Juvenile Polyposis of Colon (Weil Syndrome)

Juvenile polyposis of the large intestine (Weil syndrome) is a rare disease, in its clinical and morphological picture significantly different from other types of family multiple polyposis. Most family members who have a juvenile polyposis of the large intestine later died of colon cancer. The disease manifests itself 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 illness in the family in the past (although it should be borne in mind that even 20-25 years ago the level of diagnosis of diseases, including the colon, was much lower than at present). Polyps in this disease (by the way, on the basis of their own observations and with other hereditary multiple polyposis of the digestive tract) are basically, in fact, multiple (hundreds), in other cases they are much smaller (tens or several pieces) and, finally, in individual patients but with a typical hereditary anamnesis!) - are single, which should be taken into account in their practical activities. AM 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 with this disease largely resemble those with 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 the cystic cavities, leukocytes.

Symptoms and treatment of juvenile polyposis of the large intestine

Until some time the disease is asymptomatic. Then ulceration of polyps, intestinal bleeding, intestinal intussusception and obturation (large polyps) are possible. In these cases, urgent hospitalization in the surgical hospital and in the future, if there are no contraindications - surgical treatment. As in the other cases listed above, it is necessary to examine all direct relatives in order to identify an asymptomatically occurring hereditary polyposis. The patient and all his relatives with the revealed signs of polyposis of the large intestine need a regular dispensary examination with a view to timely detecting the carcinomatous transformation of polyps despite the well-being. True, it should be noted that with this form of hereditary polyposis of the colon, complications are observed, judging by the literature, extremely rare. With dyspeptic symptoms - symptomatic therapy, diet.