Surgical Treatment of Peutz-Jeghers Syndrome

ABSTRACT

Peutz-Jeghers syndrome (PJS) is characterized by the presence of hamartomatous gastrointestinal polyps and mucocutaneous pigmentation of the lips, buccal mucosa, and digits. It is a rare autosomal dominant disease with an incidence of 1/120,000 live-borns. This syndrome is known to require laparotomy because of intestinal obstruction, intestinal bleeding and recurrent intussusceptions. A 19-year-old male was referred to our hospital complaining of persistent abdominal pain and anemia. At age of 4, he had undergone a 10 cm small bowel resection from 50 cm distal to the Treitz ligament because of abdominal pain and intestinal bleeding due to intussusception. He was diagnosed as PJS based on the multiple intestinal hamartomatous polyposis and buccal hyperpigmentation. A small bowel series and abdominal computed tomography found a 6 cm sized mass and jejuno-jejunal intussusception on the duodeno-jejunal junction. Preoperative gastroduodenoscopy showed two polyps in the upper third of the stomach. Colonoscopic examination found 5 polyps with sizes of 4~8 mm from the ascending colon to the rectum. They were removed completely by colonoscopic snaring polypectomies, preoperatively. At exploration, we found 8 small bowel polyps within 100 cm from the Treitz ligament, 6 gastric polyps and 3 duodenal polyps; they were all removed using two jejunal enterotomies & intraoperative endoscopy. We found a total of 22 polyps from the stomach to the rectum. We performed polypectomies for 15 polyps over 0.5 cm and treated the remaining 7 polyps by electrocautery using intraoperative endoscopy without performing small bowel resection.