Mining for Hirschprung's disease in the overflowing stream

Abstract

When comparing the year 1993 with the year 2001, Gary Freed and colleagues demonstrated that there has been an increase in the proportion of care provided by both pediatrician generalists and pediatric specialists for patients with constipation (Freed et al J Pediatr 2005;146:14-19). This trend reflects growth in the market share of pediatricians relative to nonpediatrician providers as well as an increase in the overall number of physician visits for constipation. This “backlog” of patients presents the clinician with a diagnostic dilemma – although in the vast majority of infants and children with constipation no organic disease is present, Hirschprung's disease must at least be considered and, in a small subset, excluded. The “gold standard” for diagnosis of Hirschprung's disease—full thickness biopsy (FTB)—is invasive and requires general anesthesia. There is no easy path to the gold! Therefore, deLorijn et al asked whether other tests could replace FTB. They compared the diagnostic accuracy of three tests considered to be useful for the detection of Hirschprung's disease—contrast enema (CE), anorectal manometry (ARM), and rectal suction biopsy (RSB). Of 111 consecutive patients with severe constipation, Hirschprung's disease was confirmed by FTB or from the operative specimen in 28. Of the other tests, RSB had the highest sensitivity (93%) and specificity (100%) compared to CE (sensitivity = 76%; specificity = 97%) and ARM (sensitivity = 83%; specificity = 93%). These differences were not statistically significant; however, the study may have been underpowered to detect such a difference. Test results were inconclusive in 8 infants via CE, in 15 via ARM, and in 2 via RSB. These results support the concept that, short of FTB, a properly obtained RSB is the most accurate and conclusive test to diagnose Hirschprung's disease. Diagnosis of Hirschsprung's Disease: A Prospective, Comparative Accuracy Study of Common TestsThe Journal of PediatricsVol. 146Issue 6PreviewTo compare the diagnostic accuracy of contrast enema (CE), anorectal manometry (ARM), and rectal suction biopsy (RSB) for the detection of Hirschsprung's disease (HD). Full-Text PDF