eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Volvulus
Updated: May 13, 2008
Introduction
Background
Volvulus is defined as a complete twisting of a loop of intestine around its mesenteric attachment site. Such twisting can occur at various sites of the 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. This article mainly concerns midgut volvulus because it is the most common type of volvulus and is very serious in infants and children. Sigmoid volvulus is also briefly discussed below.
Numerous rotational abnormalities have been described that correlate with abnormal embryologic development of the midgut. Mall first described intestinal rotation during the embryologic period in 1898. However, Waugh in 1911 first described the clinical presentation of intestinal malrotation. Ladd, reporting in 1936, wrote the classic paper on surgical treatment of malrotation.
Pathophysiology
Embryologically, extracoelomic development with rapid elongation of the fetal intestine occurs at 4-8 weeks' gestation. The superior mesenteric artery (SMA), which supplies the small intestine and proximal colon, acts as the main supply of blood to these segments and acts as the axis of their subsequent rotation.
After extracoelomic elongation has occurred, the duodenal loop begins its counterclockwise 270° rotation around the SMA while returning to the abdomen; the loop initially passes to the right of the SMA, then passes below it, and, finally, passes to the left it, where the loop becomes fixed. The loop forms the ligament of Treitz and forms the normal duodenal C-shaped extension toward the left side across the midline. At the same time, the cecocolic loop begins its rotation below the SMA, rotating first to the left, then above, and, finally, to the right of the SMA. At this stage, the cecum descends from the right upper quadrant to the right lower quadrant.
If the duodenal loop remains on the right side of the abdomen, and the cecocolic loop remains on the left in relation to the SMA, nonrotation is the result. On the other hand, malrotation results from an interruption in intestinal rotation during the second stage of development. In malrotation, the duodenal loop lacks 90° of its normal 270° rotation, and the cecocolic loop lacks 180° of its normal rotation.
Malrotation leaves the cecum high in the mid abdomen with its peritoneal attachments, called the Ladd bands. These bands stretch from the cecum to the right lateral abdominal wall, causing compression of the duodenum and mechanical obstruction. Abnormal fixation of the ligament of Treitz causes narrowing and kinking of the duodenum near its junction with the jejunum. Along with it, the mesentery remains bunched up in the epigastrium and does not fan out.
All these anatomic factors cause the small bowel and its vascular supply to become suspended from a narrow pedicle, like a bell clapper, which predisposes to midgut volvulus and infarction.
Displaced viscera as a result of other developmental abnormalities, such as gastroschisis, omphalocele, or congenital diaphragmatic hernia, predispose to malrotation.
Malrotation is also associated with duodenal atresia, Meckel diverticulum, intussusception, small bowel atresia, prune belly syndrome, gastric volvulus, persistent cloaca, Hirschsprung disease, and extrahepatic biliary anomalies.
In contrast to midgut volvulus, sigmoid volvulus is usually the result of a dilated rectosigmoid colon on a narrow pedicle.
Frequency
United States
An incidence of 1 in 500 live births has been reported for malrotation with midgut volvulus.
International
Sigmoid volvulus is more common in developing nations than in the United States, likely because of dietary differences in terms of fiber. Chagas disease in Brazil may account for a significant proportion of cases in that country.
Mortality/Morbidity
See Complications.
Sex
No sex predilection is known; however, midgut volvulus predominates in male infants, with a male-to-female ratio of 2:1 in the neonatal period.
Age
Of those in whom volvulus occurs, 68-71% are neonates. Most cases occur by age 2 months, but as many as 41% of cases occur at an older age.
Approximately 40% of infants with malrotation develop symptoms within the first week after birth; 50% present within the first month, 75% present before age 1 year, and the remaining 25% present after age 1 year.
Clinical
History
Clinical presentation of these patients varies.
- In the first month of life, the most typical presentation includes feeding intolerance or bilious vomiting and sudden onset of abdominal pain. Bilious vomiting is the hallmark presentation and is observed 77-100% of the time. In infants of this age, consider such a presentation diagnostic of malrotation with midgut volvulus until proven 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.
- Sigmoid volvulus typically presents with abdominal pain, distention, and inability to pass stool or flatus (obstipation). Vomiting may be a late presenting feature, and cases may progress to peritonitis, sepsis, and death.
Physical
- In early cases, patients may appear well, and abdominal examination findings may be normal. In fact, normal findings on abdominal examination have been reported in as many as 50% of patients. In one series, 32% of patients had abdominal distension but no tenderness.
- Patients who present acutely usually have pain out of proportion to the degree of abdominal tenderness.
- Because the obstruction is very proximal, abdominal distension is not usually present.
- Careful examination may reveal a palpable abdominal mass in some patients.
- Signs of intraluminal blood loss, such as hematochezia or stool guaiac testing, are usually positive.
- A high index of suspicion is required to establish the diagnosis at an early stage. If the diagnosis is missed, intestinal ischemia progresses to gangrene, and bowel distension from gas-producing organisms within the intestine occurs.
- Once ischemia occurs, almost all patients develop diffuse and severe abdominal pain and signs of peritonitis. Patients with gangrene are usually tachycardic and hypovolemic. Passage of blood or sloughed mucosal tissue may be noted as vascular compromise progresses.
- As ischemia progresses to infarction and necrosis, fever, peritonitis, abdominal distension, profound hypovolemia, and septic shock develop.
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| Follow-up: Volvulus |
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Keywords
midgut volvulus, intestinal malrotation, superior mesenteric artery, SMA, midgut, rotational abnormality, extracoelomic elongation, duodenal loop, cecocolic loop, Ladd bands, sigmoid volvulus, duodenal atresia, Meckel diverticulum, intussusception, small bowel atresia, prune belly syndrome, gastric volvulus, persistent cloaca, Hirschsprung disease, extrahepatic biliary anomalies, Chagas disease, feeding intolerance, failure to thrive, constipation, bloody diarrhea, hematemesis, irritable bowel syndrome, peptic ulcer disease, kidney stones, peritonitis, hematochezia, ischemia, gangrene, intestinal ischemia, necrosis, abdominal distension, hypovolemia, septic shock
