Evaluation of Peutz-Jeghers syndrome in a family

Abstract

Five cases of Peutz-Jeghers syndrome in one family have been studied, and a review of the history and features of the syndrome are presented. This syndrome was detected in four of six siblings and in the father. Three family members underwent surgery after small-bowel obstruction was diagnosed. A gastric polyp was removed from a fouth family member. Rectal bleeding dominated the clinical picture in two patients. Colonic polyps were also found in the three patients who had small-bowel polyps. One of the four affected siblings had mucocutaneous pigmentation, but no evidence of intestinal polyps was detected. The five affected family members have been followed up for periods of 3-1/2 to 4-1/2 years.