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

Figure 1 The age distribution of 506 surgical procedures in patients with Peutz-Jeghers syndrome. Of the 506 surgical procedures performed, the majority (n = 388, 76.88%) were conducted on patients aged between 6 and 25 years. The number of surgical procedures increased markedly from the age of six years.

Figure 2 The natural cumulative incidence of the first and second surgical procedure in Peutz-Jeghers syndrome. A: The first surgical procedure in Peutz-Jeghers syndrome. The estimated cumulative natural surgical risk for Peutz-Jeghers syndrome was 5.0% (95% confidence interval [CI]: 2.9%-7.0%) at age 5, 20.6% (95%CI: 16.6%-24.4%) at age 10, 40.5% (95%CI: 35.5%-45.1%) at age 15, 58.0% (95%CI: 52.7%-62.7%) at age 20, 72.6% (95%CI: 67.3%-77.0%) at age 25, and 82.4% (95%CI: 77.0%-86.5%) at age 30. The annual growth rate of the cumulative incidence of surgery was 3.38% per year during the age range of 6-25 years, which was higher than the rates observed during the age range of 1-5 years (1.0% per year) or 26-30 years (1.96% per year); B: The second surgical procedure in Peutz-Jeghers syndrome. The estimated cumulative incidence was 3.7% (95%CI: 1.5%-5.8%) for a 1-year interval, 12.5% (95%CI: 8.6%-16.2%) for a 3-year interval, 20.3% (95%CI: 15.6%-24.8%) for a 5-year interval, 37.0% (95%CI: 30.8%-42.7%) for a 10-year interval, and 54.2% (95%CI: 46.8%-60.5%) for a 15-year interval. The annual growth rate of the cumulative incidence of a second surgery was 3.61% per year during the initial 15-year interval since the first surgery.

Figure 3 Cumulative incidence of second surgery stratified by age at first surgery. Patients were divided into two groups according to age at first surgery, and the younger group (patients who had their first surgery at age 9 or younger) had an increased risk of requiring a second surgery compared with the older group (patients who had their first surgery older than 9 years). The estimated cumulative incidence of a second surgery in the younger group was 78.15% (95%CI: 62.0%-87.44%) over a 15-year interval, in comparison to 46.9% (95%CI: 38.5%-54.1%) in the older group.