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.

Laparoscopic Transperitoneal Adrenalectomy for Adrenal Tumors in Children: Technical Considerations and Surgical Experience.

Aims: This study aimed to review our surgical experience of laparoscopic adrenalectomy (LA) for adrenal masses in the pediatric age group. Materials and Methods: The electronic medical records of all patients younger than 18 years of age who underwent LA between 2016 and 2023 were retrospectively reviewed. Children with adrenal tumors localized to the site of origin without evidence of encasement of major vasculature or involvement of adjacent organs were considered for LA. Patients with diagnosis of malignant adrenal tumor on preoperative work-up underwent open adrenalectomy. Data were collected regarding demography, clinical presentation, hormonal workup, imaging, duration of surgery, transfusion requirement, conversion rate, postoperative recovery, duration of intravenous (i.v.) analgesia and hospital stay, pathology, complications, and status at follow-up. Results: Between 2016 and 2023, LA adrenalectomy was performed in 11 patients (6 boys and 5 girls) with a mean age of 46.3 (8-120) months. A functional tumor was detected in 6 (54.5%) children, manifesting clinically with Cushing's syndrome (3), virilization (1), feminization (1), or Conn's syndrome (1). Seven (63.6%) tumors originated from the right adrenal, and 4 (36.4%) from the left adrenal gland. The mean tumor weight and size was 49.1 (10-80) g and 5.6 (3-8) cm, respectively. Histopathology included adrenal adenoma (5), ganglioneuroma (3), ganglioneuroblastoma (1), myelolipoma (1), and intermediate adrenocortical tumor (1). The mean surgery duration was 186.6 (120-265) minutes. Intraoperative blood transfusion was required in 2 (18.2%) patients and none required conversion. Full feeds were resumed at 42.7 (24-60) hours, i.v. analgesia requirement was for 54.5 (36-72) hours and mean hospital stay was 5.1 (3-8) days. All patients were symptom-free with no recurrence at mean follow-up period of 50.6 (3-83) months. Conclusions: In children with well-circumscribed, localized, and noninvasive adrenal tumors, LA is feasible, effective, and safe with all advantages of minimally invasive surgery.

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.

Comparison of diagnostic accuracy of models combining the renal biomarkers in predicting renal scarring in pediatric population with vesicoureteral reflux (VUR).

INTRODUCTION: Renal scarring is prominently observed in children with vesicoureteral reflux (VUR) and can lead to complicated renal outcomes. Although biopsy is the gold standard to detect renal scarring, it is an invasive procedure. There are established renal biomarkers which can help detect renal scarring. Individual biomarkers have not shown to have extensively good discriminatory ability for this. AIM: This paper aims at combining the values of multiple biomarkers in models to detect renal scarring. METHODOLOGY: Secondary data with the values of renal biomarkers like kidney injury molecule-1, neutrophil gelatinase-associated lipocalin (NGAL), and urinary creatinine along with the renal scarring status was considered. Logistic regression, discriminant analysis, Bayesian logistic regression, Naïve Bayes, and decision tree models were developed with these markers. The discriminatory ability of individual biomarkers along with the models was assessed using the area under the curve from ROC curve. Sensitivity, specificity, and misclassification rates were estimated and compared. RESULTS: NGAL was the most predominant renal biomarker in classifying the patients with renal scarring (AUC: 0.77 (0.67, 0.87); p value < 0.001). Each of the model performed better than individual biomarkers. Decision tree (AUC: 0.83 (0.74, 0.91); p value < 0.001) and Naïve Bayes model (misclassification rate = 20.2%) performed the best amongst the models. CONCLUSION: Combining the values of renal biomarkers through a statistical or machine learning model to detect renal scarring is a better approach as compared to considering individual renal biomarkers.

Utility of Urinary Biomarkers Neutrophil Gelatinase-Associated Lipocalin and Kidney Injury Molecule-1 as a Marker for Diagnosing the Presence of Renal Scar in Children with Vesicoureteral Reflux (VUR): A Cross-Sectional Study.

Aim: To explore the possibility of using urinary biomarkers neutrophil gelatinase-associated lipocalin (NGAL) and kidney injury molecule-1 (KIM-1) to assess the presence of renal scars in children with Vesicoureteric Reflux (VUR). Materials and Methods: This cross-sectional study was conducted in 94 children aged 0-16 years diagnosed with VUR in the Department of Pediatric Surgery, JIPMER. Urinary biomarkers were measured using the enzyme-linked immunosorbent assay kits, normalized with urinary creatinine (Cr) and compared with severity of VUR (low grade [I and II] and high grade [III, IV, and V]), presence or absence of renal scar in VUR patients and severity of renal scar. Independent Student's t-test, Mann-Whitney U-test, and analysis of variance Kruskal-Wallis test were used for comparison, and receiver operating characteristic (ROC) curve analysis for predicting the accuracy of biomarkers in detecting the presence of renal scars. Results: The median urinary NGAL (uNGAL) value was higher in children with renal scar (1.49 ng/mL) than those without renal scar (0.58 ng/mL) and was statistically significant (<0.001). Whereas median uNGAL/Cr was higher in children with renal scar (0.07) than those without renal scar (0.03) but was not statistically significant (P = 0.06). Urinary KIM-1 and urinary KIM-1/urinary Cr (uKIM-1/Cr) was not found to be a significant predictor of renal scar. The difference of uNGAL/Cr was comparable between the grades of renal scar but was not statistically significant. On ROC curve analysis, uNGAL had area under the ROC curve (AUC) of 0.769 with 71% of both specificity and sensitivity, whereas uNGAL/Cr was found to be a poor predictor of renal scar with AUC of 0.611, 60% sensitivity, and 61.2% specificity. Conclusion: uNGAL can serve as a noninvasive marker for diagnosing the presence of renal scar in children with VUR and a multicentric more extensive cohort study may be needed to strengthen or negate its role.