Natural surgical risks and age distribution in Chinese patients with Peutz-Jeghers syndrome: Real-world research based on a web survey.

Background: Complications arising from the polyps in Peutz-Jeghers syndrome (PJS) have historically been addressed through surgical treatment. Enteroscopic polypectomy is hypothesized to reduce the risk of surgery in PJS. However, the optimal timing for polyp screening and preventive intervention using enteroscopic polypectomy remains uncertain. This is primarily due to the extremely low incidence of the condition and the paucity of data regarding the natural risk of requiring surgery and its age distribution in PJS patients. In order to develop recommendations on the appropriate timing of polyp screening and preventive intervention in PJS, a comprehensive understanding of the natural surgical risks and their age distribution is essential.

Aim: To investigate the natural surgical risks associated with polyps in PJS and to clarify their age distribution.

Methods: A web-based open survey was launched to collect information from Chinese individuals suspected of having PJS. The questionnaire was distributed to the PJS instant messaging groups using a quick response code method. The data were analyzed using descriptive statistical methods, and the cumulative incidence of surgery was calculated using the Kaplan-Meier method.

Results: Of the 442 patients enrolled, 301 (68.10%) had undergone 506 surgical procedures prior to enteroscopy or the survey deadline. Among the 506 surgical procedures, 388 (76.68%) were performed on patients aged between 6 and 25 years. The cumulative incidence rates of the first surgical procedure at 5, 10, 15, 20, 25, and 30 years of age were 5.0% (95% confidence interval [CI]: 2.9%-7.0%), 20.6% (95%CI: 16.6%-24.4%), 40.5% (95%CI: 35.5%-45.1%), 58.0% (95%CI: 52.7%-62.7%), 72.6% (95%CI: 67.3%-77.0%), and 82.4% (95%CI: 77.0%-86.5%), respectively. The primary indications for the first surgical procedures were intussusception (81.40%), obstruction (13.95%), and gastrointestinal bleeding (4.65%). The cumulative incidence rates of requiring a second surgical procedure within 1, 3, 5, 10 and 15 years following the first surgical procedure were 3.7% (95%CI: 1.5%-5.8%), 12.5% (95%CI: 8.6%-16.2%), 20.3% (95%CI: 15.6%-24.8%), 37.0% (95%CI: 33.1%-45.3%), 54.2% (95%CI: 46.8%-60.5%), respectively. Patients who underwent their first surgical procedure at the age of nine years or younger presented an elevated risk of requiring a second surgical procedure ( < 0.01).

Conclusion: Chinese patients with PJS have a high natural risk of undergoing surgery. Without preventive intervention, these procedures may become necessary at an early age and may be repeated. Early screening and regular surveillance, with preventive intervention if necessary, should commence at six years of age.