A total of 34 children with normal renal function underwent either gas
trocystoplasty or continent urinary reservoirs with stomach at our ins
titutions. Severe hypochloremic hypokalemic metabolic alkalosis develo
ped in 2 patients, manifested by intractable seizure disorder in 1 and
altered mental status with respiratory depression in 1. Symptoms deve
loped at 4 and 6 months, respectively. Despite severe alkalosis, urina
ry pH was less than 5.0 and fractional excretion of chloride remained
high in both patients. Resuscitation with sodium chloride, arginine hy
drochloride and potassium chloride restored electrolyte balance in les
s than 48 hours in both patients. Serum gastrin was slightly elevated
in 1 patient (137 pg./ml., normal 0 to 125) who responded to long-term
histamine-blocker therapy. The other patient had significant hypergas
trinemia (624 pg./ml.) with secondary hyperaldosteronism. Maximum dose
s of histamine blockers, oral replacement of sodium chloride and potas
sium chloride, and the proton pump inhibitor omeprazole failed to cont
rol recurrent bouts of severe hypochloremic metabolic alkalosis. This
patient ultimately underwent removal of three-quarters of the gastric
augmentation and replacement with ileum. Postoperatively, serum gastri
n levels and electrolytes reverted to normal. The pathophysiology of t
his potentially lethal complication is further discussed.