. Patients with chronic
, uncorrected malrotation can have recurrent abdominal pain and vomiting.
Malrotation can also be entirely asymptomatic.
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 not discovered until adulthood. In adults, the "whirlpool sign" of the superior mesenteric artery
can be useful in identifying malrotation.
With this condition the appendix is often on the wrong side of the body and therefore removed as a precausionary measure during the surgical procedure.
One surgical technique is known as "Ladd's procedure", after Dr. William Ladd.
Midgut
The midgut is the portion of the embryo from which most of the intestines develop. After it bends around the superior mesenteric artery, it is called the "midgut loop"...
(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 abdomenAbdomenIn vertebrates such as mammals the abdomen constitutes the part of the body between the thorax and pelvis. The region enclosed by the abdomen is termed the abdominal cavity...
- the cecumCecumThe cecum or caecum is a pouch, connecting the ileum with the ascending colon of the large intestine. It is separated from the ileum by the ileocecal valve or Bauhin's valve, and is considered to be the beginning of the large intestine. It is also separated from the colon by the cecocolic...
is displaced (from its usual position in the right lower quadrant) into the epigastriumEpigastriumThe epigastrium is the upper central region of the abdomen. It is located between the costal margins and the subcostal plane....
- right hypochondriumHypochondriumThe hypochondrium is the upper part of the abdomen dorsal to the lowest ribs of the thorax. The word derives from the Greek term ὑποχονδρος hupochondros, meaning abdomen, or literally under cartilage.The liver is found in the right hypochondrium.... - the ligament of Treitz is displaced inferiorly and rightward
- fibrous bands (of Ladd)Ladd's bandsLadd's bands, sometimes called bands of Ladd is a fibrous stalk of peritoneal tissue that attaches the cecum to the abdominal wall, and creates an obstruction of the duodenum. This condition is found in malrotation of the intestine....
course over the horizontal part of the duodenumDuodenumThe duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear and the terms anterior intestine or proximal intestine may be used instead of duodenum...
(DII), causing intestinal obstruction. - the small intestineSmall intestineThe small intestine is the part of the gastrointestinal tract following the stomach and followed by the large intestine, and is where much of the digestion and absorption of food takes place. In invertebrates such as worms, the terms "gastrointestinal tract" and "large intestine" are often used to...
has an unusually narrow base, and therefore the midgut is prone to volvulusVolvulusA volvulus is a bowel obstruction with a loop of bowel whose nose has abnormally twisted on itself.-Types:* Volvulus Neonatorum.* Volvulus Small Intestine.* Volvulus Caecum.* Volvulus Sigmoid Colon .* Gastric volvulus....
(a twisting that can obstruct the mesenteric blood vessels and cause intestinal ischemiaIschemiaIn medicine, ischemia is a restriction in blood supply, generally due to factors in the blood vessels, with resultant damage or dysfunction of tissue. It may also be spelled ischaemia or ischæmia...
).
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
- Superior mesenteric artery syndromeSuperior mesenteric artery syndromeSuperior mesenteric artery syndrome is a very rare, life-threatening gastro-vascular disorder characterized by a compression of the third portion of the duodenum by the abdominal aorta and the overlying superior mesenteric artery...
Causes
The exact causes are not known. It is not associated with a particular gene, but there is some evidence of recurrence in families.Presentation
Patients (often infants) present acutely with midgut volvulus, manifested by bilious vomiting, crampy abdominal pain, abdominal distention, and the passage of blood and mucusMucus
In vertebrates, mucus is a slippery secretion produced by, and covering, mucous membranes. Mucous fluid is typically produced from mucous cells found in mucous glands. Mucous cells secrete products that are rich in glycoproteins and water. Mucous fluid may also originate from mixed glands, which...
in their stool
Human feces
Human feces , also known as a stool, is the waste product of the human digestive system including bacteria. It varies significantly in appearance, according to the state of the digestive system, diet and general health....
. Patients with chronic
Chronic (medicine)
A chronic disease is a disease or other human health condition that is persistent or long-lasting in nature. The term chronic is usually applied when the course of the disease lasts for more than three months. Common chronic diseases include asthma, cancer, diabetes and HIV/AIDS.In medicine, the...
, uncorrected malrotation can have recurrent abdominal pain and vomiting.
Malrotation can also be entirely asymptomatic.
Diagnosis
With acutely ill patients, consider emergency surgery laparotomyLaparotomy
A laparotomy is a surgical procedure involving a large incision through the abdominal wall to gain access into the abdominal cavity. It is also known as coeliotomy.- Terminology :...
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
Radiocontrast
Radiocontrast agents are a type of medical contrast medium used to improve the visibility of internal bodily structures in an X-ray based imaging techniques such as computed tomography or radiography...
enema
Enema
An enema is the procedure of introducing liquids into the rectum and colon via the anus. The increasing volume of the liquid causes rapid expansion of the lower intestinal tract, often resulting in very uncomfortable bloating, cramping, powerful peristalsis, a feeling of extreme urgency and...
, 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. In adults, the "whirlpool sign" of the superior mesenteric artery
Superior mesenteric artery
In human anatomy, the superior mesenteric artery arises from the anterior surface of the abdominal aorta, just inferior to the origin of the celiac trunk, and supplies the intestine from the lower part of the duodenum through two-thirds of the transverse colon, as well as the pancreas.-Location...
can be useful in identifying malrotation.
Treatment
Resuscitate the patient with fluids to stabilize them before surgically- correcting the malrotation
- cutting the fibrous bands over the duodenumDuodenumThe duodenum is the first section of the small intestine in most higher vertebrates, including mammals, reptiles, and birds. In fish, the divisions of the small intestine are not as clear and the terms anterior intestine or proximal intestine may be used instead of duodenum...
.
With this condition the appendix is often on the wrong side of the body and therefore removed as a precausionary measure during the surgical procedure.
One surgical technique is known as "Ladd's procedure", after Dr. William Ladd.
See also
External links
- Macrorad Teleradiology Intestinal Malrotation of Adults