The latter two groups commonly present with abdominal pain, weakness, rectal
bleeding, diarrhea, or rectal prolapse. Laboratory findings include anemia,
hypoalbuminemia, hypokalemia, and skin test anergy. Juvenile polyposis is a
premalignant condition with changes seen in children as young as 3 years of age.
Patients with generalized involvement require surgical intervention.
Subtotal colectomy and ileoproctostomy are the procedures of choice.
Patients with a small number of polyps may choose instead to undergo
periodic colonoscopy with colonoscopic polypectomy. Approximately 10 to
20% of affected patients have extracolonic abnormalities. These include
finger clubbing and hypertrophic pulmonary osteoarthropathy related to
pulmonary arteriovenous fistulas. Congenital cardiac defects, macrocephaly,
cleft lip/palate, extra teeth, arteriovenous malformations of the skin, gut
malrotation, psoriasis, and genitourinary abnormalities have been described.