Complications, treatment, and follow-up of peutz-jeghers syndrome: About 2 case reports

Abstract

INTRODUCTION: Peutz-Jeghers syndrome is an inherited disorder distinguished by hamartomatous polyps in the gastrointestinal tract and pigmented mucocutaneous lesions. Treatment of the polyps is never definitive, with most patients needing several laparotomies. For this reason, surgeons should be economical in terms of surgical resection to prevent a short bowel syndrome in the long run. In this paper, we report two observations of patients presented a Peutz Jeghers syndrome (PJS). CASES PRESENTATION: Case report 1: A 32-year-old women, who was operated on for an intestinal perforation related to a Peutz-jeghers hamartoma of the small bowel and was later re operated on for colonic intussusception, Case report 2: A 15-year-old patient that has been operated on three times already for small bowel intussusception and later for duodenal obstruction. CLINICAL DISCUSSION: In an attempt to reduce complications, the 2010 guidelines updated in 2021 by the European Hereditary Tumor group introduced obligatory monitoring by fibroscopy and colonoscopy associated with an entero-MRI or a videocapsule from the age of 8 years. Laparotomy is indicated when endoscopic treatment is impossible or in emergency setting. When surgery is indicated, intestinal resection should be reserved for rare cases in order to avoid short bowel syndrome. The association of an intraoperative endoscopic treatment is recommended by some authors. CONCLUSION: Peutz Jeghers syndrome is a rare entity with a complicated surveillance. Adequate polyp mapping is necessary for adequate planning of the treatment. The need for multiple laparotomies makes a comprehensive approach to surgery mandatory to prevent short bowel syndrome.