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Near total colonic aganglionosis is one of the rarest forms of Hirschsprung's disease and until recent years it has been considered deadly. Establishing a correct diagnosis has proven to be challenging, because while the clinical and radiological features can be useful, they are not pathognomonic. Chronic intestinal obstruction and long-term parenteral nutrition dependency are associated with a high mortality risk for these patients. While there is no current consensus with regards to apmid:25375063 fatcat:hmcignpdcvgmpoevknevehwd6q