Mario Trompetto M.D., Giuseppe Clerico M.D., Ezio Ganio M.D., Alberto Realis Luc M.D.
Surgical approach to juvenile gastrointestinal polyposis
Colorectal Eporediensis Centre – Unit of Coloproctology – Ivrea – Italy
Familial adenomatous polyposis and Gardner syndrome are rare dominantly inherited syndromes characterized by hundreds to thousands of colonic adenomatous polyps. Colonic cancer occurs at a young age in both of them unless the colon is removed. Peutz-Jeghers syndrome and familial juvenile polyposis are inherited hamartomatous polyposis conditions with a low but well defined risk of colonic cancer. These four polyposis syndromes together account less than 1% of cases of colonic malignancies.
Familial juvenile polyposis is an autosomal dominant condition in which affected patients develop upper or lower gastrointestinal juvenile polyps, or both, and have a high possibility to develop a cancer of the gastrointestinal tract. Up to date the risk of G1 cancer in these patients has not been well defined because of the small number of families and lack of follow-up.
Single or few polyps can be frequently found in patients of any age and can occur also in infancy.
Polyps in children are classified as malignant or benign (tab. 1).
Table. 1. Pediatric tumors of the GI tract.
| ||mnNon familial|
| ||mnmnNon polyposis|
|Non polypoid|| |
Isolated inflammatory polyps (juvenile or retention polyps) are the most frequent and occur often in the first decade of life, particularly between 3 and 4 years of age. Some studies confirm their presence in 1% to 2% of children of this age. More than 60% of them are localized in the distal part of the colon or in the rectum.
Juvenile polyposis coli is characterized by multiple inflammatory polyps in the colon in contrast with generalized juvenile polyposis in which polyps can be found anywhere in the gastrointestinal tract.
Histologically juvenile polyps are hamartomas with distended mucus filled glands, oedema, cystic dilation and multiple blood vessels. The mucus retention and criptic dilation give them also the term of retention polyps.
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