Abstract
Hirschsprung's disease is characterized by the absence of ganglia in the distal colon,
resulting in a functional obstruction. It is managed by excision of the aganglionic
segment and anastomosis of the ganglionated bowel just above the dentate line. The
level of aganglionosis is determined by performing multiple seromuscular biopsies
and/or full thickness biopsy on the antimesenteric border of the bowel to determine
the level of pullthrough. The transition zone is described as being irregular, and
hence a doughnut biopsy is recommended so that the complete circumference can be assessed.
Herein, we described a child in whom there was a selective absence of ganglion cells
in 30% of the circumference of the bowel along the mesenteric border for most of the
transverse colon. This case defies the known concept of neural migration in an intramural
and transmesenteric fashion and emphasizes the importance of a doughnut biopsy of
the pulled-down segment.
Keywords
atypical aganglionosis - Hirschsprung's disease - skip lesions - zonal aganglionosis
