Abstract
Total intestinal aganglionosis is the rarest form of Hirschsprung's disease, with absence of ganglia from the duodenum to the rectum. In addition to those cases reported in the literature, a neonate with total intestinal aganglionosis with involvement of the stomach is presented. Diagnosis of this extensive form of Hirschsprung's disease is a major problem. These patients lie beyond the scope of present surgical expertise, and the outcome is universally fatal.
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