Challenges in the diagnosis of intestinal neuronal dysplasia type B: A look beyond the number of ganglion cells

Table 2 Summary of the histopathological criteria for the diagnosis of intestinal neuronal dysplasia type B

Diagnostic criteria	Definition
Frankfurt Consensus[32], 1990	Mandatory criteria	Submucosal plexus hyperplasia
		Increased activity of the AChE enzyme in nerve fibers around submucosal blood vessels
	Complementary criteria	Neuronal heterotopy
		Increased AChE activity in the lamina mucosa
Meier-Ruge et al[33], 2004	Quantitative criteria	At least 20% of giant nerve ganglia1 in the submucosa, in 25 submucosal ganglia
	Age criteria	Patients must be older than 1 yr

¹Giant nerve ganglia: A ganglion with more than eight ganglion cells.

AChE: Acetylcholinesterase.