High cumulative risk and clinical feature of intussusception in patients with Peutz-Jeghers syndrome

Abstract

Objective To analyze the characteristics,risk and induced factors of intussusception in Peutz-Jeghers syndrome (PJS).Methods From March 2nd 2005 to May 25th 2013,a total of 130 patients with PJS were selected.The clinical data of patients were collected,which included gender,age,the diagnostic age of PJS,family history,the diagnostic age of intussusception,clinical features,location,treatment and the maximum diameter of polyps which caused intussusception.Kaplan-Meier was performed to analyze the cumulative risk of intussusception.Results Among 130 patients with PJS,90 cases had intussusception.The age when first time diagnosed was from four to 33,median age was 16.The cumulative risks of intussusception at 10,15,20,25 and 30 years were 12.308％ (16/130),31.538％(41/130),51.538％(67/130),64.615％(84/130) and 66.923％(87/130).There was no significant difference in the cumulative risk of intussusception between male and female; with family history and no family history (both P＞0.05).A total of 131 intussusception happened in 90 patients,of which diagnosed by surgery,imaging examination and history reviewer was 125,four and two times,respectively.The initial symptom of 111 times of intussusception was acute abdomen and 15 were abdominal pain and vomiting.The left five intussusception was found by regular:examination.One hundred and fifteen intussusception was in small intestine and 16 in colon.There was 127,three and one time treated with surgery,conservative treatment,endoscopic therapy (dual airbags intestinal endoscopic polypectomy),respectively.The maximum diameter of polyps which caused intussusception was from 15 to 70 mm,average 40 mm.Conclusions Intussusception of patients with PJS is at young age and with a high cumulative risk.Intussusception is generally caused by diameter over 15 mm polyps. Key words: Peutz-Jeghers syndrome; Intussusception; Endoscopy, gastrointestinal; Intestine,small; Intestinal polyps