Diversity in polyp pathology and distribution of Familial Juvenile Polyposis Syndrome

OBJECTIVE: Juvenile polyposis syndrome is a rare autosomal dominant disorder with incomplete penetrance. The aim of this study was to review our experience with juvenile polyposis syndrome with emphasis on the diversity of polyp pathology and distribution and the recommended treatment.

METHODS: Over the period January 1994 through February 2001, 10 family members were managed at Princess Basma Teaching Hospital, Irbid, Jordan. Two siblings with juvenile polyposis syndrome are discussed.

RESULTS: The polyps were unusually concentrated in the rectum. In one patient the polyps were purely of the adenomatous type. The father suffered from non-polyposis colon cancer at the age of 35.

CONCLUSION: Proctocolectomy and ileal pouch-anal anastomosis is recommended as the treatment of choice. Screening of juvenile polyposis syndrome patients and their relatives is emphasized for early detection of malignancy.