Diagnosis of Hirschsprung's disease

OBJECTIVE. Our aims were to determine the validity of using low-osmolality water-soluble contrast enemas (WSCE) in neonates and infants with suspected Hirschsprung's disease (HD) and to devise a scoring system that uses a checklist of radiologic signs to determine the probability of HD. MATERIALS AND METHODS. The records of all patients referred by pediatric surgeons from 1988 through 1992 for the radiologic investigation of possible HD were retrospectively reviewed. Thirty-eight patients who were from 2 days to 9 months old were studied; 20 of them were neonates (less than 1 month old). Of all the patients, 24 underwent WSCE and the other 14 underwent barium enemas, For all patients, HD had been diagnosed by rectal biopsy or excluded by biopsy, clinical follow-up, or both, Radiographs were read by a gastrointestinal radiologist who used a checklist of diagnostic criteria reported in the literature. The sensitivity and specificity of the findings were compared with those in the literature. RESULTS. Of the 18 patients with HD, 12 were neonates. All reported radiologic diagnostic criteria were seen; the frequency, sensitivity, and specificity of the findings were reported, Twenty percent (n = 2) of HD patients in the WSCE group (n = 10) had negative findings. Two of the 12 neonates developed colonic perforation, one during the enema and the other within 24 hr of the procedure. CONCLUSION. WSCE has a sensitivity and specificity equivalent to those of the barium enema for the detection of HD. For the two patients with perforation, the use of WSCE was of considerable benefit, avoiding the problems associated with barium spillage into the peritoneal cavity. A scoring system for diagnostic enemas is feasible.