Related Experiment Video
Updated: Jun 24, 2025
Colonial Wig Pancreaticojejunostomy
Published on: March 12, 2019
Peutz-Jeghers syndrome: management for recurrent intussusceptions
Anju Verma1, Pujana Kanneganti2, Basant Kumar2
1Department of Paediatric Surgery, SGPGIMS, Lucknow, 226014, India. anju_v2006@rediffmail.com.
Insights
Peutz-Jeghers syndrome (PJS) patients often require multiple surgeries for intussusception. Conservative management and limited polypectomy are recommended over extensive bowel resection to prevent short bowel syndrome.
Area of Science:
- Pediatric Surgery
- Gastroenterology
- Genetics
Background:
- Peutz-Jeghers syndrome (PJS) is an autosomal dominant disorder.
- Characterized by hamartomatous gastrointestinal polyps and mucocutaneous freckling.
- Recurrent intussusception in PJS can lead to short bowel syndrome due to multiple surgeries.
Purpose of the Study:
- To present management experience in pediatric PJS patients with recurrent intussusceptions.
- To address the management dilemma in these complex cases.
- To evaluate outcomes of different surgical and conservative approaches.
Main Methods:
- Retrospective review of pediatric PJS patients with recurrent intussusceptions (January 2015 - December 2023).
- Data collected on presentation, management, and follow-up.
- Diagnosis based on World Health Organization (WHO) criteria.
Main Results:
- Nine patients (age 4-17 years) presented with recurrent intussusceptions.
- Eighteen laparotomies performed; 11 at the center.
- Limited resection or enterotomy with polypectomy was favored over extensive resection (8/11 cases).
Conclusions:
- Children with PJS face high risk of multiple laparotomies due to polyps.
- Extensive bowel resection should be avoided due to diffuse gut involvement.
- Conservative management and limited polypectomy are preferred when surgical dilemma exists.
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.
Related Concept Videos
01:21Inflammatory Bowel Disease V: Surgical Management
Here are some common surgical interventions for IBD:
01:25Peptic Ulcer Disease V: Surgical Management and Nursing Care
Surgical Interventions for Peptic Ulcer Disease
01:15Enteral Nutrition II: Nasointestinal and Gastrostomy Feeding
Nasointestinal Feeding
Nasointestinal feeding involves placing a tube...
01:26Peptic Ulcer Disease IV: Management
The therapeutic approach involves ensuring adequate rest, implementing drug therapy, promoting smoking cessation, making dietary modifications, and emphasizing long-term follow-up care.
Pharmacological management
The prevailing therapy for peptic ulcers involves a combination of managing the patient's current...
01:29Gastroesophageal Reflux Disease II: Clinical Features and Management
Clinical Manifestations
GERD presents itself in a multitude of ways, with symptoms varying from person to person. The hallmark symptoms are...
01:21Barrett Esophagus-II: Clinical Manifestations and Management
To diagnose Barrett's esophagus, healthcare providers often recommend an endoscopy for those showing symptoms of acid reflux. The procedure...