Barium enema findings in total colonic aganglionosis: a single-center, retrospective study

Abstract

Background Preoperative diagnosis of total colonic aganglionosis is important for the rational choice of treatment. The present study aimed to evaluate the diagnostic performance of radiographic signs on preoperative barium enema in patients with total colonic aganglionosis. Methods Forty-four patients [41 (3-659) days] with total colonic aganglionosis, including 17 neonatal patients, who received preoperative barium enema at Beijing Children’s Hospital, from January 2007 to December 2019 were included. All radiographs were retrospectively restudied by 2 pediatric radiologists to ascertain radiographic signs including rectosigmoid index, transition zone, irregular contraction, gas-filled small bowel, microcolon, question-mark-shaped colon and ileocecal valve reflux. Kappa test was performed to assess the accuracy and consistency of the radiographic signs. Results The 2 radiologists showed slight agreement for gas-filled small bowel, microcolon and rectosigmoid index, fair agreement for transition zone and irregular contraction, and moderate agreement for question-mark-shaped colon and ileocecal valve reflux (Kappa values, 0.043, 0.075, 0.103, 0.244, 0.397, 0.458 and 0.545, respectively). In neonatal patients, the 2 radiologists showed moderate agreement for ileocecal valve reflux and substantial agreement for question-mark-shaped colon (Kappa values, 0.469 and 0.667, respectively). In non-neonatal patients, the 2 radiologists showed substantial agreement for ileocecal valve reflux (Kappa value, 0.628). In 36 patients with total colonic aganglionosis extending to the ileum, the accuracies of question-mark-shaped colon, ileocecal valve reflux and the combination of both were 47%, 53%, and 75%, respectively, in one radiologist and 53%, 50% and 72%, respectively, in the other radiologist. Conclusions Ileocecal valve reflux is a relatively reliable radiographic sign for diagnosing total colonic aganglionosis and could improve the diagnostic accuracy upon combination with question-mark-shaped colon.

High-Resolution Anorectal Manometry as a Screening Tool for Hirschsprung’s Disease: A Comprehensive Retrospective Analysis

“…For many years, contrast enema (CE) was recommended as the first diagnostic test for HD [ 20 ]. The authors report that its sensitivity and specificity in the diagnosis of HD vary 76–86.9% and 92.1–97%, respectively [ 21 , 22 ]. However, CE has limitations, as it involves irradiation of the urogenital area.…”

“…Furthermore, in cases of early HD when there is no dilation yet, CE may not reveal any abnormalities [ 14 ]. In cases of ultra-short segment or total aganglionosis, diagnosis can also be challenging, although these represent a minority of all HD cases [ 22 ]. The test results are delayed due to the need to take the X-rays with the 24 h interval and an experienced radiologist assessment.…”

Gastrointestinal Emergency in Neonates and Infants: A Pictorial Essay

“…Imaging findings are variable, including a normal caliber colon, a microcolon, and a question-mark-shaped colon that is foreshortened with rounded flexures ( Fig. 9C ) [ 25 30 ].…”

Diagnostic value of the microcolon using ultrasonography in small bowel atresia

“…A contrast enema helps investigate suspected Hirschsprung disease [ 5 ]. Although barium and water-soluble enema can detect microcolon and facilitate SBA diagnosis, the radiation and risk of intestinal perforation have led many scholars to recommend minimising their use [ 6 – 9 ]. Ultrasonography (US), a safe, convenient and inexpensive imaging method, is suitable for neonates because of their thin abdominal wall.…”