A Syndrome of Diarrhea, Anasarca, and Nail Dystrophy: A Curious Case of Cronkhite-Canada Syndrome

Document Type

Conference Proceeding

Publication Date

10-25-2023

Publication Title

Am J Gastroenterol

Abstract

Introduction: Cronkhite Canada Syndrome (CCS) is an extremely rare disorder only over 500 cases reported worldwide. It is characterized by multiple gastrointestinal polyps including inflammatory, hyperplastic, hamartomatous, and adenomatous polyps. Other features include protein-losing enteropathy, alopecia, and nail atrophy. This is a non-inherited disease with about 2-third of cases seen in the Japanese population. Case Description/Methods: A 50-year-old White man with a history of recurrent Clostridioides difficile infections presented to the hospital with 7 months of worsening diffuse anasarca, diarrhea, malnutrition, nail pitting, and eventual nail loss (Figure 1A). He has had many admissions for similar symptoms in the past few months and has been treated for recurrent C. difficile. infections. Laboratory evaluation on this presentation revealed severe electrolyte derangements including hyponatremia (130 mmol/L), hypocalcemia (6.5 mg/dL), hypoalbuminemia (1.8 g/dL), vitamin A and E deficiency (< 13 and < 459 ug/dL, respectively). C. difficile testing was negative. CT scan of his abdomen and pelvis showed severe gastric wall thickening, perigastric and mesenteric adenopathy, and multiple thickened and enhancing distal small bowel loops and cecum. Patient underwent endoscopy and colonoscopy that showed innumerable gastric, small bowel, and colonic polyps. Biopsies were taken from polypoid areas of the stomach, duodenum, and colon, and background colonic mucosa. Separate fragments of polypoid and non-polypoid colonic mucosa as well as gastric and duodenal biopsy fragments from polypoid mucosa revealed lamina propria edema, acute inflammation, cystically dilated glands and reactive changes, suggestive of hamartomatous polyps (Figure 1B). Findings were overall consistent with a diagnosis of CCS. Patient was started on total parenteral nutrition and was treated with intravenous diuresis with overall improvement in peripheral edema. He was discharged home with plans to follow up with a genetics specialist and gastroenterologist. Discussion: CCS is a very rare disease with an unknown etiology, and there is no uniform consensus on treatment. It is important to consider CCS when a patient presents with diarrhea, anasarca, and dermatological changes. In addition, alternative means of nutrition should be considered early in these patients to help recuperate electrolyte and fluid losses. Patients should be counseled on the importance of surveillance as the potential malignant nature of the polyps remains controversial.

Volume

118

Issue

10

First Page

S1990

Last Page

S1991

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