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Imaging Findings in Hirschsprung's Disease

The cardinal sign in Hirschsprung's disease, shown on a contrast or barium enema, is the transitional zone between the narrow and dilated bowel segment, most often seen at the recto-sigmoid junction (Fig. 6.9b,c). It may be represented by an abrupt change of calibre, or may have a more conical appearance (Fig. 6.9d). The absence of a demonstrated transitional zone does not exclude the diagnosis of Hirschsprung's disease. A classic aganglionic segment may not be visible before days 10-14 of life. A narrow segment refers to a segment of rectum where the relationship of the diameter of the rectum to the sigmoid colon is 1:1 or less. A transitional zone with a mega colon may be seen only after 4-6 weeks. The wall of the colon often has a 'saw-tooth' appearance as a result of non-peristaltic contractions. There may be marked retention of barium on delayed films after 24 h, and mixing of barium and stools after 24 h is a common finding.

The differential diagnosis in the neonatal period is that of small left colon syndrome. In this condition the left colon is a reduced calibre, typically, from the level of the splenic flexure distally, with an abrupt transitional zone between the dilated transverse colon and the narrow calibre descending colon. These findings are visible right from the first few days of life in small left colon syndrome, but would not typically be present in Hirschsprung's disease for some delayed period.

Fig. 6.9a-d. Imaging of Hirschsprung's disease. a AXR in total colonic Hirschsprung's disease showing the absence of gas in the large bowel but otherwise non-specific appearances. b Contrast enema showing an irregular and non-dilated rectum with a transition to a markedly dilated and stool-filled sigmoid colon. c Contrast enema showing a transition zone at the recto-sigmoid junction. The recto-sigmoid ratio is greater than 1. d Contrast enema in a neonate with Hirschsprung's disease with a conical transition zone at the splenic flexure. There is also a long meconium plug in the distal colon and the differential diagnosis would be functional immaturity of the colon. The final diagnosis was made by biopsy

Fig. 6.9a-d. Imaging of Hirschsprung's disease. a AXR in total colonic Hirschsprung's disease showing the absence of gas in the large bowel but otherwise non-specific appearances. b Contrast enema showing an irregular and non-dilated rectum with a transition to a markedly dilated and stool-filled sigmoid colon. c Contrast enema showing a transition zone at the recto-sigmoid junction. The recto-sigmoid ratio is greater than 1. d Contrast enema in a neonate with Hirschsprung's disease with a conical transition zone at the splenic flexure. There is also a long meconium plug in the distal colon and the differential diagnosis would be functional immaturity of the colon. The final diagnosis was made by biopsy

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