VIP-Secreting Ganglioneuroblastoma as an Unusual Cause of Watery Diarrhoea in Childhood

Koen Huysentruyt, Wim Arts, Jutte van der Werff ten Bosch, Willy Van de Casseye, Francis Lemmens, Jan De Koster, Yvan Vandenplas, An Van Damme


A 15-month-old boy with a 2-month history of watery diarrhoea presented with cachexia, hypokalaemia, hypochloraemia and metabolic acidosis. During a 2-week investigation period infection, celiac disease and cystic fibrosis were excluded and trial therapy with antibiotics, probiotics and dietary measures proved unsuccessful. This led to the suspected diagnosis of a vasoactive intestinal polypeptide (VIP)-secreting tumour with watery diarrhoea, hypokalaemia, achlorhydria (WDHA)-syndrome. MRI showed an infrarenal mass infiltrating the neuroforamen, which on pathological examination was consistent with a VIP-secreting neuroganglioblastoma. Serum VIP and urinary catecholamine levels were elevated. Treatment consisted of 2 courses of chemotherapy and a partial resection of the tumour, following which the diarrhoea disappeared. Refractory watery diarrhoea that persists during fasting in a young child should alert to the diagnosis of WDHA, and prompt investigations to diagnose a VIP-secreting tumour, one of the most common causes of secretory diarrhoea in the developed world. Timely diagnosis could avoid morbidity due to unnecessary investigations and protracted diarrhoea.

J Med Cases. 2013;4(4):234-236


Child; Ganglioneuroblastoma; Neuroblastoma; Secretory diarrhoea; Vasoactive intestinal polypeptide; Vipoma; WDHA syndrome

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