Peutz–Jeghers syndrome: management for recurrent intussusceptions
link.springer.com shows its articles on its own site.
Read the article on link.springer.com ↗Article · Jun 2024 · 1 min read
In brief
In brief
This retrospective study of nine pediatric PJS patients demonstrates a bowel-preservation approach to managing recurrent intussusceptions. The authors advocate for enterotomy with polypectomy over extensive resection to prevent short bowel syndrome, emphasizing conservative management and combined endoscopic-surgical strategies.
- PJS patients require multiple surgeries for recurrent intussusception; avoid extensive resection to prevent short bowel syndrome.
- Enterotomy with polypectomy after intussusception reduction preserves bowel length better than resection and anastomosis.
- Combined endoscopy (UGIE/LGIE) and intraoperative enteroscopy enables comprehensive polyp surveillance and targeted intervention.
- Conservative management under close observation is preferred when surgical indication is unclear to minimize cumulative bowel loss.
- Early aggressive resection should be avoided given diffuse GI polyp involvement; prioritize bowel-sparing techniques.
Written by the GCMD Library team from the article.
Abstract
Background
Peutz–Jeghers syndrome (PJS) is an autosomal dominant disorder characterized by hamartomatous gastrointestinal polyps along with the characteristic mucocutaneous freckling. Multiple surgeries for recurrent intussusception in these children may lead to short bowel syndrome. Here we present our experience of management in such patients.
Methods
From January 2015 to December 2023, we reviewed children of PJS, presented with recurrent intussusceptions. Data were collected regarding presentation, management, and follow-up with attention on management dilemma. Diagnosis of PJS was based on criteria laid by World Health Organization (WHO).
Results
A total of nine patients were presented with age ranging from 4 to 17 years (median 9 years). A total of eighteen laparotomies were performed (7 outside, 11 at our centre). Among 11 laparotomies done at our centre, resection and anastomosis of bowel was done 3 times while 8 times enterotomy and polypectomy was done after reduction of intussusception. Upper and lower gastrointestinal endoscopy (UGIE & LGIE) was done in all cases while intraoperative enteroscopy (IOE) performed when required. Follow-up ranged from 2 months to 7 years.
Conclusion
Children with PJS have a high risk of multiple laparotomies due to polyps’ complications. Considering the diffuse involvement of the gut, early decision of surgery and extensive bowel resection should not be done. Conservative treatment must be tried under close observation whenever there is surgical dilemma. The treatment should be directed in the form of limited resection or polypectomy after reduction of intussusception.