Sudden death from hyponatremia and hypokalemia in a woman with Gardner syndrome

The authors present the case of a 39-year-old woman with Gardner syndrome w ho died from marked hyponatremia and hypokalemia. Gardner syndrome is a rar e variant of the familial adenomatous polyposis syndrome in which the affec ted individual develops thousands of polyps within the gastrointestinal tra ct, with a 100% risk of eventual malignant change. Individuals with Gardner syndrome also develop a variety of extra gastrointestinal abnormalities. I n the case presented, a woman with a clinical history of Gardner syndrome w ho had previously undergone a total colectomy with ileorectal anastomosis p resented to the hospital with a recent history of sore throat, fever, diarr hea, and abdominal pain. The symptoms were considered clinically to be due to a viral gastroenteritis. She was admitted to the hospital, where she had episodes of collapse believed to be vasovagal in origin. She suffered a ca rdiorespiratory arrest and died 24 hours after admission. After her death, electrolyte estimation performed on blood taken shortly before death reveal ed severe hyponatremia and hypokalemia. Postmortem examination showed the g astric mucosa to be virtually covered by innumerable adenomatous and hyperp lastic polyps. Fewer polyps were seen within the small bowel. There was no evidence of malignancy. The features were consistent with Gardner syndrome. Hyponatremia and hypokalemia have been described in patients with villous adenomas and in familial adenomatous polyposis syndromes associated with nu merous colonic polyps. The cause of death in this case was considered to be hyponatremia and hypokalemia associated with florid gastric polyps in a wo man with Gardner syndrome. Viral gastroenteritis contributed to the death b y causing further electrolyte depletion. To the best of the authors' knowle dge, death in Gardner syndrome has not been described as attributable to su ch metabolic disturbance, in particular in those who have only gastric, sma ll bowel, and rectal polyps remaining after total colectomy.