1. Background

• Juvenile Polyposis Syndrome is classified into three disorders each associated with inflammatory polyps:
  1. Juvenile Polyposis of Infancy
  2. Juvenile Polyposis Coli
  3. Gastrointestinal Polyposis

2. Polyps:

• can be divided into several types:
  1. Benign

  • Juvenile (Inflammatory)
  • Hamartomatous
  • Inflammatory Fibroid
  • Lymphoid
  2. Neoplastic

  • Adenomatous - tubular
    • tubulovillous
    • villous
• elevated localized lesions either pedunculated (stalked) or sessile
• usually arise from the mucosa (epithelial) but can also involve the submucosa
• histologic exam needed to identify the type of polyp
• cell type may vary within a polyp
• may present as single, multiple, or as a polyposis
• malignant change probably starts in the polyp head with initial spread and submucosal invasion of the bowel wall before transmural, local lymphatic, and metastatic spread

1. Gastrointestinal Manifestations

2. Other Manifestations

• anorexia, failure to thrive, weight loss
• iron deficiency anemia
• no known associated anomalies

1. Colonoscopy

• procedure of choice

2. Imaging Studies

• barium enema with air contrast (Double Contrast)

3. Biospy

• inflammatory +/- adenomatous polyps
• areas affected: colon and rectum (one third within reach of a finger)

4. Serum

• microcytic hypochromic anemia
• reduced serum iron and ferritin with elevated transferrin

1. Supportive

• iron supplementation for anemia
• nutritional support

2. Surgery

• most polyps infarct spontaneously and are passed in the stool
• if significant complications such as an adenomatous polyp
  • snare-loop polypectomy with diathermy