OBJECTIVE: To describe the demographic, clinical characteristics and associated diseases in eight patients with the Peutz-Jeghers syndrome seen at the INCMNSZ and literature review. Setting: National Institute of Health. DESIGN: Description of eight cases of Peutz-Jeghers syndrome and literature review. MATERIAL AND METHODS: A retrospective review of all the discharge diagnosis was doing between January 1987 to February 2004. The diagnosis of Peutz-Jeghers syndrome was made on clinical and anatomical grounds. The clinical features, follow-up, treatment and last visit clinical status were analyzed in all the patients. PRESENTATION OF CASES: Eight patients with the Peutz-Jeghers syndrome were investigated, five women and three men. The median of time at diagnosis was 31 years-old (range, 26-37). All the patients had mucocutaneous pigmentation and hamartomatous polyps. The ileum and large bowel were the most frequent sites of the polyps, and there were generally sessile and pedunculated. The major abdominal symptoms were abdominal pain, GI bleeding, intestinal obstruction, weight loss and intussusception. One case of small-bowel cancer and one of serous cystadenoma of the ovary were detected. Surgical interventions were doing in five patients; the most frequent indication was polyp-induced bowel obstruction. All the patients are alive to date.

Clinical classification of Peutz-Jeghers syndrome.Nan Fang Yi Ke Da Xue Xue Bao. 2006 Jan;26(1):79-81.

OBJECTIVE: To propose the clinical classification of Peutz-Jeghers syndrome (PJS). METHODS AND RESULTS: Retrospective analysis of 52 patients with PJS admitted in Nanfang Hospital from 1980 to 2003 was conducted. Twenty-four patients were found to have family history of PJS, who had a mean age of 19 years. In the PJS patients, the incidence of gastric polyps was 64.4%, colorectal polyps 76%, and small bowel polyps 95%. The number of polyps was above 50 in 19 of the 31 patients with gastric polyps, in 18 of the 38 patients with colorectal polyps, and in 8 of the 19 patients with small bowel polyps. The pathology of the majority of the polyps (63/108) was characterized by hamartomas, and the incidence of malignancy was 13.5% in the PJS patients. CONCLUSIONS: PJS can be classified according to family history and location, pathology, and number of the polyps. As most patients with over 50 polyps require surgical intervention, 50 polyps is recommended as the criteria for PJS classification. Endoscopic surgery may suffice for management of patients with fewer polyps (<50), while in patients with more polyps or small bowel polyps, open surgery combined with intraoperative endoscopic surgery is recommended.