Stepwise management of children with Peutz–Jeghers syndrome

Background. Peutz–Jeghers syndrome in children is frequently complicated by small-bowel intussusception and obstruction, resulting in repeated operations and bowel resections.

Aim. To evaluate a stepwise management algorithm designed to reduce bowel resections through regular surveillance, prioritization of endoscopic treatment, and mandatory post-endoscopy monitoring.

Materials and methods. This retrospective single-center study included 30 pediatric patients managed at the National Medical Research Center for Children’s Health between 2016 and 2025. When available, earlier medical records from 2013 to 2026 were used to reconstruct prior interventions and history. Diagnosis was based on clinical and endoscopic criteria and/or family history; STK11 testing results were considered when available. The algorithm comprised initial abdominal ultrasound, an endoscopic step (including device-assisted enteroscopy when indicated) with polyp removal, mandatory ultrasound reassessment after endoscopy, and escalation to surgery in cases of persistent/ recurrent intussusception and/or inability to remove the causative polyp endoscopically, favoring bowel-sparing procedures (reduction, enterotomy with polypectomy without resection when no irreversible changes were present).

Results. Among the patient sample, boys predominated (63.3 %). STK11 alterations were detected in 90.0 % of patients. A de novo pattern was recorded in 53.3 %, while an inherited pattern was observed in 30.0 % of patients. Intussusception occurred in 86.7 % of patients; endoscopic interventions were performed in 90.0 %; bowel resection was documented in 70.0 %. A total of 155 hospitalizations with known admission and discharge dates were analyzed; the median length of stay was 8 days (IQR 7–11).

Conclusion. A stepwise strategy combining regular surveillance, endoscopic prioritization, and mandatory post-endoscopy monitoring enables timely identification of persistent intussusception and supports bowel-sparing surgical decisions. These measures are essential for reducing bowel resections in children with Peutz–Jeghers syndrome.

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