A case of idiopathic portal hypertension complicated by cronkhite-canada syndrome presenting with refractory ascites

The patient was a 40-year-old woman who initially presented with ascites in September 2016. She was observed for suspected fatty liver disease and IgA nephropathy. In April 2023, she was admitted to the hospital after sustaining a self-inflicted injury. She presented with ascites and impaired liver and kidney function. A liver biopsy revealed no significant fibrosis, and she was diagnosed with idiopathic portal hypertension (IPH). The ascites did not improve with diuretics or paracentesis. A colonoscopy revealed multiple hyperplastic polyps, and a protein leakage scintigraphy identified protein leakage from the sigmoid colon. This led to a diagnosis of Cronkhite-Canada syndrome (CCS). She presented with characteristic skin symptoms of CCS, including nail atrophy and taste disturbances. Prior to the onset of symptoms, she reported experiencing psychological stress. Steroid therapy improved the ascites and pleural effusion, and the liver dysfunction gradually improved. This case suggests that an autoimmune background may have triggered or exacerbated the IPH and that CCS should be considered in the differential diagnosis of refractory ascites.