TIPSS was successfully performed in a 10-year-old female cystic fibrosis (CF) patient with bleeding gastric varices due to portal hypertension; precipitation of portosystemic encephalopathy later unveiled the presence of a latent colonic stricture associated with high potency pancreatic enzymes. The unusual sequence of events resulting from the co-existence of two CF pathologies are described, and the implications of treatment discussed.
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