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Small intestinal hamartomatous polyp due to Peutz-Jeghers syndrome in middle childhood

Toshihiko Kakiuchi1, Takashi Akutagawa2

  • 1Department of Pediatrics, Faculty of Medicine Saga University Saga Japan.

Clinical Case Reports
|July 5, 2022
PubMed

Insights

Early small-bowel surveillance is crucial for individuals with Peutz-Jeghers syndrome (PJS). Consistent monitoring from age 8 can prevent serious complications like intussusception and avoid emergency surgery.

Area of Science:

  • Gastroenterology
  • Pediatric Oncology
  • Clinical Genetics

Background:

  • Peutz-Jeghers syndrome (PJS) is a rare genetic disorder.
  • PJS patients have an increased risk of gastrointestinal and extraintestinal cancers.
  • Small-bowel surveillance is recommended for PJS patients.

Observation:

  • A case of intussusception and strangulated ileus in an 11-year-old PJS patient.
  • This complication occurred despite the general recommendation for surveillance.

Findings:

  • Current surveillance guidelines for PJS may not be sufficient to prevent acute complications.
  • Intussusception can occur in PJS patients even with recommended surveillance protocols.

Implications:

  • Small-bowel surveillance in PJS patients should be rigorously implemented starting at age 8.
  • Timely and effective surveillance is critical to prevent severe complications and the need for urgent surgical intervention.
  • Further research may be needed to optimize surveillance strategies for PJS to mitigate risks of intussusception and ileus.

Keywords:
Peutz–Jeghers syndromeintussusceptionsmall‐bowel polypsmall‐bowel surveillance

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