Juvenile polyposis of the colon (Weil's syndrome)

Juvenile polyposis of the colon (Weil's syndrome) is a rare disease, significantly different in its clinical and morphological picture from other types of familial multiple polyposis. Most family members who have juvenile polyposis of the colon later die from colon cancer. The disease manifests itself both in early childhood and at an older age. It should be noted that this disease is unexpectedly detected in young people who have not had a similar or similar disease in their family in the past (although it should be taken into account that 20-25 years ago the level of diagnosis of diseases, including those of the colon, was significantly lower than at present). Polyps in this disease (by the way, based on my own observations and other hereditary multiple polyposis of the digestive tract) are mainly, indeed, multiple (hundreds), in other cases there are significantly fewer (tens or several pieces) and, finally, in some patients (but with a typical hereditary anamnesis!) - single, which should be taken into account in their practical activities. A. M. Veake et al. in 1966 described 11 cases of this disease in 4 families. As it became clear after a detailed questioning, in the past some relatives died from colon cancer.

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Pathomorphology

Polyps in this disease are largely similar to those in multiple hereditary polyposis of the small intestine: they have abundant connective tissue stroma, a small number of conglomerates of intestinal glands of various sizes and mucus inside cystic cavities, and leukocytes.

Symptoms and treatment of juvenile colon polyposis

For some time the disease is asymptomatic. Then ulceration of polyps, intestinal bleeding, possible intestinal intussusception and obstruction (by large polyps) occur. In these cases, emergency hospitalization in a surgical hospital is necessary, and then, if there are no contraindications, surgical treatment. As in other cases listed above, it is necessary to examine all immediate relatives in order to identify asymptomatic hereditary polyposis. The patient and all his relatives with detected signs of colon polyposis need regular dispensary examination in order to promptly identify cancerous transformation of polyps despite good health. However, it should be noted that with this form of hereditary colon polyposis, complications are observed, judging by the literature, extremely rarely. In case of dyspeptic symptoms - symptomatic therapy, diet.