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September 1982

Hyperplasia of the Myenteric Plexus: Its Association With Early Infantile Megacolon and Neurofibromatosis

Author Affiliations

From the Greenwood (SC) Genetic Center (Dr Saul) and the Departments of Pediatrics (Dr Sturner) and Pathology (Dr Burger), Duke University Medical Center, Durham, NC.

Am J Dis Child. 1982;136(9):852-854. doi:10.1001/archpedi.1982.03970450094023

• 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.

(Am J Dis Child 1982;136:852-854)