Peutz-Jeghers Syndrome in a 7-Year-Old Male Presenting with Jejunojejunal Intussusception and a Giant Incidental Meckel’s Diverticulum: A Case Report

Articles in Press

Document Type : case report

Authors

1 Department of Surgery, School of Medicine, Ardabil University of Medical Sciences, Ardabil, Iran.

2 Department of Pediatric Surgery, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

3 Department of Pediatric Gastroenterology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

4 Department of Pathology, School of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.

10.22038/jpp.2026.94847.5632

Abstract

Background: Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by gastrointestinal hamartomatous polyposis, most commonly the small intestine. Although intussusception is a recognaized complication of PJS, primary intussusception is uncommon in children older than three years of age, necessitating a high index of suspicion for underlying pathological lead points.
Case Presentation: We report a case of type B1 PJS in a seven-year-old male who initially presented with an acute abdomen. Subsequent laparotomy revealed a jejunojejunal intussusception along with an incidentally discovered large Meckel’s diverticulum. Notably, the patient lacked the classic mucocutaneous hyperpigmentation typically associated with PJS.
Conclusion: Approaching an acute abdomen in a pediatric patient requires a careful diagnostic evaluation. In children older than three years presenting with intussusception, secondary causes such as PJS must be considered, even in the absence of pathognomonic mucocutaneous pigmentation.

Keywords

References

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Journal of Pediatric Perspectives

Articles in Press, Accepted Manuscript
Available Online from 01 June 2026

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