| Summary: | The Hirschsprung disease characterised by congenital absence of ganglion cells, is confirmed by sero muscluar biopsies of the involved bowel, hence the initial leveling colostomy/ileostomy followed by subsequent definitive pull through procedure. However only the full circumference of the pulled down bowel having ganglion cells in all four quadrants is confirmatory. Histopathological disparity, differences or errors in diagnosis can arise in both sero muscular and full circumference full thickness biopsy samples, challenging the surgeon in further management of the these children; such a rare case has been described here with is its successful surgical management. Bishop koop procedure classically described in meconium ileus can be attempted in such confusing cases before contemplating major surgical intervention.
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