Catheter-associated Bloodstream Infection in Children with Tunneled Central Venous Catheters: A Single-center Experience.

Background: Tunnelled catheters improve venous access in haematological diseases and malignancies, but are associated with complications. We prospectively analysed the episodes of CABSI and its associated risk factors. Aims: To study the incidence and risk factors for CABSI in children with tunnelled central venous catheters (both Hickmans and Chemoports). Materials and Methods: This is a prospective observational study done at our institute. Children under the age of 18 who underwent insertion of a Hickman or Chemoport from March 2018 to Dec 2022 were enrolled. Episodes of CABSI were noted and its risk factors were analysed. Results: In total, 258 catheters were inserted in 250 children. Age ranged from 1 month to 18 years (median 67 months) with 60% being boys. A total of 152 Hickmans, 106 chemoports were inserted. Indications for insertions were for requirement of BMT and chemotherapy in majority of cases. CABSI were seen in 28.6% of catheters. Younger children (< 4years), Neutropenia (counts < 1000) and use of TPN were significantly associated with CABSI(p value<0.05). Infection was more with externalized catheters (Hickman) than implantable ports (Chemoports) but was not statistical significant(p value>0.05). Almost 30% of catheters with CABSI required removal because of florid sepsis. Others could be salvaged with aggressive antibiotics. Conclusions: CABSI is a serious complications related to tunnelled catheter. Smaller children (<4 years), neutropenia and usage of TPN is a risk factor for development of CABSI. It can be treated with appropriate antibiotics and required removal in almost a third of all the cases.

Transplanting Livers in Young Children - Looking Back at 100 Cases.

Introduction: Despite advances in medical therapy, liver replacement continues to be the only definitive mode of therapy for children with end-stage liver disease (ESLD). However, its acceptance in India has been discouraging more due to financial and logistic reasons than the availability of expertise. This report outlines our journey and highlights issues pertinent to circumstances in an emerging economy like India. Aim: The aim is to review a single center's 100 case experience with liver transplantation (LT). Materials and Methods: A prospective analysis of all children who underwent LT since 2005 at our institute was done. The data were collated from a maintained structured database. Results: Hundred children underwent LT. Sixty-four were boys. Age ranged from 5 to 144 months, with a median of 17 months. The mean weight of the cohort was 7.5 kg (ranging from 3.7 to 31.5 kg), with 60% of our children weighing between 5 and 10 kg. Biliary atresia is the most common indication (57%); others include metabolic disorders, progressive familial intrahepatic cholestasis, and hepatoblastoma. Two patients were for acute liver failure. Ninety-one children underwent live donor LT (mothers being the majority of the donors). None of the donors had any major postoperative complications. Major intraoperative complications include sepsis (39%), vascular complications (17%), biliary leak (11%), and intestinal complications (11%). Early postoperative deaths occurred in 18% of patients with sepsis being most common cause. The mean follow-up was 8.5 years and the overall survival is 70%. The mean survival is time (months) = 176.1. All surviving patients were followed up and had achieved good catch up growth by 3rd and 5th years posttransplant. The long-term sequalae include recurrent intercurrent infections, graft rejection (9%), posttransplant lymphoproliferative disease (5%), and portal vein stenosis (5%). Conclusion: Our experience demonstrates the feasibility of LT in children with ESLD in India. With longer-living grafts, patients often struggle with other issues such as compliance with follow-up, financial issues, recurrent infections, and neurological problems. Close monitoring with regular follow-up of patients helps in early recognition and treatment of late-onset complications, thus helping the overall long-term outcomes.

Redo Surgeries in Anorectal Malformations: A Single-center Experience.

Aims and Objectives: Complications following posterior sagittal anorectoplasty (PSARP) for ARM are well known. In this article, we present our experience of managing five patients who required major redo surgeries for complications resulting from previous attempts to correct ARM. Materials and Methods: We reviewed all patients who underwent major redo surgeries in our hospital for complications from previous repairs for ARM, from June 2013 to June 2019. Data was obtained from hospital records and analysed. Results: Five patients whose ages ranged from 5 months to 14 years were included in the study. Four were boys and 1 was a girl. All patients had undergone PSARP in other hospitals. The presentations were retained distal bowel causing urinary retention and constipation (n=1), pulled through proximal urethra and bladder neck presenting as passage of urine from neo-anus (n=1), retained common channel (of cloaca) causing a 'H' type configuration (n=1), mispositioned neo-anus (n=1) following a primary PSARP and lastly undivided recto-urethral fistula causing fecaluria (n=1). All of them underwent redo repairs by posterior sagittal approach with documented improvement in their symptoms. Two of them required total bowel management to remain clean. Conclusion: All the complications reported here have been described in literature nevertheless, this report will add to the body of experience. Posterior sagittal approach (PSA) has proved to be very successful technique in correcting these complications.

Sestamibi-guided thoracoscopic excision of functional mediastinal parathyroid adenoma in a child: A case report and review of literature.

Primary hyperparathyroidism secondary to ectopic parathyroid (in anterior mediastinum) is rare in children. We report the case of a 12-year-old girl with a history of multiple fractures, renal calculi and limb deformities. She was diagnosed with hyperparathyroidism secondary to an intrathymic parathyroid adenoma. Sestamibi scan showed a lesion in the anterior mediastinum. A biochemical evaluation revealed hypercalcaemia, elevated alkaline phosphatase and parathormone levels. The lesion was marked with radioisotope and confirmed intraoperatively using a gamma camera. The child underwent thoracoscopic left thymectomy with the adenoma. Immediate decrease in calcium and parathyroid hormone values were noted intraoperatively and serial monitoring showed a downward trend. On follow-up, the child is doing well. Ectopic parathyroid adenoma is very rare. CT with radioisotope scans is helpful in diagnosis. Thoracoscopic excision of ectopic adenoma is safe in children.

Laryngeal Cyst in Children : A Retrospective Analysis.

Aim: To study the presentation, management and outcome of laryngeal cysts in children. Materials and methods: This is a retrospective observational study of children with Laryngeal cysts who were managed in the Department of Paediatric Surgery between April 2015 to Jan 2022. Observations and Results: Eight children with laryngeal cysts were included, 5 were girls. Age ranged from 3 days to 10 years (median 5 months). Weight ranged from 3kg to 40kg (median 6.3 kg). All presented with stridor. Three children had co-morbid conditions, one each with Leucocyte adhesion deficiency type -1, atrial septal defect & PHACES syndrome. Two children had severe respiratory distress and required tracheostomy at presentation. All children underwent MLB. The cysts were 2 subglottic cysts (one inflammatory cyst) and 6 vallecular cyst. Five underwent endoscopic excision of cysts, 1 underwent excision with lateral cervical approach, 1 underwent endoscopic de-roofing of cyst and airway reconstruction with anterior costal cartilage graft (associated Sub glottis stenosis). Conclusion: Laryngeal cysts are a rare cause of stridor in children. MLB is the best diagnostic tool. Treatment with excision / deroofing of the cyst endoscopically, is safe and effective in paediatric age group. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03495-w.

Kasai Portoenterostomy at a Slightly Delayed Age and Native Liver Survival in Children With Biliary Atresia: Single Center Experience.

OBJECTIVES: We studied the modifiable prognostic factors that extend native liver survival at 2 years after Kasai portoenterostomy (KPE). METHODS: We reviewed hospital records of patients with neonatal cholestasis, with focus on infants diagnosed with biliary atresia in a tertiary care hospital between January, 2014 and May, 2021. We determined the association of outcome with clinical and laboratory variables. RESULTS: Infants who underwent KPE at a median (IQR) age of 76 (72-79) days had best outcomes, with minimal severe post-KPE complications and 2-year survival rate of 84.6%, compared to other infants (younger and older days at KPE). The median (IQR) weight at KPE in this group was 4.66 (4.2, 5.0) kg. CONCLUSION: In contrast to traditional recommendations, babies with median age at KPE of 76 days had superior native liver survival (84.6%) and reduced post-KPE complications, as compared to earlier KPE age. Nutritional status and weight of infant at KPE could be associated with this survival difference. This observation needs confirmation through multicentric prospective study.

Concealed Penile Duplication - Presenting Late.

We report a case of concealed penile duplication without many symptoms and normally looking external genitalia in a 10-year-old boy. He was evaluated with appropriate imaging and had successful surgical reconstruction of his genitalia with good cosmesis and functional outcome.

Adrenocorticotropic Hormone-Independent Hypercortisolism Managed by Bilateral Retroperitoneoscopic Adrenalectomy: Report of a Case and Review of Literature.

Bilateral adrenalectomy is the reference treatment for Cushing's syndrome related to adrenocorticotropic hormone-independent macronodular adrenal hyperplasia (AIMAH). We report a case of AIMAH managed by sequential retroperitoneoscopic adrenalectomy and review the literature of the same.

Melanotic Neuroectodermal Tumor of Infancy: Does Enucleation Alone Suffice?

Melanotic neuroectodermal tumor of infancy (MNTI) is a rare melanin-containing mesenchymal tumor of neural crest origin. We present a case of MNTI in a 1-year-old girl. It was managed successfully with conservative excision (enucleation).

Endoscopic Appendectomy for Inverted Appendix: A Case Report and Review of Literature.

Inverted appendix is a very rare anomaly and presents a diagnostic challenge. We report a case of inverted appendix in a 10-year-old boy and its management.

Cardiac Perivascular Epithelioid Cell Tumor: Report of a Child and Review of the Literature.

Cardiac tumors presenting as mediastinal masses in childhood are rare. This report describes the case of a 6-year-old girl who presented to us after a failed attempt at resection of a mass arising from the left atrial appendage. Computed tomography-guided biopsy suggested a perivascular epithelioid cell tumor (PEComa). Sirolimus was started in order to reduce the mass size. She subsequently underwent resection of the mass by midline sternotomy approach and cardiopulmonary bypass. Recovery was uneventful and the child is doing well at two-year follow-up. Contemporary literature is reviewed. Only seven cases, including two children, with mediastinal PEComa have been reported. Pathology and treatment options are discussed.

Respiratory Morbidity Following Pediatric Orthotopic Liver Transplantation.

We evaluated the pulmonary complications following orthotopic liver transplantation in 45 children (age <18 y). 22 patients (49%) developed respiratory complications. Pediatric end-stage liver disease (PELD) score >25 and positive fluid balance were independent risk factors. Patients with respiratory complication had significantly higher mortality and intensive care unit stay.

Quality of Life of Indian Pediatric Surgeons: Results of a Survey (of Indian Association of Pediatric Surgeons Members).

INTRODUCTION: Much is debated on the quality of life of pediatric surgeons practicing in India, all based on anecdotal and personal experiences. There is no systematic study on this. This study addresses this and attempts to glean a clearer picture of the life as a pediatric surgeon in India. METHODOLOGY: This questionnaire-based study was administered via an online survey to all Indian Association of Pediatric Surgeons members. The responses were anonymous and investigators blinded. Data were collated and analyzed using STAT11.1. RESULTS: A total of 173 pediatric surgeons responded. Eighty-six percent were men. About 73.7% of the surgeons were between 31 and 50 years of age. Almost 63.4% practiced in urban areas, whereas 36% in other smaller towns. About 0.6% reported that their practice was rural. Almost 26.4% were in private/solo practices, whereas 53.4% were in institution-based practice. Almost 80% felt that they were adequately trained while starting their practice. About 78% are professionally satisfied with their work. Only 44.5% of surgeons felt that they were compensated adequately financially. Reading was the favorite pass time. Almost 40% of the surgeons felt that they were either overweight or obese. About 41% of the surgeons exercise more than 3 times a week. Only 11.4% smoke, whereas 36% drink. Fifty-three percent of surgeons felt that their personal savings were adequate. Seventy-six percent use Facebook. Sixty-eight percent were satisfied with their quality of life. Age was significantly associated with professional satisfaction, financial satisfaction, and quality of life and all improve as one's age progresses. None were affected with one's gender, type of practice, and the place of practice. Age, weight, exercise, and one's savings significantly affected ones quality of life. CONCLUSION: This is the first study which objectively highlights that most surgeons are happy professionally and financially in due course of time and demolishes the common belief that pediatric surgeons are unsatisfied. It also acts as a point of reference and encouragement to newer aspirants in pediatric surgery.

Pediatric liver transplantation: A report from a pediatric surgical unit.

BACKGROUND: Liver transplantation is well established worldwide as an effective treatment for end-stage liver disease in children. Acceptance in India has been slow because of considerations of cost, infections, inability to support long-term care, and non-availability of expertise. AIM: This study was designed to report our experience with pediatric liver transplantation. MATERIALS AND METHODS: Twenty-eight children underwent liver transplantation. RESULTS: Biliary atresia was the commonest indication (n = 15) followed by metabolic liver disease. Twenty-six children had living donor transplants, mothers being the donors in a majority of these. Common surgical complications included bile leaks (n = 3) and vascular problems (n = 6). Common medical complications included infections, acute rejection, and renal failure. Overall, patient survival was 71%, while that for the last 14 cases was 92%. All survivors are doing well, have caught up with physical and developmental milestones and are engaged in age appropriate activities. CONCLUSIONS: The study demonstrates the feasibility of a successful pediatric liver transplant program in our country.