A 41-year-old man with
Cronkhite-Canada syndrome presented with multiple juvenile
polyps with hyperplastic and adenomatous changes throughout his stomach and entire colorectum.
Dysgeusia was recognized and the degree of
hypoproteinemia was remarkable. A barium enema study and colonofiberscopy also revealed an advanced
cancer in the rectum.
Chronic hepatitis B and
membranous glomerulonephritis were also present. It was difficult to design a conservative protocol using
steroids for the treatment of
protein-loosing enteropathy because the patient was a hepatitis B virus carrier. As a result, a subtotal
colectomy while preserving the cecum with cecorectal anastomosis was performed. Pathologically, the ulcerated
rectal tumor was a moderately differentiated
adenocarcinoma with invasion into the muscularis propria. Most
polyps showed cystically dilated glands without dysplasia or edematous stroma with inflammatory cell infiltration. A few
polyps were juvenile-type
polyps with
adenoma components. Although no remarkable improvement was observed in the
hypoproteinemia postoperatively, an alpha1-antitrypsin clearance test showed a significant decrease in
protein loss from the gastrointestinal tract, which was only about one third of the loss seen preoperatively. These findings lead us to conclude that when improvement using
conservative treatment can be neither obtained nor is expected, then the use of surgery should be considered when treating patients with
Cronkhite-Canada syndrome.