Intestinal volvulus is defined as a complete twisting of a loop of intestine around its mesenteric

attachment site. It is related to but not precisely synonymous with malrotation, a more general term used
when the normal process of rotation and fixation of the midgut goes awry. Malrotation may lead to 2
critical complications: mechanical obstruction of the proximal intestine and twisting of the intestines with
subsequent ischemia (with or without necrosis) of part or all of the midgut.
Volvulus can occur at various sites in the gastrointestinal (GI) tract, including the stomach, small intestine,
cecum, transverse colon,[1] and sigmoid colon. Midgut volvulus refers to twisting of the entire midgut
around the axis of the superior mesenteric artery (SMA). Patients with intestinal malrotation are at risk for
developing a midgut volvulus. This article primarily focuses on midgut volvulus because it is the most
common type of volvulus and it can have devastating consequences if not promptly recognized. Midgut
volvulus secondary to intestinal malrotation is more common in infants and children, but it can occur in
persons of all ages. Sigmoid volvulus, more common in elderly persons, is also briefly discussed.
Volvulus involving the GI tract can cause severe clinical problems; the most feared complication is
ischemia and necrosis of the entire midgut, which can be fatal.
Laboratory findings are nonspecific. Imaging studies are an integral part of the diagnostic process for a
patient suspected of having malrotation or other GI obstruction. Confirmation and definitive diagnosis are
accomplished more easily with an upper GI contrast series, but other imaging modalities can also be used
(as will be discussed here).
The management of abnormalities of rotation and volvulus is well established. Treatment of midgut
volvulus secondary to congenital intestinal malrotation is surgical; no other effective treatment is
available. High priority must be placed on early recognition of midgut volvulus before serious
complications develop. The surgical techniques used to treat this condition are discussed in this article.
The midgut is that portion of the intestine supplied by the SMA. It includes the distal duodenum, all of the
jejunum and ileum, and the colon as far as the mid transverse. Normally, the duodenum courses posterior
to the SMA and the superior mesenteric vein (SMV), and the transverse colon lies anterior to these
vessels.
The bowel is relatively fixed at several points: the duodenum (including the duodenojejunal [DJ] junction),
the ascending colon, the splenic flexure, and part of the descending colon. If the normal process of
rotation and fixation fail to occur, the midgut may not be normally arranged and lacks physiologic points of
fixation, and thus may be subject to twisting or volvulus.

Volvulus
Volvulus of the midgut may result in several manifestations, depending on the degree of twisting. Venous
and lymphatic obstructions occur first because of lower intravascular pressures. Vascular congestion
leads to bowel edema and possible oozing of blood, potentially causing GI bleeding. Lymphatic
congestion causes the formation of a mesenteric cyst and/or chylous ascites. It is not uncommon to find
chylous fluid in the peritoneal cavity and enlarged lymph nodes at the time of corrective surgery for
malrotation and volvulus.

If volvulus is intermittent, children may have chronic malabsorption from congestion and edema or
intermittent bouts of symptoms, usually vomiting and possibly alternating diarrhea and constipation.
Arterial compromise is seen when the twisting is significant enough to occlude venous and arterial
vessels. This represents an acute and dangerous event. Sequelae include (in order of less to more
severe) ischemia, mucosal necrosis, intramural air formation, bacterial translocation, gramnegative sepsis, full-thickness intestinal wall necrosis, perforation, peritonitis, and death.

Etiology
Specific causes of malrotation are unknown, although repeated associations have been made with
congenital syndromes such as Down syndrome and the VACTERL (vertebral, anal, cardiac, tracheal,
esophageal, renal, limb) association of anomalies. Because malrotation is an embryologic development
abnormality, it may be assumed that any interference during critical periods of fetal development can lead
to malrotation.
Associated anomalies include the following:

Trisomy 21
Congenital heart disease
Imperforate anus
Ileal and jejunal atresia or duplication
Omphalocele
Duodenal atresia and stenosis
Diaphragmatic hernia
Meckel diverticulum
VACTERL association
Trisomy 13, trisomy 18
Esophageal atresia
Situs inversus and asplenia (may be associated with biliary atresia)
Kidney abnormalities
Agenesis of corpus callosum
Pyloric stenosis
Annular pancreas
Erythroblastosis
Intussusception
Cystic fibrosis
Meconium ileus
Hirschsprung disease
Duodenal web
Biliary atresia

History
The clinical presentation of patients with volvulus varies. No unique signs or symptoms pathognomonic
for intestinal malrotation and volvulus are recognized; however, certain findings are commonly observed.
In the first month of life, the most typical presentation includes feeding intolerance with bilious (ie, yellow
or green) vomiting and sudden onset of abdominal pain.

Bilious vomiting is the hallmark presentation and is observed 77-100% of the time. Although bilious
vomiting can occur in various other medical conditions, such a presentation in young infants, should be
considered diagnostic of malrotation with midgut volvulus until proved otherwise.
In older children, symptoms can be vague and may include chronic intermittent vomiting and abdominal
cramping, failure to thrive, constipation, bloody diarrhea, and hematemesis. Children with vague clinical
features are sometimes incorrectly diagnosed as having irritable bowel syndrome, peptic ulcer disease,
kidney stones, or even psychogenic or emotional disorders. However, even in older children, intermittent
bilious vomiting is commonly seen with malrotation.
Sigmoid volvulus typically presents with abdominal pain, distention, and inability to pass stool or flatus
(obstipation). It is usually associated with a history of constipation and/or megasigmoid. Vomiting may be
a late presenting feature, and cases may progress to peritonitis, sepsis, and death.

Volvulus
Malrotation and volvulus are 2 distinct entities. Malrotation may cause intermittent and incomplete signs
and symptoms of proximal intestinal obstruction with mesenteric congestion. If volvulus has developed as
a consequence of intestinal malrotation, the obstruction is typically complete, and compromise of the
blood supply of the midgut has started as a consequence of the twisting of the mesentery (see the images
below) at the narrow pedicle of the superior mesenteric artery (SMA). This results in ischemia and
possibly necrosis.

Operative photograph illustrating midgut volvulus of full-term newborn who

underwent upper GI contrast study. Note complete twist (> 360) of entire small bowel over narrow pedicle of its mesentery.
Note appearance of small bowel and congestion and cyanosis due to vascular compromise from volvulus. Fortunately, early
operative intervention prevented development of necrosis, and emergent untwisting combined with Ladd procedure was

successful.
Operative photograph of midgut volvulus due to intestinal malrotation in 10-year-old
patient. Note twisting at base of mesentery with evidence of intestinal congestion and ischemia but no necrosis.

Thus, the signs and symptoms depend on the degree of ischemia. Manifestations can range from
lymphatic and venous congestion with simple edema to full intestinal necrosis secondary to arterial and
venous thrombosis. Once intestinal ischemia develops, pain becomes a more pronounced symptom, and
the patient may have signs of an acute abdomen with rigidity and tenderness to palpation.
Because the vascular territory of the SMA includes the distal duodenum to the midtransverse colon, the
entire midgut may become necrotic and nonviable if the volvulus is not corrected in time. Necrosis of the
entire midgut is incompatible with life (see the image below).

Operative photograph of patient with midgut volvulus in which diagnosis was made
late. Note that entire small bowel is necrotic and nonviable. This infant did not survive.

Volvulus
In the event of malrotation with midgut volvulus, vascular compromise develops, and this determines the
severity of the clinical presentation. The physical signs may range from mild abdominal pain to severe
pain with an acute abdomen or even a shocklike picture with dehydration, lethargy, and respiratory
distress.
In early infancy, the clinical picture of volvulus is one of proximal intestinal obstruction that may progress
to shock and peritonitis if unrecognized. In older children, the main clinical feature may be recurrent
midabdominal colic, with or without vomiting. A rare manifestation of nonrotation is left-sideappendicitis,
which occurs when the ileocecal loop remains on the left side of the abdomen. [10]