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Correlation of Radiographic and Histologic Transition Zones in Hirschsprung's Disease - Abstract

Ike Njere, Bijan Hedayati, Reza Salari, Dorothy Kufeji, Alireza S. Keshtgar


Purpose: Hirschsprung’s disease (HD) is a common paediatric surgical condition with an incidence of 1 in 5000 live births. There has been renewed interest in the role of contrast enema for predicting the level of aganglionosis with single stage endoanal pullthrough in HD. Few studies have evaluated the accuracy of contrast enema in identifying the transition zone. The aim of this study was to ascertain the correlation between the radiographic and histologic transition zones (HTZ) in Hirschsprung’s disease and to determine the sensitivity and specificity of contrast enema in identifying the transition zone.

Material and method: We retrospectively studied 61 patients with Hirschsprung’s disease, presenting between January 2006 and October 2010. Case notes, radiology and histology reports were reviewed. Correlation was defined as the Radiographic Transition Zone (RTZ) in the same or adjoining segment of bowel to the transition zone established by histology. All patients were initially managed by rectal washouts prior to obtaining contrast enema.

Results: Of the 61 patients, there was complete data in 52/61. Of these 22 had contrast enema. Seventeen of them were males. Majority were term babies with a median age at diagnosis of 10 days. An RTZ was clearly defined in 18 of 22 that had a contrast enema. In 16/18 patients there was concordance between the RTZ and HTZ. Contrast enema had a sensitivity of 93% and specificity of 50% for identifying the RTZ in the recto-sigmoid region. The discordant rate was 11.1% (2/18).

Conclusions: In the majority of patients, the transition zone can be predicted by contrast enema (88% concordance). The radiological appearance is not affected by prior rectal washout. Long segment disease and catheter tip beyond the anal canal contribute to discordance.

Keywords: Hirschsprung’s, contrast enema, histology, concordance


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