Hirschsprung disease: accuracy of the barium enema examination

To determine the relative accuracy of the various radiologic signs of Hirschsprung disease (HD), we retrospectively reviewed both radiographs obtained after a barium enema and the medical records of 62 children who had surgery to prove or exclude the diagnosis of HD. The visualization of a rectosigmoid transition zone was highly predictive of HD, but nonvisualization did not rule out HD. A false positive transition zone at the splenic flexure was seen in four neonates who had small left colon syndrome rather than HD. Retention of barium seen on radiographs obtained 24 hours after a barium enema was not a specific sign, but it was the only sign of HD in seven neonates, including two who had total colonic aganglionosis. Anal manipulation prior to the barium enema examination did not affect the diagnostic value of that procedure. We conclude that the single most reliable radiographic sign of HD is the presence of a rectosigmoid transition zone. Statistically, the use of three radiographic features combined (rectosigmoid transition zone, retention of barium, and stool mixed with barium) correlated better with the presence or absence of HD than did any of these features alone. A comparison of 24 and 48 hour postevacuation radiographs may help to differentiate HD from meconium plug syndrome.