A 3-year-old girl presents with refusal to walk, weakness, leg
cramps, and a 20-month history of chronic diarrhea. Initial
laboratory workup revealed a significant hypokalemia.
Thoracic ganglioneuroma presenting with the watery diarrhea,
hypokalemia, and achlorhydria (WDHA) syndrome.
Findings indicative of a posterior mediastinal mass on plain film
and chest computed tomography (CT) scans suggest the diagnosis of a
neurogenic tumor, as 95% of pediatric posterior mediastinal masses
are of neurogenic origin. The primary differential considerations
are tumors of sympathetic ganglion origin (i.e., neuroblastoma,
ganglioneuroblastoma, and ganglioneuroma).1
patient's age favored a neuroblastoma or ganglioneuroblastoma, as
ganglioneuroma tends to occur in older children. Plain film
findings of thoracic neuroblastoma include a posterior mediastinal
mass, possibly associated with ribs that are splayed or eroded or
both; calcifications; and enlarged intervertebral foramina in cases
with intraspinal extension of tumor. CT demonstrates calcification
in approximately 90% of neuroblastomas. Magnetic resonance (MR)
imaging is the test of choice for thorough evaluation of
intraspinal extension of tumor.2
The WDHA syndrome is an unusual paraneoplastic condition caused by
excess vasoactive intestinal peptide (VIP) secretion by certain
tumors, including the
neuroblastoma-ganglioneuroblastoma-ganglioneuroma spectrum in
children and non-beta pancreatic islet cell tumors, squamous cell
tumors of the lung or esophagus, cutaneous mast cell tumors, and
pheochromocytomas in adults.3
Ganglioneuromatosis of the
small intestine and pancreas, causing hypersecretion of VIP, has
The WDHA syndrome has also been referred
to as the vipoma syndrome, pancreatic cholera, the watery diarrhea
syndrome, and the Verner-Morrison syndrome after its discoverers in
Clinically, the onset of WDHA syndrome is
typically insidious and diagnosis is often delayed for months or
even years. The clinical hallmark is an unremitting secretory
diarrhea that persists despite fasting. Diagnosis requires
documentation of elevated serum VIP levels with subsequent
localization of the VIP-secreting tumor. Surgical resection is
usually curative, as it was in this case.
- Hedlund GL, Griscom NT, Cleveland RH, Kirks
DR: Respiratory system. In: Kirks DR (ed): Practical
Pediatric Imaging: Diagnostic Radiology of Infants and Children, ed
3, pp 790-792. Philadelphia, Lippincott-Raven, 1998.
- Abramson SJ:Adrenal neoplasms in children.
Radiol Clin North Am 35:1415-1453, 1997.
- Grier JF:WDHA (watery diarrhea, hypokalemia,
achlorhydria) syndrome: Clinical features, diagnosis, and
treatment. South Med J 88:22-24, 1995.
- Shulman DI, McClenathan DT, Harmel RP, et al:
Ganglioneuromatosis involving the small intestine and pancreas of a
child and causing hypersecretion of vasoactive intestinal
polypeptide. J Pediatr Gastroenterol Nutri 22:212-218, 1996.
- Castleberry RP:Biology and treatment of
neuroblastoma. Pediatr Clin North Am 44:919936, 1997.