Context: Stage IV Wilms tumor is associated with poor prognosis, and recent changes in management have been suggested based on genetic markers and response to chemotherapy in this subgroup of patients. Objective: The objective was to evaluate the outcomes of children with Stage IV Wilms tumor who were managed with the AIIMS-WT-99 protocol. Materials and Methods: All the children with Stage IV Wilms tumor who were managed by us from October 2000 to December 2012 were included in the study. All the patients who had received primary treatment elsewhere were excluded from the study. All patients were managed as per the AIIMS-WT-99 protocol. After appropriate investigations, tumors that were deemed resectable underwent an upfront surgery. Unresectable and inoperable tumors received chemotherapy after cytological confirmation of the diagnosis. Chemotherapy was administered as per the NWTS-5 study. Pulmonary and flank radiotherapy was advised to all patients. Patients with poor response to chemotherapy or with recurrence were managed with an alternative chemotherapy regimen. The outcomes that were assessed the 4-year overall survival (OS) and the 4-year event-free survival (EFS). Statistical Analysis Used: Kaplan–Meier survival estimates. Results: During the study period, 219 patients with Wilms tumor were treated. Of these, 36 (16.4%) had Stage IV disease, and they formed the study group. The 4-year OS was 48% with a mean survival time of 59 months limited to 115 months (95% confidence interval: 41.3–75.9 months). The 4-year EFS was 42.4%. Patients with liver metastases had a poor outcome, whereas patients with good response to chemotherapy had a good outcome. Conclusion: Stage IV Wilms had a poor prognosis, and the survival rates in the index study are lower than those quoted in the literature. Although the exact reason for this poor result eludes us, these patients may benefit from the intensification of chemotherapy.

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Introduction: Giant occipital encephalocele (GOE) is a term used when the size of the OE is greater than or equal to the size of the head. It has been limited to case reports, with only sporadic exclusive series. This is a series of GOE managed at our center over time with emphasis on practical problems faced in management. Materials and Methods: This was a retrospective observational study. The patients were evaluated for the age of presentation, sex, and head size. Any associated neural tube defect was also looked for. Imaging was used for associated brain anomalies and to plan the surgical procedure. The requirement of ventriculoperitoneal (VP) shunt was also assessed. Results: During the study period of 7 years, 11 patients of GOE were admitted. Apart from one, all other patients were <1 year of age. Nine patients underwent surgical intervention, which included excision and repair of swelling with or without VP shunt placement. The content of the sac was only cerebrospinal fluid (CSF) in six patients and CSF and gliotic brain tissue in remaining patients. The attendants of two patients did not give consent for surgery and left against medical advice. Conclusion: GOE is an uncommon entity with limited information about management. Careful evaluation, proper imaging of patient, and care during intraoperative and postoperative periods with emphasis of factors determining the prognosis may provide satisfactory results.

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Published articles in scientific journals are a key method for knowledge-sharing. Researchers can face the pressures to publish and this can sometimes lead to a breach of ethical values, whether consciously or unconsciously. The prevention of such practices is achieved by the application of strict ethical guidelines applicable to experiments involving human subjects or biological tissues. Editors too are faced with ethical problems, including how best to handle peer-review bias, and find reviewers with experience, probity, and professionalism. This article emphasizes that authors and their sponsoring organizations need to be informed of the importance of upholding the guidelines in research and ethical rules when disclosing scientific work.

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Anorectal malformations (ARMs) have coexisting congenital anomalies. These can affect the overall prognosis. Anomalous craniofacial associations are less common. Recently, we managed two patients of ARM associated with unilateral microphthalmia, without any other major systemic anomalies. This was found to be a rare association on extensive literature search.

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Mediastinal pancreatic pseudocyst (MPP) is an infrequent complication of acute pancreatitis in children. A contrast-enhanced computed tomogram (CECT) of the chest and abdomen can aid in diagnosing pleural effusion and MPP. We describe a child with MPP in whom a transcutaneous computed tomogram-guided external drainage was curative. The case is being presented, and the relevant literature is highlighted in view of rarity of this entity.

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Newborn babies presenting with difficulties related to the aerodigestive tract (ADT) are often provisionally diagnosed and managed as having oesophageal atresia +/- tracheo-oesophageal fistula. Continuing difficulties with management and abnormal findings on investigations should lead to the consideration of other congenital anomalies of the ADT, including complete larnygo-tracheo-oesophageal cleft (LTOC). We present two patients who were eventually diagnosed with complete LTOC and care was withdrawn. We discuss the inherent difficulties in reaching this diagnosis and present an algorithm to help manage these rare and challenging situations.

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Arteriovenous malformations (AVMs) of the scrotum are very rare, with only 35 adult cases in the literature. An 8-year-old boy presented with an ulcerated bleeding AVM of the scrotum. The patient was resuscitated and managed conservatively initially. After the control of bleeding, oral propranolol was started. There was a decrease in the size of scrotal and penile swelling, healing of ulcer with total healing by 1 month, and no recurrence of bleeding episode. To the best of our knowledge, this was the first case of pediatric scrotal AVM treated by oral propranolol.

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Transverse testicular ectopia (TTE) is an uncommon anatomical abnormality where both the gonads migrate toward the same hemiscrotum. Embryologically, several theories regarding the origin of TTE have been suggested including adhesion and fusion of developing Wolffian canals, aberrant gubernaculum, testicular adhesions, defective formation of the internal inguinal ring, and traction on the testis by persistent Mullerian structures. To date, about 100 cases of TTE have been reported in the literature. Herein, we report five cases of TTE operated in the Department of Paediatric Surgery, Sardar Patel Medical College, Bikaner, over a period of 5 years. All cases were in the age group of 3 months to 4 years, out of which four were diagnosed preoperatively by clinical examination and ultrasonography (USG). Two of these four cases presented with inguinoscrotal swellings and contralateral undescended testes and other two presented with absence of testes in the scrotum. One case presented with a scrotal abscess which revealed both the testes in the abscess cavity. All five cases were operated upon with herniotomy and transseptal orchidopexy and discharged successfully. TTE, although rare, has a spectrum of presentations and should be kept in mind as a possibility in cases of inguinoscrotal disorders in young age group. A preoperative USG may be helpful in conformation of diagnosis.

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Renal cholesteatoma or keratinizing desquamative squamous metaplasia is infrequent in adults and rare in children. We report a case of renal cholesteatoma in a 4-year-old male child who was referred to us as a case of multiple renal calculi with hydronephrosis. We also discuss his management with a review of relevant literature.

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Aim: The aim of this study is to report the experience with minimally invasive surgery (MIS) in neonates with congenital malformations in a tertiary care pediatric hospital. Materials and Methods: Design: descriptive study. All neonates undergoing MIS from 2013 to 2018 were included in the study. Perinatal data, characteristics of surgery, type and duration of analgesia, postoperative mechanical ventilation duration, postoperative hospitalization, and postoperative morbidity and mortality surgery-related rates were recorded. Results: Seventy-one neonates were included. Gestational age and weight at surgery ranged from 24 to 41 weeks and from 1350 g to 4830 g, respectively. Procedures performed were esophageal atresia/tracheoesophageal fistula repair, congenital diaphragmatic hernia repair, diaphragmatic plication, fundoplication/gastrostomy, intestinal atresia repair, and pancreatectomy. The median follow-up period was 14 months. Five neonates (7%) were converted to open, for surgical difficulties. Nine (12.6%) neonates had intraoperative complications, with decreased oxygen saturation as the most common complication. The median duration of analgesia and postoperative mechanical ventilation was 3 days in most procedures. The morbidity and mortality rates were 36.6% and 2.8%, respectively. Conclusions: In this first experience with MIS in neonates, the duration of analgesia and hospitalization was shorter for some procedures. However, intraoperative and postoperative complications were still high, which was possibly attributed to the learning curve. Thus, it is expected that the frequency of the complications presented in this study will be reduced in future.

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Background: Children with biliary atresia (BA) have impaired metabolism of trace elements (TEs) (i.e., zinc, copper, selenium, and manganese), leading to an alteration in the serum levels. However, this alteration in serum level has any correlation with liver histopathological changes is not yet clear. Materials and Methods: This prospective study attempts to evaluate the preoperative serum levels of TE in comparison to controls and its correlation with liver histology in children with BA. Kasai portoenterostomy (KPE) and liver biopsy were performed in all cases. On liver histology, various parameters assessed and were graded according to predefined criteria. Serum levels of TE were determined again 12 weeks post-KPE and compared with the preoperative levels. Results: Mean(±standard deviation [SD]) preoperative serum Zn, Cu, Se, and Mn levels (in μg/dl) in BA patients were 41.6 ± 12.8, 130.6 ± 12.8, 50.0 ± 10.0, and 32.0 ± 20.0, respectively; in controls, these levels were 77.9 ± 13.7, 133.7 ± 13.7, 87.0 ± 13.0, and 8.0 ± 5.5, respectively. Mean postoperative levels in all patients were 68.5 ± 19.0, 91.7 ± 19.0, 79.0 ± 19.0, and 28.0 ± 12.0, respectively. Mean(±SD) postoperative serum Zn, Cu, Se, and Mn levels in BA patients with bile excretion were 73.8 ± 14.9, 83.6 ± 13.8, 85.0 ± 15.0, and 26.0 ± 10.0, respectively, whereas in those with no bile excretion, they were 40.6 ± 12.8, 134.0 ± 23.0, 49.0 ± 11.0, and 44.0 ± 16.0, respectively. In liver histology, specific parameters showed correlation with high Mn and low Zn levels. Conclusion: Serum TE levels are altered in children with BA and the establishment of successful biliary drainage may change the subsequent postoperative serum concentration. Serum Zn and Mn levels can signify specific histopathological liver changes and the extent of liver damage.

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Aims and Objectives: The aim of this study was to find out the level of satisfaction among couples receiving antenatal counseling provided jointly by fetal medicine specialists and pediatric surgeons. Materials and Methods: This was a questionnaire-based observational study. A total of 110 consecutive couples who were antenatally diagnosed with fetal structural anomaly and received counseling by fetal medicine specialist and pediatric surgeon together, were given a validated patient satisfaction questionnaire (PSQ-18) after delivery to assess their level of satisfaction regarding the antenatal care they received. Results: A total of 120 couple responded to the questionnaire, mean gestational age at delivery was 33.8 ± 7.14 weeks. In PSQ, 75.8% gave high scores for general satisfaction, maximum subjects provided high scores for interpersonal manner (IM) (77.5%) and communication (77.5%), and the least number gave high scores for time spent with the doctor (50.8%) and accessibility (42.5%). The technical quality (TQ) subscale was significantly high for the stillbirth/abortion group compared to live birth (P = 0.020). Significantly high scores for TQ (P = 0.037) and IM (P = 0.023) were obtained in the <20 weeks group. Conclusion: The joint counseling provided good interaction opportunity to the couple but still fell short of their expectations regarding time spent with the doctor and their accessibility.

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Objectives: The objective of the study was to review the imaging features of proven pediatric primary lung tumors, with a purpose of detecting key distinguishing features among the various entities. Materials and Methods: We retrospectively reviewed multidetector computed tomography (CT) images of 17 pediatric patients with primary lung tumors. For each examination, various CT image descriptors were used to characterize the pulmonary nodules/masses; including location, size, number, morphology, cavitation, calcification, intense enhancement, airway involvement, chest wall/pleural involvement, mediastinal/vascular involvement, and nodal enlargement. Results: The age of the patients ranged from 2 to 18 years (mean age of 9.5 years). Approximately 35.3% of tumors were benign and 64.7% were aggressive/malignant. Nine distinct histopathologic tumor entities were found. Common tumor types were recurrent respiratory papillomatosis (4) and inflammatory myofibroblastic tumor (4) with two endobronchial tumors including carcinoid and mucoepidermoid carcinomas. Besides invasion and nodal enlargement, large size and central location (P < 0.05) were predictors of aggressiveness/malignancy. Multiple lesions and cavitation (P < 0.05), on the other hand, were frequent in benign lesions. Conclusion: On imaging, location and morphological markers can allow diagnosis in majority of the tumors.

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Introduction: Critically ill surgical neonates are physiologically challenged and delicately poised on ventilator and inotropic support systems. They experience significant stress in the event of surgery. Shifting them poise further addition to this stress. We here share our experience of operating such surgical neonates for certain conditions in the neonatal intensive care unit (NICU). Methods: We retrospectively analyzed the data of operated patients in the NICU. We collected the demographic data, diagnosis, and preoperative stability of the patient, ventilator and inotropic requirements, need for extra anesthetic drugs, procedures performed, complications, and outcome. Operations were performed at bedside in the NICU in critically ill, unstable neonates who needed emergency surgery, neonates of very low birth weight (<1000 g), and neonates on special equipment such as high-frequency ventilators. We excluded minor routine procedures such as drain placement, central line placement, ventricular taps, incision and drainage, and intercostal drainage procedures. Results: We performed seven surgical procedures in the NICU. These included bowel resections and stoma creation, fistula ligation, lung biopsies, and ventricular reservoir placement. Gestational age ranged between 24 and 34 weeks (mean, 28 weeks). Birth weights ranged between 800 and 2500 g (mean, 1357 g). Age at surgery was between 2 and 18 days (mean, 10.2 days). All our patients were on inotropic support and were intubated and mechanically ventilated. Conclusion: Doing surgery for critically ill neonates in the NICU definitely has a place. It was the need of the hour based on the condition of the neonates; however, we feel that neonatal surgery in the NICU should be the norm as it can improve survival. Surgery in the NICU can give a fighting chance to these patients; however, operation theaters in the NICU would be an ideal setting.

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