Article révisé par les pairs
Résumé : We report a rare case of a 7-weeks-old infant presenting with a vasoactive intestinal polypeptide (VIP)-secreting intestinal ganglioneuroma giving rise to a " Watery Diarrhea Hypokalemia Achlorydria syndrome ". After surgery, digestive symptoms resolved quickly and serum VIP level decreases to normal value. The atypical intramural, centered in the muscularis propria, proliferation of this solitary ganglioneuroma can not exclude the diffuse type of ganglioneuromatosis. Diffuse type of ganglioneuromatosis may be associated with tumor-predispositing syndromes like multiple endocrine neoplasia (MEN) type 2B or more rarely neurofibromatosis type 1 (NF1). In this case, the mutation of RET proto-oncogen is absent. According to the literature, this is the fifth reported pediatric case of vasoactive intestinal polypeptide (VIP)-secreting intestinal ganglioneuroma and the first in a so young infant.