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January 3, 2011

Picture of the Month—Diagnosis

Arch Pediatr Adolesc Med. 2011;165(1):85-86. doi:10.1001/archpediatrics.2010.265-b

Denouement and Discussion: Sigmoid Volvulus

The plain radiograph of the abdomen showed massive dilation of the bowel, involving the sigmoid colon, consistent with sigmoid volvulus. She received broad-spectrum antibiotics, as well as aggressive fluid rehydration and pain control. In the emergency department, flexible sigmoidoscopy was performed with successful decompression of the volvulus, and a rectal tube was placed. Two days later, the patient developed severe abdominal pain again and was found to have a recurrence of her sigmoid volvulus. A barium enema was performed, which reduced the volvulus and showed an extremely redundant and capacious sigmoid colon. The patient then underwent surgery for sigmoidectomy and primary anastomosis at the site of the resected segments. She was discharged from the hospital 1 week later with no further complications.

Volvulus refers to torsion of a segment of the alimentary tract, often leading to bowel obstruction. Sigmoid volvulus is an exceptionally rare but potentially life-threatening condition in the pediatric age group, rarely considered in the differential diagnosis of abdominal pain because of its predilection for elderly individuals.1 In a review of sigmoid volvulus in children younger than 18 years, only 63 cases were reported from 1940 to 1999.2

Sigmoid volvulus occurs when a redundant sigmoid loop rotates around its narrow and elongated mesentery, producing venous and arterial obstruction of the affected segment and distention of the closed loop. This can lead to hemorrhagic infarction, perforation, septic shock, and death.3

The majority of patients with sigmoid volvulus present with abdominal pain, nausea, abdominal distension, and constipation; vomiting is less common. The pain associated with sigmoid volvulus is usually continuous and severe, becoming more colicky with peristalsis. Younger patients may have a more insidious presentation with recurrent attacks of abdominal pain; in these cases, resolution is due to spontaneous detorsion. Given the chronic nature of the pain, patients may be erroneously diagnosed and mistakenly treated for irritable bowel syndrome, chronic constipation, or functional abdominal pain.2

The etiology of this disorder is not completely understood. One theory suggests that children with sigmoid volvulus have a congenital elongation of the sigmoid colon, with progressive redundancy secondary to chronic constipation.4 Other predisposing factors include a narrow-based mesocolon, malrotation, prune belly syndrome, and Hirschsprung disease.5

The diagnosis of sigmoid volvulus should be suspected based on historical features and physical examination findings and confirmed with a radiologic study. A plain film of the abdomen demonstrating a dilated sigmoid colon and/or multiple small intestinal air-fluid levels supports the diagnosis.3 In some cases, radiographs may reveal a coffee bean–like shape formed by grossly dilated and closely apposed sigmoid loops, known as the coffee bean sign.6 Alternatively, a barium enema may be used both to diagnose and treat nongangrenous or nonperforated cases of sigmoid volvulus.2 Finally, computed tomographic scans may reveal a whirled pattern of the dilated sigmoid loop around associated mesocolon and a bird-beak appearance of the involved segments.3

The management of children with sigmoid volvulus should begin with fluid resuscitation and broad-spectrum antibiotics.2 Immediate interventions aim to prevent the development of gangrene; further management must identify causative anatomic abnormalities. Any signs or symptoms of intestinal gangrene or peritonitis require immediate surgical exploration. In selected cases, detorsion of the volvulus using either rigid or flexible sigmoidoscopy serves as a less invasive emergent intervention to restore perfusion to the affected bowel. As with our case, gentle pressure, combined with minimal insufflation, permitted advancement of the sigmoidoscope, causing straightening of the sigmoid colon. A sudden expulsion of gas indicated successful reduction of the volvulus. Following this procedure, a rectal tube should be placed with its proximal end beyond the area of twisting to lessen colonic distension and reduce the chance of recurrent volvulus in the acute setting.7 Alternatively, reduction of the sigmoid volvulus can be achieved using a barium enema.2 In all cases, definitive treatment requires sigmoidectomy with primary anastomosis,1,2 as was done in this case. Because Hirschsprung disease may be present in up to 17% of cases, rectal biopsy at the time of operative intervention is prudent.2

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Correspondence: Kari R. Posner, MD, Division of Emergency Medicine, The Children's Hospital of Philadelphia, 34th and Civic Center Boulevard, Philadelphia, PA 19104 (posnerkr@email.chop.edu).

Accepted for Publication: April 16, 2010.

Author Contributions:Analysis and interpretation of data: Posner and Friedlaender. Drafting of the manuscript: Posner and Friedlaender. Critical revision of the manuscript for important intellectual content: Posner and Friedlaender. Study supervision: Friedlaender.

Financial Disclosure: None reported.

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