Cronkhite-Canada syndrome with hypothyroidism: Clinical, endoscopic and pathologic pitfalls

Abstract

Cronkhite-Canada Syndrome (CCS) is a rare non-hereditary protein-losing enteropathy, and characterized by ectodermal and gastrointestinal abnormalities. The etiology remains unclear, however, some studies suggest the likelihood of immune dysregulation. The diagnosis of CCS is challenging because the clinical symptoms are non-specific and the endoscopic and pathologic findings can overlap with other processes. This can potentially lead to delay in diagnosis, resulting in delayed patient management. We reported a 74-year-old male presented with initial symptoms of abdominal pain and nausea, and later with diarrhea, weight loss, hypothyroidism and multiple strokes. Gastric Antral Vascular Ectasia (GAVE) was suspected on the initial endoscopy. The diagnosis of CCS was rendered on the third biopsy. After steroid treatment, the patient’s clinical symptoms were relieved. In this paper, we discussed the clinical, endoscopic and pathologic pitfalls of CCS.