[Diffuse digestive ganglioneuromatosis]

Discovery of intestinal ganglioneuromatosis implies the presence of the MEN IIb syndrome or, more rarely, von Recklinghausen's neurofibromatosis. The two conditions are due to dysfunction of the neural crest. This very rare intestinal pathology is illustrated by two observations. The intestinal motility disorders, due to the diffuse proliferation of the peripheral autonomous nervous system which ganglioneuromatosis represents, may have serious implications and indeed prove fatal. The etiologic role of excessive nerve growth factor production in these two entities is mentioned. The physiopathologic implications of an increase in vasoactive intestinal polypeptide, and of possible ectopic secretion of calcitonin by ganglioneuromatosis, are discussed.