Familial Polyposis of the Entire Gastrointestinal Tract

Because of the extreme rarity of this condition it is difficult to determine whether it represents a variant of the usual "familial polyposis of the colon," but the literature was reviewed, three additional cases presented, and a thorough presentation of the subject follows.

Abstract

WITHIN the past two years, three similar cases of familial polyposis diffusely involving the entire gastrointestinal tract have been encountered. In all cases, innumerable polyposis were demonstrated in the stomach, small bowel, and colorectum by x-ray film studies. Because of the extreme rarity of this condition it is difficult to determine whether it represents a variant of the usual "familial polyposis of the colon." The literature was reviewed, three additional cases presented, and a thorough presentation of the subject follows. Report of Cases Case1.—A 9-year-old girl was first seen at the City of Hope Medical Center on March 27, 1966, with a history of frequent stools (three to four a day) and vague abdominal pain. She had passed mucus and blood through the rectum one week prior to admission. A barium enema showed extensive polyposis of the entire colon. The family history revealed that her father died at age