PEUTZ-JEGHER’S POLYPOSIS: CHILDHOOD PRESENTATION OF ACUTE AND CHRONIC INTERMITTENT BOWEL OBSTRUCTION CAUSED BY INTESTINAL POLYPS IN RURAL SOUTH AFRICA: A CASE SERIES

Tebogo Thelele [1], Elliot Motloung [2]

[1] Department of Pediatric Surgery, University of Witwatersrand, Johannesburg, Gauteng, South Africa

[2] Department of Pediatric Surgery, Sefako Makgatho Health Science University, Pretoria, Gauteng, South Africa

Abstract

Introduction: Peutz-Jegher’s Syndrome (PJS) is a rare hereditary condition characterized by the development of gastrointestinal polyps and mucocutaneous freckling. These polyps can cause intussusception and acute bowel obstruction, requiring urgent intervention due to the high risk of bowel ischemia. This paper aims to report two different presentations of PJS in children who presented to the pediatric surgery department. One case involved the acute onset of bowel obstruction, while the other involved chronic intermittent bowel obstruction.

Case presentation: We present 2 cases of PJS that presented to our department. A 9-year-old boy with an acute history of symptoms of intestinal obstruction. Diagnostic laparoscopy revealed intussusception. We elected to convert to an open laparotomy and manual reduction of the intussusception. The polyp was resected, the bowel repaired, and the patient was subsequently discharged home and did not have any recurrence. The second was a 6-year-old boy with a 3-year history of abdominal pain and intermittent bowel obstruction. A large polyp was discovered and resected during laparotomy.

Conclusion: PJS may present differently in patients due to the size andlocation of the polyps. It is essential to maintain high clinical suspicion and refer these patients for long-term surveillance by a pediatric surgeon.