Introduction
Background
Midgut volvulus is a condition in which the intestine has become twisted as a result of malrotation of the intestine during fetal development. Malrotation of the intestine occurs when the normal embryologic sequence of bowel development and fixation is interrupted. The malrotated bowel is prone to torsion (as demonstrated in the first image below), which can lead to midgut volvulus. The second image, from a GI series, shows a lateral view of midgut volvulus.
Malrotation with midgut volvulus shows torsion around the narrow mesenteric stalk.
Upper GI series shows malrotation with midgut volvulus in a lateral view.
Recent studies
Sizemore et al assessed the usefulness of upper GI series in the detection of intestinal malrotation and midgut volvulus in infants and youths. The authors reviewed data from 166 patients aged 21 years or below (40% of whom were neonates and 73% of whom were below age 12 months). The UGI series had a sensitivity of 96%, providing positive results in 156 of the 163 patients with surgically verified malrotation. In addition, the series showed midgut volvulus in 30 out of 38 patients in whom volvulus was surgically verified. Data from the study indicated that jejunal position can result in inaccurate interpretation of UGI series.1
Gilbertson-Dahdal et al described the clinical, imaging, and surgical findings in 4 neonates with radiographic findings that suggested duodenal atresia (double-bubble sign) but who were subsequently found to have malrotation with midgut volvulus. According to the authors, when surgical treatment of patients with the double-bubble sign is delayed, an upper GI radiographic or ultrasound study is needed to identify possible malrotation with midgut volvulus.2
Yu et al reported on Ladd procedures in heterotaxy patients with asymptomatic intestinal malrotation and noted that the risk of midgut volvulus, morbidity from elective operations, and overall prognosis must be considered in such patients. Midgut volvulus was present at surgery in 27% of the heterotaxy patients. After a Ladd procedure, patients had a hospital stay of 12.9 days, a 9.7% risk of small bowel obstruction, and an in-hospital mortality of 9.7%. The authors concluded that elective Ladd procedures are well tolerated by heterotaxy patients and that, because of the risk of midgut volvulus and because of improved survival beyond infanthood, Ladd procedures should be provided to heterotaxy patients with asymptomatic malrotation.3
Stanfill et al performed a retrospective analysis of open and laparoscopic Ladd procedures (120 open; 36 laparoscopic) in 156 children with rotational anomalies of the midgut. Overall, 75% of patients were symptomatic, with the most common symptoms being emesis and pain. The duration of surgery was similar, but the time to starting feeds and the amount of time to attain full feeding were found to be significantly less in the laparoscopic patients. The laparoscopic procedures were also associated with decreased postoperative length of stay. The conversion rate to open was 8.3%. The authors concluded that laparoscopic Ladd procedures can be performed safely in selected patients with midgut malrotation with no increase in complications.4
Pathophysiology
Development of the human gut takes place during the first months of fetal life. In normal embryos, physiologic herniation of the gut through the umbilicus at 6 weeks’ gestation is accompanied by a 270° counterclockwise rotation of the developing intestine around the superior mesenteric artery (SMA). By 10-12 weeks, the intestine returns to the abdomen and assumes its normal adult anatomic position. Normal small bowel mesentery has a broad attachment stretching diagonally from the duodenojejunal junction (DJJ), in the left upper quadrant, to the cecum, in the right lower quadrant.5 This point of attachment at the DJJ is called the ligament of Treitz.
Malrotation is most commonly caused by incomplete rotation of the intestine (<270° of counterclockwise rotation, occurring in weeks 5-12). Malrotation disorders can be divided into 3 categories:
- Nonrotation (0° to 90° of counterclockwise rotation, occurring before 6 weeks)
- Reverse rotation (abnormal rotation between 90° and 180°, causing obstruction or reversal of the normal duodenal/SMA relationship, occurring in weeks 6-10)
- Malrotation most often associated with malfixation (between 180° and 270° of counterclockwise rotation, occurring after 10 weeks)
The small bowel may be in the right abdomen; however, this finding is not reliable. Often, the large bowel is in the left abdomen.
Malrotation predisposes patients to 2 problems: midgut volvulus and small bowel obstruction.
Frequency
United States
Although malrotation is estimated to occur in 1 in 500 live births, the actual frequency of malrotation is unknown because many asymptomatic patients probably never present to a physician.
International
Incidence is the same as in the United States.
Mortality/Morbidity
- Midgut volvulus: The close proximity of the cecum to the duodenum is associated with a narrow stalk of mesentery around which the gut may twist, resulting in midgut volvulus (as seen in the illustration below). Accompanying superior mesenteric vascular compromise (first venous, followed by arterial) can lead to life-threatening ischemia of the small bowel and gangrenous necrosis. Mortality associated with midgut volvulus is at least 15%, and there is a high incidence of short gut syndrome, total parenteral nutrition dependence, and resultant cirrhosis.
- Duodenal obstruction: Coiling of the duodenum with the ascending colon produces complete or partial duodenal obstruction. Ladd bands are abnormal peritoneal reflections that cross the duodenum and pass to the undersurface of the liver or posterior abdominal wall; they cause duodenal obstruction in some patients.
Malrotation with midgut volvulus shows torsion around the narrow mesenteric stalk.
Race
No racial predilection is seen.
Sex
No gender predilection is seen.
Age
In approximately 60% of patients, malrotation presents by 1 month of age. Another 20-30% of patients present at 1-12 months of age. Malrotation may remain clinically "silent" for some time and can present at any age.
Anatomy
In malrotation, the following relationships may be observed in the gut:
- The DJJ is low and to the right of the normal location, as seen in the image below.
- The proximal small bowel (jejunum) is in the right upper quadrant.
- The cecum is in the upper and/or left abdomen.
- The large bowel is in the left abdomen.
- Other associated anomalies are seen around the ampulla of Vater.
Upper GI series shows malrotation without midgut volvulus in an infant with vomiting but without failure to thrive. The duodenojejunal junction is low and to the right of the spine on this frontal view.
Presentation
In neonates, malrotation with midgut volvulus classically presents with bilious vomiting and, as the radiograph below demonstrates, high intestinal obstruction. While most neonates with bilious vomiting do not have midgut volvulus, this diagnosis must be ruled out.
Supine radiograph in a newborn with midgut volvulus shows a high intestinal obstruction.
Older children with malrotation may manifest a failure to thrive, chronic recurrent abdominal pain, malabsorption, or other vague presentations. The older the child is, the less readily identifiable is the clinical presentation. Nonrotation of the intestine may be asymptomatic and is an incidental finding on upper GI series performed for other reasons.
Associated anomalies are seen in approximately 60% of patients and include congenital heart disease with heterotaxy (abnormal positioning and arrangement of the abdominal organs, such as the spleen, liver, and major blood vessels; right-sided or left-sided isomerism). Malrotation is almost always present in patients with congenital diaphragmatic hernia and abdominal wall defects, such as omphalocele and gastroschisis. Also, malrotation is more common with imperforate anus, duodenal atresia, duodenal web, stenosis, preduodenal portal vein, annular pancreas, and biliary atresia. Malrotation in a patient with congenital heart disease and heterotaxy is shown below.
Upper GI series and a small bowel study show malrotation without midgut volvulus in a patient with complex congenital heart disease and heterotaxy. Note the small bowel in the right abdomen.
Preferred Examination
The diagnostic test of choice in a child with possible malrotation, with or without midgut volvulus, is an upper GI series.1,6
Limitations of Techniques
In most patients with malrotation, an upper GI series is easy to perform and, in experienced hands, is easy to interpret, with the following exceptions:
- With complete duodenal obstruction, an upper GI series does not differentiate between the causes of proximal intestinal obstruction. In such cases, surgical exploration is indicated.
- A redundant duodenum, seen in some normal individuals, can be confused with malrotation. A careful following of the barium shows a normal DJJ; if uncertainty exists, barium can confirm normal rotation if the cecum is seen in the right lower quadrant.
Differential Diagnoses
Duodenal Atresia
Gastroesophageal Reflux
Hypertrophic Pyloric Stenosis
Necrotizing Enterocolitis
Other Problems to Be Considered
Malrotation without midgut volvulus
Duodenal stenosis
Duodenal web
Annular pancreas
More on Midgut Volvulus |
 Overview: Midgut Volvulus |
| Imaging: Midgut Volvulus |
| Follow-up: Midgut Volvulus |
| Multimedia: Midgut Volvulus |
| References |
| Further Reading |
| Next Page » |
References
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Yu DC, Thiagarajan RR, Laussen PC, Laussen JP, Jaksic T, Weldon CB. Outcomes after the Ladd procedure in patients with heterotaxy syndrome, congenital heart disease, and intestinal malrotation. J Pediatr Surg. Jun 2009;44(6):1089-95; discussion 1095. [Medline].
Stanfill AB, Pearl RH, Kalvakuri K, Wallace LJ, Vegunta RK. Laparoscopic Ladd's Procedure: Treatment of Choice for Midgut Malrotation in Infants and Children. J Laparoendosc Adv Surg Tech A. Mar 11 2010;[Medline].
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Further Reading
Related eMedicine topics
Disorders of Rotation/Fixation and Midgut Volvulus
Volvulus
Cecal Volvulus
Gastric Volvulus
Sigmoid Volvulus
Intestinal Malrotation
Keywords
midgut volvulus, volvulus, small bowel obstruction, malrotation volvulus, bilious vomiting, volvulus bowel, malrotation with midgut volvulus
