We conducted a systematic review to determine and compare the diagnostic accuracy of contrast enema (CE), anorectal manometry (ARM) and rectal suction biopsy (RSB) in infants suspected of Hirschsprung disease.Design:
This is a systematic review.Data Sources:
Articles were identified through electronic searches in Medline, EMBASE.com and Cochrane Controlled Trials Register. Searches were limited to articles published after 1966 in PubMed and after 1980 in EMBASE.com.Study Selection:
Studies were included if infants underwent at least one of the following tests: CE, ARM or RSB, followed by full-thickness biopsy and/or clinical follow-up as the reference standard.Data Extraction:
Two reviewers independently assessed the methods of data collection, patient selection, blinding and prevention of verification bias and description of the test protocol and reference standard. Data to construct 2 × 2 tables were abstracted for each test.Results:
Twenty-four studies met our inclusion criteria, but 2 studies were subsequently excluded for statistical analysis because data was missing to construct the 2 × 2 table. RSB (14 studies for a total of 993 patients) was the most accurate test, having both the highest mean sensitivity (93%; 95% confidence interval [CI], 88%-95%) and mean specificity (98%; 95% CI, 95%-99%). Sensitivity and specificity of ARM (9 studies for a total of 400 patients) were similar to those of RSB (91% vs 93%, P = 0.73 and 94% vs 98%, P = 0.08, respectively). Sensitivity and specificity of CE (12 studies for a total of 425 patients) were significantly lower than those of RSB and ARM, with mean sensitivity and mean specificity of 70% and 83%, respectively.Conclusions:
RSB and ARM are the most accurate tests in the diagnostic workup of Hirschsprung disease.