Ganglion cell distribution in the normal rectum and anal canal A basis for the diagnosis of Hirschsprung's disease by anorectal biopsy

Abstract

The normal distribution of ganglia and ganglion cells in the anal canals and lower rectums of 22 babies and children has been plotted and enumerated. Ganglion cells were distributed in three separate plexuses, the myenteric, the deep submucous, and the superficial submucous plexuses. A “hypoganglionic” zone has been defined and found to extend an average of 4 mm. in the myenteric plexus, 7 mm. in the deep submucous plexus, and 10 mm. in the superificial submucous plexus from and cranial to the anal valves or pectinate line of the anal canal. Any biopsy which includes the plexus above the theoretical upper limit of the hypoganglionic zone should be adequate for the diagnosis of Hirschsprung's disease. In particular, mucosal buttons obtained by suction capsule provide the pathologist with suitable material for the detection of ganglion cells in the superficial submucous ganglion cell layer provided the sections are stained with hematoxylin and eosin after paraffin processing. These ganglion cells are extremely difficult to identify by frozen section and this method is not suitable for these small specimens. Presence of ganglion cells in specimens obtained from the hypoganglionic zone or a point 3 cm. inside the anal valves excludes the diagnosis of Hirschsprung's disease. Absence of ganglion cells in the hypoganglionic zone does not exclude Hirschsprung's disease, but absence at the point 3 cm. inside the anal canal is diagnostic of this disease.