Abstract
Objectives: To compare radiological findings with the histological diagnosis of Hirschsprung disease (HD) to establish the usefulness of contrast enema as an initial screening and diagnostic tool. To correlate accuracy of radiological diagnosis at Grey’s Hospital with international standards.Materials and methods: Systematic searches were conducted through the Picture Archiving and Communication System and the National Health Laboratory Service records for patients aged 0–12 years, with clinically suspected HD, for whom both contrast enemas and rectal biopsies were performed between 01 January 2011 and 31 August 2015 in a tertiary-level hospital. A total of 54 such patients were identified. Diagnostic accuracy levels were calculated by comparing radiological results with histology results, which is the gold standard.Results: Diagnostic accuracy of contrast enema was 78%, sensitivity was 94.4% and the negative predictive value was 95.7%. Specificity (68.8%) and positive predictive values (63%) were considerably lower. A lower false-negative rate of 5.6% was obtained at Grey’s Hospital as compared with the international reports of up to 30%.Conclusion: Contrast enema remains useful as an initial screening and diagnostic test for HD. Results of this South African tertiary referral hospital were consistent with the best international results for sensitivity of the contrast enema (approximately 80% – 88% in excluding the disease).
Highlights
Hirschsprung disease (HD), known as colonic aganglionosis, is a rare cause of constipation
The diagnostic accuracy of the contrast enema is, of continued interest to both clinicians and radiologists, and it is important to constantly assess how accurately radiologists are able to make the diagnosis of HD, with a view to both encouraging good radiological practice as well as identifying possible factors that may contribute to reduced diagnostic accuracy
A systematic search was conducted through the Picture Archiving and Communication System (PACS) for patients aged 0–12 years who had been referred for contrast enemas between 01 January 2011 and 31 August 2015 inclusive
Summary
Hirschsprung disease (HD), known as colonic aganglionosis, is a rare cause of constipation. It is defined as a functional obstruction of bowel caused by the lack of distal enteric ganglion cells with a reported incidence of 1:5000–7200 in newborns.[1] Variable lengths and segments of bowel may be involved. It is associated with significant morbidity if not diagnosed and treated early; clinicians are often cautious to promptly identify or exclude this disease. The diagnostic accuracy of the contrast enema is, of continued interest to both clinicians and radiologists, and it is important to constantly assess how accurately radiologists are able to make the diagnosis of HD, with a view to both encouraging good radiological practice as well as identifying possible factors that may contribute to reduced diagnostic accuracy
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