Peut-Jeghers Syndrome

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  • autosomal dominant
  • benign intestinal polyps
    • hamartomas
    • usually jejunal
    • may be GI obstruction
    • may be massive GI haemorrhage
  • pigmentation on lips, oral mucosa, face, palms and soles
  • malignant change in up to 3%
    • typically from duodenal polyps

Treatment

  • usually conservative
  • local excisions may be needed
 

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