Hyperplasia of the myenteric plexus. Its association with early infantile megacolon and neurofibromatosis
R. A. Saul, R. A. Sturner and P. C. Burger
An infant with cutaneous criteria for neurofibromatosis had hyperplasia of
the intestinal myenteric plexus and a clinical presentation mimicking
Hirschsprung's disease. Many of his phenotypic features are also found in
multiple endocrine neoplasia type IIb, a condition in which hyperplasia of
the myenteric plexus is common. This case illustrates the overlapping
nature of neural crest-derived conditions and the variable presentation of
megacolon (ganglionic or aganglionic) in infancy.
