Medical Management of Cronkhite-Canada Syndrome

Abstract and Introduction

We report the case of a patient with Cronkhite-Canada syndrome (CCS) successfully treated with combination medical therapy. This rare, noninherited gastrointestinal polyposis syndrome is associated with characteristic ectodermal abnormalities. The etiology and pathogenesis of CCS are not known. No medical therapy has been shown to be consistently effective, and the disease is usually fatal. The patient described herein was successfully treated with a novel combination regimen consisting of H₁- and H₂-receptor antagonists, cromolyn sodium, prednisone, and suppressive antibiotics. This regimen has resulted in sustained improvement in symptoms, and follow-up endoscopy has shown regression of the diffuse polyposis. Our patient's response to such therapy may provide insight into the optimal treatment for CCS.

Cronkhite-canada syndrome (CCS) is a rare, noninherited gastrointestinal polyposis syndrome first described in 1955.^[1] The cardinal manifestations of the syndrome are gastrointestinal polyposis, diffuse skin hyperpigmentation, alopecia, and onychodystrophy.^[2] The polyps are nonadenomatous juvenile or hamartomatous polyps that occur throughout the gastrointestinal tract except for characteristic sparing of the esophagus.^[3] Presenting symptoms in CCS include diarrhea, weight loss, abdominal pain, hematochezia, and hypogeusia.^[3] Laboratory evaluation usually reveals evidence of gastrointestinal malabsorption, including anemia, electrolyte disturbances, and hypoproteinemia.^[3] The diagnosis is based on these features in the setting of characteristic clinical and endoscopic findings. The pathogenesis of CCS is unknown. The syndrome has never been described in relatives of an affected patient. Most patients are over the age of 50 at the time of diagnosis, with a mean age of 59 at onset, and there is a slight male predominance. Most patients are of European or Asian descent.^[3]

A number of therapeutic agents have been used to treat CCS, but no single medication has been shown to be consistently effective. Corticosteroids,^[3,4,5,6] anabolic steroids,^[3,7] various antibiotics,^[3,8] dietary supplementation,^[3] ranitidine,^[9] and symptomatic treatment for diarrhea and abdominal pain^[3] have all been used. Patients will rarely achieve remission while being treated with these agents and more commonly have only temporary symptomatic improvement. The optimal therapy for this condition, therefore, is not known.

Cronkhite-Canada syndrome tends to follow a progressive course, with a high rate of mortality despite treatment. Immune dysfunction with an increased susceptibility to infection has also been reported.^[10] Fatal complications of the disease include gastrointestinal bleeding, infection, and congestive heart failure.^[3] Adenomatous changes in the polyps and carcinomas occurring in the stomach and colon have been reported in association with CCS,^{[11,12,13,14,15]} but whether CCS is truly a precursor of malignancy is controversial.

South Med J. 2002;95(2) © 2002 Lippincott Williams & Wilkins

Cite this: Medical Management of Cronkhite-Canada Syndrome - Medscape - Feb 01, 2002.