High Cumulative Risk of Intussusception in Patients With Peutz–Jeghers Syndrome: Time to Update Surveillance Guidelines?

Abstract

PJS patients carry a high cumulative intussusception risk at young age. Intussusceptions are generally caused by polyps >15 mm and treatment is mostly surgical. These results support the approach of enteroscopic surveillance, with removal of small-intestinal polyps >10-15 mm to prevent intussusceptions. The effect of such an approach on the incidence of intussusception remains to be established in prospective trials.

Double-balloon enteroscopy in the management of patients with Peutz-Jeghers syndrome: a retrospective cohort multicenter study

“…Intussusceptions are generally caused by polyps larger than 15 mm (with polyp size probably being the most important risk factor for small-bowel intussusception), present predominantly as an acute abdomen and lead to surgical emergencies (8). These findings support the rationale of enteroscopic surveillance to prevent complications.…”

“…PJS patients carry a high cumulative intussusception risk at young age (50 % at the age of 20 years) (8). Intussusceptions are generally caused by polyps larger than 15 mm (with polyp size probably being the most important risk factor for small-bowel intussusception), present predominantly as an acute abdomen and lead to surgical emergencies (8).…”

“…Gastrointestinal polyps may cause gastrointestinal bleeding, anemia and abdominal pain due to intussusception, obstruction or infarction (5,7). Half of patients with PJS experience symptoms before 20 years of age, mainly due to obstruction and intussusception, leading to multiple laparotomies with intestinal resection that can ultimately result in short-bowel syndrome and/or severe adhesions (5,8).…”

Interventional balloon-enteroscopy

“…27,28 The effect on the incidence of intussusceptions remains to be established in prospective trials, though. 29 Adenoma or adenocarcinoma were observed in 9 of 30 polyps (30.0%) > 20 mm and in 1 of 80 polyps (1.3%) ≤20 mm and were resected endoscopically. 30 Crohn's disease is a chronic relapsing inflammatory disease of the intestinal tract and involvement of the small bowel may cause significant morbidity.…”

Adult Intussusception in Patients with Peutz-Jeghers Syndrome: Case Series and Review of Literature

“…They may also show recurrent intussusception that needs repeated operations [19] [20] [21]. Early diagnosis of PJS associated with intussusception is important to allow for early intervention and to prevent further complications [22] [23]. Including symptoms of intussusception, there are at least two of the following clinical criteria that should be present: a family history of the syndrome, melanin deposits, and bowel polyposis [5].…”