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.

Appendectomy in Management of Malrotation of Gut is it Necessary?

Introduction: Malrotation is a congenital anatomical anomaly that affects the normal positioning of the intestines. Traditional management of malrotation, as described by Ladd, consists of detorsion of the volvulus if present, division of Ladd's bands, widening of the mesenteric root, proper positioning of the small and large bowels, and a prophylactic appendectomy. This study was done to determine whether appendectomy should be an integral part of the Ladds procedure or if it can be avoided. Materials and Methods: This retrospective observational study was conducted in one pediatric surgical unit in the tertiary care center of North India. All the cases of malrotation of the gut managed from January 2008 to December 2018 were reviewed. The details of the patients were recovered from the electronic data recording system of the hospital and manual operation theater records. The cases that have a follow-up of <5 years were not included in the study. The details were charted in an Excel Sheet for the analysis. No statistical test was performed because there was no event in patients in whom prophylactic appendectomy was not done. Results: The data analysis revealed that a total of 66 malrotation patients were managed during this period, those who fulfilled the inclusion criteria. Among 66 cases included in the study, in 41 cases, prophylactic appendectomy was done, whereas in the rest 25, prophylactic appendectomy was not done. During the follow-up, none of the patients in whom the appendix was preserved presented with signs or symptoms related to appendicitis or any other complications. Conclusion: Ladds procedure without appendectomy can be performed in view of the potential use of the appendix in the future, and with the fact that in the current era of advanced medicine, appendicitis can be diagnosed early due to the advancement of imaging and better record keeping.

Congenital colonic stenosis: an unusual cause of colonic obstruction masquerading as Hirschsprung's disease in infancy.

Congenital colonic stenosis (CCS) is an extremely rare cause of large bowel obstruction in early infancy. Only 35 cases of CCS have been reported in literature to date. CCS often causes a diagnostic quandary as it is difficult to distinguish it clinically from Hirschsprung's disease. We report a case of an infant with CCS who was managed with resection of the diseased colonic segment with critical stenosis at two sites and colo-colonic anastomosis. In our report, we discuss the challenges faced in the diagnosis and surgical management of this unusual case.

Comparative Evaluation of Altered Taste Perception among Oral Submucous Fibrosis Patients.

BACKGROUND: Taste perception is an important factor in sustaining human life. Impairment of taste is one of the important features of oral submucous fibrosis (OSMF), and it has not received much attention, owing to limited research work in the field. Therefore, the present study was conducted to determine taste alteration in OSMF patients. MATERIALS AND METHODS: A total of 200 participants, both males and females with the age range of 20 years to 55 years, were included in the study. Four basic tastants (i.e., sweet, salt, sour, and bitter) were prepared as follows: sucrose for sweet (0.1-1.0 mol/l), sodium chloride for salty (0.01-1.0 mol/l), citric acid for sour (0.320-0.032 mol/l), and quinine sulfate for bitter (0.01-1.0 mol/l) and full mouth rinse test was performed for a complete taste response examination, after which punch biopsy was taken from buccal mucosa to determine histopathological staging. The data obtained were tabulated and analyzed by the Pearson Chi-square test; P < 0.05 was considered statistically significant. RESULTS: The overall results suggested that there was a significant alteration of taste. The sweet taste was altered followed by salty and bitter was least affected. CONCLUSION: The study points out at the significance of alteration in taste perception is OSMF patients related to sweet, salt, sour, and bitter taste by using physiological stimuli tastants.