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.

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.

Retroperitoneal yolk sac tumour encroaching the liver and adrenal gland with tumour thrombus in cavo-atrial region and hepatic veins.

Paediatric germ cell tumours (GCT) are rare tumours and are unique because of varied clinical presentation and locations. Yolk sac tumour is the predominant malignant histology and a serum marker; alpha fetoprotein is used to see treatment response and recurrent disease. It is extremely rare to find a retroperitoneal GCT with tumour thrombus extending up to the cavo-atrial region with involvement of the hepatic veins. We report a case of retroperitoneal yolk sac tumour (RPYST) with extension to the liver and right adrenal gland along with tumour thrombus in the inferior vena cava and in the right and middle hepatic veins. The child was operated after satisfactory response to chemotherapy. Excision of the tumour along with the right adrenal gland and around 5 cm of retro-hepatic caval resection was done. Inferior vena cava resection was tolerated without reconstruction. Currently child is disease-free and symptom-free at 22 months of follow-up with normal serum marker.

Bronchial Carcinoid Tumor in an Adolescent Female: Diagnosis and Management by a Multi-Disciplinary Team.

Bronchial carcinoid is the most common primary malignant lung tumor in children; however, it remains a very rare diagnosis due to the overall low incidence of childhood lung malignancies. We report a case of a 17-year-old girl with respiratory symptoms who was initially misdiagnosed as a case of COVID pneumonia. She was later detected to have a right mainstem bronchial carcinoid which was managed successfully by a multi-disciplinary team.

Giant renal parapelvic cyst with pelvi-ureteric junction obstruction in an infant: challenges in diagnosis and laparoscopic management.

Renal parapelvic cysts (RPC) have an incidence of approximately 1%-3% in the general population. However, they rarely present in children with only two cases reported in literature. RPC are often misdiagnosed as it is difficult to distinguish them from hydronephrosis on preoperative imaging. We report a case of an infant with a giant RPC with associated pelvi-ureteric junction obstruction, who was managed successfully with laparoscopic complete excision of RPC and dismembered pyeloplasty. In our report, we discuss the challenges faced in the diagnosis and surgical management of this unusual case.