Radiological findings in total colon aganglionosis and allied disorders

The aim of this study was to review the radiological findings of three cases of total colon aganglionosis (TCA), hypoganglionosis, and immature ganglionosis, and to compare the differences in diagnosis and follow-up of these three disease entities.

Three neonates with neonatal onset of abdominal distension with vomiting were investigated, and the cases were diagnosed as TCA, hypoganglionosis, and immature ganglionosis, respectively. Radiological examination of each neonate was performed during the neonatal period and at follow-up.

A plain abdominal radiograph showed massive abdominal bowel gas and multiple air-fluid levels in all cases. Barium enema findings including no transition zone, normal rectosigmoid index, reflux of barium into a dilated ileum, and retention of barium on delayed film were observed in all three cases. In aganglionosis and hypoganglionosis, a normal-sized colon, irregular contraction, shortening of the colon, and lack of redundancy were observed. In immature ganglionosis, microcolon was present but there was no shortening of the colon or loss of redundancy. Barium studies following ileostomy during childhood revealed no efficient peristalsis after the neonatal period in patients with aganglionosis and hypoganglionosis. Conversely, the patient with immature ganglionosis showed maturity of colonic function on barium studies after infancy.

The clinical and radiological findings of TCA and allied disorders are similar in neonates. Sequential contrast intestinal studies could reveal peristalsis of the colon wall, suggesting maturity of the ganglion cells.