Hyperplasia of the myenteric plexus. Its association with early infantile megacolon and neurofibromatosis

Am J Dis Child. 1982 Sep;136(9):852-4.


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.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Hyperplasia
  • Infant, Newborn
  • Male
  • Megacolon / complications*
  • Myenteric Plexus / pathology*
  • Neurofibromatosis 1 / complications*
  • Paraneoplastic Endocrine Syndromes / pathology
  • Skin Neoplasms / complications*