Watery Diarrhea Hypochloremia Achlorhydria Syndrome in an 18-Month-Old Thai Girl with Chronic Secretory Diarrhea

Main Article Content

Anundorn Wongteerasut
Nopaorn Phavichitr
Danai Meekaewkunchorn


Watery diarrhea, associated with hypokalemia and achlorhydria (WDHA) syndrome, is caused by a hypersecretory state of the vasoactive intestinal peptide (VIP). The most usual cause of this condition involves VIP-producing tumors and a rare cause of persistent diarrhea in the pediatric age group. We reported a case of an 18-month-old girl who presented profound chronic secretory diarrhea along with severe fluid-electrolyte imbalance. Physical examination revealed severe dehydration and an abdominal mass palpated at the left upper part of the abdomen. Low stool osmotic gap, hypokalemia as well as metabolic acidosis were demonstrated. Abdominal ultrasonography and computerized tomography further documented a retroperitoneal mass at the left suprarenal area. Octreotide and chemotherapy were provided without clinical response; thus, surgical exploration for tumor removal was performed 2 months later. The histologic report identified the mass as ganglioneuroma. Additionally, the stool output and fluid electrolyte disturbance were greatly reduced after the surgical operation. WDHA syndrome is rarely prevalent among children; however, precise physical examination and imaging are important parts of the diagnosis. Tumor removal is also the treatment of choice for this condition.

Article Details

รายงานผู้ป่วย (Case Report)


1. Bloom SR, Polak JM, Pearse AG. Vasoactive intestinal peptide and water diarrhea syndrome. Lancet 1973;11:14-6.
2. Scheibel E, Rechnitzer C, Fahrenkrug J, Hertz H. Vasoactive intestinal polypeptide (VIP) in children with neural crest tumours. Acta Paediatr Scand. 1982;71:721-5.
3. Verner JV, Morrison AB. Islet cells tumor and a syndrome of refractory watery diarrhea and hypokalaemia. Am J Med. 1958;25:374-80.
4. Belei OA, Heredea ER, Boeriu E, Marcovici TM, Cerbu S, Mărginean O, et al. Verner-Morrison syndrome. Literature review. Rom J Morphol Embryol. 2017;58:371-6.
5. Swift PG, Bloom SR, Harris F. Watery diarrhea and ganglioneuroma with secretion of vasoactive intestinal peptide. Arch Dis Child 1975;50:896-9.
6. Petersom HD, Collins OD 3 rd. Chronic diarrhea and failure to thrive secondary to ganglioneuroma. Arch Surg. 1967;6:934-6.
7. Sackel SG, Manson JE, Harawi SJ, Burakoff R. Watery diarrhea syndrome due to an adrenal pheochromocytoma secreting vasoactive intestinal polypeptide. Dig Dis Sci 1985;30:1201-7.
8. Said SI, Faloona GR. Elevated plasma and tissue levels of vasoactive intestinal polypeptide in the watery diarrhea syndrome due to pancreatic bronchogenic and other tumors. N Engl J Med 1975;293:155-60.
9. Iida Y, Nose O, Kai H, Okada A, Mori T, Lee PK, et al. Watery diarrhoea with a vasoactive intestinal peptide-producing ganglioneuroblastoma. Arch Dis Child. 1980;55:929-36.
10. Parbhu SK, Adler DG. Pancreatic neuroendocrine tumors: contemporary diagnosis and management. Hosp Pract (1995). 2016;44:109-19.
11. de Herder WW, Rehfeld JF, Kidd M, Modlin IM. A short history of neuroendocrine tumours and their peptide hormones. Best Pract Res Clin Endocrinol Metab. 2016;30:3-17.
12. Yeh PJ, Chen SH, Lai JY, Lai MW, Chiu CH, Chao HC, et al. Rare Cases of Pediatric Vasoactive Intestinal Peptide Secreting Tumor With Literature Review: A Challenging Etiology of Chronic Diarrhea. Front Pediatr. 2020;8:430.
13. Zhang WQ, Liu JF, Zhao J, Zhao SY, Xue Y. Tumor with watery diarrhoea, hypokalaemia in a 3-year-old girl. Eur J Pediatr. 2009;168:859-62.
14. Funato M, Fujimura M, Shimada S, Takeuchi T, Kozuki K, Iida Y. Rapid changes of serum vasoactive intestinal peptide after removal of ganglioneuroblastoma with watery-diarrhea-hypokalemia-achlorhydria syndrome in a child. J Pediatr Gastroenterol Nutr. 1982;1:131-5.
15. Yamaguchi K, Abe K, Otsubo K, Haniuda C, Suzuki M, Shimada A, et al. The WDHA syndrome: clinical and laboratory data on 28 Japanese cases. Peptides. 1984;5:415-21.
16. Kawahara S, Ueki T, Tsutsumi K, Oda T, Kobayashi S, Fujisawa T, et al. Use of a somatostatin analog to improve a patient's condition and the subsequent diagnosis of pancreatic VIPoma:a case report. Nihon Shokakibyo Gakkai Zasshi. 2020;117:84-91.
17. Várkonyi TT, Velösy B, Nagy I, Németh J, Lonovics J. Verner-Morrison (WDHA) szindróma diagnózisa és kezelése Sandostatin és streptozotocin adagolással [Diagnosis of Verner-Morrison syndrome (VDHA) and its treatment with sandostatin an streptozocitin]. Orv Hetil. 1993;134:2599-602.
18. Decarolis B, Simon T, Krug B, Leuschner I, Vokuhl C, Kaatsch P, et al. Treatment and outcome of Ganglioneuroma and Ganglioneuroblastoma intermixed. BMC Cancer. 2016 Jul 27;16:542.
19. Kogner P. Neuropeptides in neuroblastomas and ganglioneuromas. Progr Brain Res. 1995;104:325-38.
20. Lacey SR, Gribble TJ, Kosloske AM. Favorable prognosis of vasoactive intestinal peptide-secreting ganglioneuroblastoma. Pediatr Surg Int. 1989;4:217-9.