Total intestinal aganglionosis with involvement of the stomach |
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| Authors: | Osman Faruk Şenyüz Nur Danişmend Ergun Erdoğan Gül§en Özbay Cenk Büyükünal |
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| Affiliation: | (1) Department of Pediatric Surgery and Department of Pathologic Anatomy, Cerrahpa§a Medical School, University of Istanbul, Istanbul, Turkey;(2) Okumu§ Adam Sokak, N.3 D.5 Fatih/Istanbul, Turkey |
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| 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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| Keywords: | Total intestinal aganglionosis Hirschsprung's disease |
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