Abstract
Near total aganglionosis represents the most extreme and rare form of Hirschsprung's disease. It can affect more than one member of family. We report three cases of near total intestinal aganglionosis in a family presenting with intestinal obstruction at birth. All of them were operated and a jejunostomy was performed. Outcome was dismal.
Highlights
Absence of ganglionic innervations throughout the entire gastrointestinal tract represents the most extreme and rare form of Hirschsprung Disease (HD)
The infant presented with bilious vomiting and delayed passage of meconium
The stoma still not worked and died at 4 weeks of age. Biopsy specimens of both ileostomy and jejunostomy stained with Hematoxylin and Eosin showed aganglionosis
Summary
Absence of ganglionic innervations throughout the entire gastrointestinal tract represents the most extreme and rare form of Hirschsprung Disease (HD). HD affects more than one member of the family in 4-8 % of cases. We report three cases of near total HD within one family. The infant presented with bilious vomiting and delayed passage of meconium. There was no family history of Hirschsprung disease.
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