Hirschsprung's disease is a congenital anomaly affecting neural crest cell migration and proliferation in the myenteric plexi resulting in dysmotility, which can present as bilious emesis, delayed meconium passage, and bowel obstruction in neonates, or chronic constipation in older children. Depending on the extent of aganglionosis, this disease can involve the whole gut. Treatment involves a temporary ostomy and interval definitive surgical reconstruction. In patients with near or total intestinal aganglionosis, however, there is no consensus on the most effective surgical reconstruction as consideration of the length and function of the normal remnant bowel create concerns for complications with short bowel syndrome post-operatively. We present a case of near-total intestinal aganglionosis highlighting the various options for definitive surgical reconstruction.
Keywords: aganglionosis; bowel obstruction; colonic aganglionosis; congenital aganglionosis; transanal pull through procedure.
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