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Management of Peutz-Jeghers syndrome. Experience with patients from the Danish Polyposis Register
I Rebsdorf Pedersen1, A Hartvigsen, B Fischer Hansen
1Department of Surgical Gastroenterology, Hvidovre Hospital, Copenhagen, Denmark.
Insights
A new Danish follow-up program for Peutz-Jeghers syndrome (PJS) tailors screening frequency to polyp regrowth rates. This personalized approach aims to optimize patient care and reduce unnecessary examinations for PJS patients.
Area of Science:
- Gastroenterology
- Clinical Medicine
- Genetics
Background:
- Peutz-Jeghers syndrome (PJS) is a rare genetic disorder characterized by polyps in the gastrointestinal tract.
- Effective surveillance strategies are crucial for managing PJS due to an increased risk of gastrointestinal and extraintestinal cancers.
Purpose of the Study:
- To introduce and describe a newly developed, literature-informed follow-up program for Danish patients diagnosed with Peutz-Jeghers syndrome.
- To personalize surveillance intervals based on individual polyp regrowth rates and symptom presentation.
Main Methods:
- The program integrates upper and lower gastrointestinal endoscopy, small bowel enema, and surgical interventions like laparotomy with intraoperative total enteroscopy and polypectomy when indicated.
- Extraintestinal examinations including pelvic, cervical smear, breast, and testicular exams are initiated after age 30.
Main Results:
- The follow-up program stratifies patients based on polyp regrowth rates and symptom presence, leading to tailored examination frequencies.
- Asymptomatic patients with slow polyp regrowth undergo less frequent surveillance, optimizing resource allocation and patient burden.
Conclusions:
- The implemented follow-up program offers a personalized and evidence-based approach to managing Peutz-Jeghers syndrome in Denmark.
- This tailored strategy aims to enhance early detection of complications while minimizing patient exposure to invasive procedures.
Abstract:
Danish patients with Peutz-Jeghers syndrome are registered in the Danish Polyposis Register. We have initiated a new follow-up programme based on recent literature including our own cases. This has been adjusted to the regrowth rate of the polyps whereby patients with no symptoms and low regrowth rate are followed with few examinations. The programme includes upper and lower gastrointestinal endoscopy, small bowel enema and in cases with large or symptomatic polyps, laparotomy with intraoperative total enteroscopy with polypectomy as required. Pelvic examination, cervical smear, breast and testicular examination are carried out after the age of 30 years.