Peutz-Jeghers syndrome: management for recurrent intussusceptions.

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.

Safety and Efficacy of Enhanced Recovery after Surgery Protocol in Pediatric Patients Undergoing Hepato-Pancreatico-Biliary Surgery: A Prospective Randomized Control Study.

Introduction: Enhanced recovery after surgery (ERAS) is a multidisciplinary approach to optimize patient care. The goal of this approach is to reduce the body's reaction to surgical stress by optimizing the perioperative nutritional status, promoting analgesia without opioids, and early postoperative feeding. In pediatric patients, very limited literature is available for the application of ERAS protocol. This study was done to evaluate the application of ERAS protocol in pediatric hepatobiliary and pancreatic patients. Materials and Methods: This is a randomized prospective study conducted over a period of 2 years at a tertiary center in North India. A total of 40 hepatobiliary and pancreatic patients who were willing to participate in the study were included in the study. Patients were randomized by computer-generated method and data were collected regarding demography, clinical diagnosis, preoperative and postoperative workup, and peri-operative care including analgesia, pain scores, postoperative recovery, hospital stay, and complications. These patients were followed for 6 months postoperatively and the results were evaluated using SPSS software. Results: The study included 20 patients each in both the conventional and ERAS group with median ages of 11.5 years and 7.1 years, respectively. The data analysis showed that the ERAS group of patients had better outcomes in terms of hospital stay and drain removal time with significant statistical differences. Pain scores and complications are almost the same in both groups. Conclusion: Principles of ERAS can be safely applied in pediatric patients undergoing major surgery in the present era of emerging infections and also increasing patient burden without morbidity.