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Background: Unilateral Wilms' tumor (uWT) is the most common renal malignancy in the pediatric population. Although the onset of surgical intervention like radical nephrectomy (RN) has substantially reduced the mortality rate, recent evidence has raised concerns regarding several postoperative complications associated with this procedure. Nephron-sparing surgery (NSS) has been reported to avoid such postoperative complications and have high technical success rate. However, not much literature is available comparing the efficacy of RN and NSS for managing nonsyndromic uWT, which makes it less acceptable globally. Materials and Methods and Results: A systematic identification of the literature was performed according to the Preferred Reporting Items for Systematic Reviews and Meta-analyses guidelines on four academic databases: MEDLINE, Scopus, EMBASE, and CENTRAL. Out of 1017 records, 20 manuscripts including 312 patients were included in the review; 4 patients treated by the author (unpublished data) have also been included. The studies included 7 retrospective case series with no comparison group, 4 retrospective case series with comparison group, 4 retrospective series with a cursory mention of NSS in uWT, 2 case reports, 1 protocolized prospective trial with comparison group, 1 administrative database analysis with comparison group, and 1 randomized controlled study. In all, 316 patients with nonsyndromic uWT were studied. The pretherapy volumes of the WT ranged from 2.4 to 640 ml. Majority of them were administered preoperative chemotherapy. About half of the patients (n = 159) (50.3%) were Stage I tumors. Stage II, III, and IV tumors were seen in 23 (7.3%), 20 (6.3%), and 3 (1%) patients. Stage was not specified in 111 (35.1%) patients. Majority of them were “intermediate risk” WT. All these tumors were nonanaplastic. Surgical margins were reported positive in 13 (4%) patients. Overall and event-free survivals were 100% in most of the studies; only handful of relapses were noted in this systematic review. Conclusions: This qualitative systematic review recommends the use of NSS for nonsyndromic uWT. The procedure accounts for higher postoperative renal function and lesser incidence of hypertension as compared to the radical nephrectomy. Overall and event-free survivals are comparable or even better with NSS.

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Aim: The aim of the study was o define the preoperative diagnostic clinical and radiological features in girls with congenital pouch colon (CPC). Materials and Methods: Records of 47 girls with CPC, managed from 1996 to 2018, were reviewed. There were two age groups: Group A (newborn to 12 months; n = 26) and Group B (>12 months to 20 years; n = 21). The important clinical and radiologic features to help in a preoperative diagnosis were noted. Results: The most common subtype was Type II (57.4%), followed by Type I (23.4%) and Type III (12.8%). The features common to both the groups were abdominal distension (A = 53.8%; B = 9.52%), severe perineal excoriation (A = 19.2%; B = 23/8%), and urinary incontinence (A = 30.7%; B = 85.7%). In addition, in Group B, fecaloma on abdominal palpation was noted in 28.6% of patients. The characteristic appearance of the perineum including external genitalia and findings on plain abdominal X-ray (AXR) were 100% accurate and hence diagnostic. These unique features helped us formulate an algorithm for preoperative diagnosis of this uncommon form of anorectal malformation in girls seen in North India. Conclusions: The characteristic features on clinical examination should alert one to the presence of CPC in the outpatient clinic. The AXR was diagnostic in 100% of cases and is mandatory. If any doubt persists, examination of the genitalia under anesthesia with more retraction of the labial folds and endoscopy can be performed for confirmation. These measures should enable a clinician to make an accurate preoperative diagnosis in every girl with CPC.

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Introduction: Posterior urethral valve (PUV) is life threatening congenital anomaly of urinary tract. Aim of the study was to correlate urethral ratio (UR) and bladder wall thickness (BWT) with cystoscopic findings in PUV patients to assess residual valves and to validate UR as a diagnostic tool for residual valves. It also aimed to assess the utility of bladder wall thickness in diagnosis of residual valves. Materials and Methods: A total of 31 patients were included in the prospective study done from 2017 to 2019. Calculation of UR was done in oblique VCUG films by dividing maximum posterior and anterior urethral diameter without the catheter insitu. Measurement of BWT was done at dome and bladder neck at full distension with feeding tube insitu and was done at same volume in follow up. The procedure was repeated at 3 months follow up. The findings were compared with cystoscopic findings for the status of residual valves as gold standard. Each patient served as control for self in the study. Results: Median age of presentation was 1 years with range of 1day to 10 years. The most common complains at the time of presentation in our study was straining (35.48%) followed by antenatally diagnosed patients (25.81%) and recurrent UTI (19.36%). Pre fulguration median UR was 2.45. Post Fulguration median UR was 1.20. It showed a statistically significant reduction (p < 0.001) after fulguration. Pre fulguration median BWT was 4 mm. Post fulguration median BWT was 2.5 mm. BWT showed a statistically significant reduction (p < 0.001) after fulguration as well. ROC curve was plotted for UR and BWT. BWT more than 1.95 mm (sensitivity-80%) and UR more than 1.2 (sensitivity-70%) indicates residual valves. Conclusion: A step ladder approach including BWT, UR and check cystoscopy can serve as a new diagnostic algorithm for the assessment of residual valves thereby avoiding extra radiation and general anesthesia exposure.

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Introduction: The extraction of smooth spherical objects is challenging as they are difficult to grasp within the jaws of the forceps and tend to slip distally. Objective: The authors herein have shared their experience with the use of a Fogarty catheter (FC) for safe extraction of smooth and spherical “foreign body (FB) with a hole.” Materials and Methods: Report on pediatric cases (n = 4) of airway “FB with a hole” wherein the FC was used for their extraction. Mean age was 27.5 months (range: 17 months–39 months). The male: female ratio was 3:1. The technique of FB extraction with a FC has been described, including the principle of the technique, indications, and contra-indications, technical problems and troubleshooting. Results: The FB spectrum included a necklace bead (n = 2), nonnecklace bead (n = 1) and a fragmented end-piece of the housing of a ball-pen (n = 1). The locations of the FBs were right main bronchus (n = 1), secondary bronchus on the right (n = 1), and in the left main bronchus (n = 2). Successful removal of FB with use of FC during rigid broncoscopy was possible. The bead had to be rotated in n = 2 patients to align the hole with the FC. Problems associated with threading the hole and disimpaction of the FB have been highlighted. No complications were observed. The advantages and limitation of the technique have been discussed. Conclusions: The use of FC with the described technique offers a safe, effective and reproducible method for removal of airway “FB with a hole” in a controlled environment while minimizing the possibility of iatrogenic injury to the wall of the surrounding airways.

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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.

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Aims: This study aimed to evaluate the outcome of perineal trauma in children and to a define protocol for their management. Methods: It is a retrospective study of children who presented with perineal injury between August 2012 and December 2020. The patients were classified into three groups: Group-1 included children with perineal and genitourinary injuries; Group-2 included patients with perineal and anorectal injuries; and Group-3 included patients with perineal, genitourinary, and anorectal injuries. All patients underwent primary repair. Those with full-thickness anorectal injury underwent an additional covering colostomy, while urethral disruption was initially managed by a diverting suprapubic cystostomy (SPC). Results: A total of 41 patients were studied. Impalement injury (n = 11; 27%) and sexual abuse (n = 11; 27%) were the most common mechanisms of injury. Twenty (49%) patients had anorectal injuries with 10 (24%) each of partial-thickness and full-thickness injury. There were 24 (59%) genital injuries and five (12%) urethral injuries. One patient each developed anal and vaginal stenosis, both were managed with dilatation. One patient developed a rectovaginal fistula repaired surgically at a later date. Conclusion: Perineal injuries with resultant anorectal or genital damage require a careful primary survey. Following stabilization, an examination under anesthesia as a set protocol will help determine the treatment strategy. A colostomy is essential in the acute management of severe anorectal injuries to reduce local complications and preserve continence. Urethral injuries may warrant an initial diverting SPC in selected cases.

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Objectives: The objective of this study is to compare the changes in renal function and drainage following open dismembered pyeloplasty with and without renal pelvis reduction. Materials and Methods: Randomized prospective study of children with ureteropelvic junction obstruction undergoing pyeloplasty with (Group 1) and without (Group 2) pelvis reduction over an 18-month period. Postoperative function and drainage were assessed by ethylene dicysteine (EC) scan and intravenous urography (IVU) and renal pelvis size by ultrasonography. Results: Forty-two patients (2 months-11 years) participated. The mean preoperative EC scan function was Group 1: 45.88% ± 14.42% (5%–80%) and Group 2: 39.22% ± 9.75% (21%–53%). (P = 0.117). The mean postoperative EC scan function of Group 1 was 42.64% ± 9.62% (17%–54%) and 43.75% ± 9.88% (17%–58%) and of Group 2 was 44.77% ± 12.82% (20%–68%) and 42.25% ± 8.56% (23%–58%) at 3 months (P = 0.584) and ≥ 1year (P = 0.385), respectively, with no significant difference. None required re-do pyeloplasty. The number of patients with slow drainage, especially at 3 months and also at ≥1-year postoperative period on EC scan was slightly higher in Group 2 compared to Group 1 but did not attain statistical significance. There was postoperative improvement in function and drainage on IVU with no significant difference between the two groups, (P = 0.214; P = 0.99, respectively). At a mean follow-up of 45.5 months, Group 2 also showed significant reduction in pelvis size on ultrasound (P = 0.011). Conclusion: Postoperative function remained stable in both groups. More number of patients achieved unobstructed drainage by 3 mo postoperative after reduction pyeloplasty but drainage patterns were mostly similar between reduction and nonreduction of pelvis group in late follow-up.

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Background: Gastric outlet obstruction (GOO) is a very rare condition in children, when infantile hypertrophic pyloric stenosis is excluded as a cause. Five cases of pediatric GOO were successfully managed at our institute. Materials and Methods: We retrospectively evaluated children with idiopathic GOO from 2009 to 2016. Medical records were reviewed for demographic data including age, sex, presenting symptoms, diagnostic investigations, treatment methods, complications, and long-term follow-up. Results: During 7 year period (2009–2016), 5 cases of GOO admitted to our hospital with a history of persistent vomiting. The vomiting used to occur 12–18 h after meal and vomitus contained foul-smelling undigested meal. There was no history of any caustic ingestion. Their age ranged from 2 to 10 years, with a median age of 6 years. Out of 5 cases, 2 were females and the rest were males. The diagnosis of idiopathic GOO was confirmed by history, clinical examination, contrast study, endoscopy, and endoscopic mucosal biopsy. The remarkable finding was the cicatrization causing stricture of the pyloric region in all cases. Out of 5 cases; we have done Heineke-Mikulicz pyloroplasty in 1 case, V-Y advancement antropyloroplasty in 2 cases and Kimura's Diamond-shaped Gastroduodenostomy in 2 cases. The length of the strictured segment determined the type of surgical procedure. All cases did well postoperatively with no mortality. Conclusions: Length of the stricture determines the procedure to be done. In long and narrow stricture V-Y advancement antropyloroplasty and in small stricture Kimura's diamond-shaped Gastroduodenostomy are better procedures to be done. Heineke-Mikulicz pyloroplasty should be avoided as it is difficult to suture transversely after vertical incision because of the presence of fibrosis in the strictured segment.

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Introduction: Pancreatic pseudocysts (PPCs) and walled-off necrosis (WON) in children following acute pancreatitis are uncommon. The various modalities of therapy possible are conservative treatment, external drainage, endoscopic stenting, and internal surgical drainage procedures. There are no existing guidelines for the management of PPC in children. We evaluate the outcomes of laparoscopic cystogastrostomy (LCG) performed at our center. Materials and Methods: Eight children (median age: 10 years) underwent LCG for large PPC (median size: 12.5 cm). There were seven patients with PPC and one with WON. Seven underwent LCG by a transgastric approach and one underwent LCG by a retrogastric approach. Results: Seven out of the eight patients had complete resolution of symptoms and the PPC. The median follow-up period was 32 months (interquartile range: 9.5–55.5 months). There were no conversions. There was one patient with a WON who developed a recurrence. Conclusion: LCG is a safe and effective treatment option for large PPC/WON in children. A posterior retrogastric approach, when indicated, is a safe approach with a comparable outcome.

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Introduction: Despite remarkable improvement in Wilms' tumor (WT) survival in Western world, sub-optimal outcome in resource-constrained settings is influenced by late presentation, larger size, and poor access to treatment. This prompted us to study the outcome at a tertiary care center and to identify the global and local practice gaps. Materials and Methods: A retrospective, observational study of WT was conducted from October 2009 to September 2019 at a tertiary care setting. Following the National Wilms' Tumor Study Group protocol, an upfront nephrectomy (unilateral resectable tumors) and preoperative chemotherapy (large/unresectable Stage I–III) were followed. The records were reviewed for demographics, stage, preoperative chemotherapy, predictive factors, and outcome. Survival curves were plotted by the Kaplan–Meier method, and analysis was performed using the SPSS software version 16. Results: One hundred and fifty-six children were included, median age was 4.1 years, with a male predominance. The most common stages of the presentation were II (40.4%) and III (34.6%). An upfront surgery was done in 27.6%, while remaining received preoperative chemotherapy. The median follow-up was 22 months, and the events included relapse in 46 (29.48%) and death in 54 (34.61%). The mean survival time was 45.7 (95% confidence interval [CI], 41.08–50.30). The 2-year overall survival was 65.38% (95% CI, 59–73), and the 2-year event-free survival was 36% (95% CI, 32–41). On comparison of the impact of preoperative chemotherapy, the survival estimates in Stages I–III and relapse rate were statistically similar, tumor size reduced significantly, and tumor spill was significantly lower (P < 0.05). Conclusion: WT is associated with late presentation, sub-optimal survival, and higher relapse in our setting associated with practice gaps related to the management including practice violations.

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Septic thrombophlebitis of the portal vein or pylephlebitis is a rare cause of morbidity and mortality in children. The common causes include infective intra-abdominal pathology (acute appendicitis or diverticulitis) or inflammatory conditions (acute pancreatitis and inflammatory bowel disease). Management involves a multidisciplinary team approach for favorable outcome. We present a case report of pylephlebitis secondary to perforated appendicitis with incomplete resolution of thrombosis.

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Castleman's disease is a rare lymphoproliferative disorder of poorly understood etiology. It is most commonly located in the mediastinum. Castleman's disease mimicking adrenal neoplasm is a very rare differential diagnosis and unusual presentation. We report a rare suprarenal unicentric hyaline vascular variant of the disease in a 16-year-old boy with atypical symptoms mimicking an adrenal neoplasm.

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Midline vascular abdominal wall lesions are likely to be mistaken for vascular malformations in young children. We report a case of large yolk sac tumor located in the anterior abdominal wall just below xiphisternum in a 20-month-old girl diagnosed by raised serum alpha fetoprotein levels and fine-needle aspiration cytology. Preoperative chemotherapy helped in reducing its size allowing wide resection and primary wound closure. This case is reported for the unusual location and role of chemotherapy in management.

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Anatomical fusion abnormalities of the urinary system and ureters are not uncommon. However, triplication of the ureter is very rare. A boy with this abnormality is being presented in view of its rarity. The importance of a thorough preoperative evaluation is being projected that may help us to choose the best management avenue.

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Introduction: Core needle biopsies of solid masses in children are a minimally invasive technique. It guides to a definitive diagnosis and facilitates management. Aims and Objectives: To determine the accuracy, sensitivity, and specificity of core needle biopsies in diagnosing pediatric solid masses. Materials and Methods: A retrospective analysis of 430 children, who underwent core needle biopsy for solid masses between January 2007 and December 2016 at CMC Vellore, was done. Results: Retroperitoneal and intra-abdominal masses constituted 66% of cases. Real-time image guidance was used in 44% of cases. An accurate diagnosis was obtained in 93.6% of cases, while results did not correlate with the final diagnosis in 3.4%. Three percent had inadequate or necrotic tissue. None of the children had postprocedure complications. Conclusion: Core needle biopsies serve as good diagnostic modality, with minimal risks, in making a conclusive diagnosis and deciding on the line of management.

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Lipoblastoma is a relatively rare tumor that occurs in infancy and early childhood which arises from embryonic fat. It occurs mostly in trunk and extremities, but abdomen and pelvic lipoblastomas are rare. It presents as a mass with or without mass effect. Computed tomography/magnetic resonance imaging of the abdomen with pelvis is the investigation of choice. We present two cases of pelvic lipoblastoma with extension in ischiorectal fossa and the intra-abdominal lipoblastoma which were excised completely by laparoscopy. We are reporting these cases because of rarity of the disease and feasibility of complete laparoscopic excision.

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Cirsoid aneurysm (CA) is a rare arteriovenous fistula of the scalp. There exists scant literature on the incidence and approach to CA in children. We describe a case of CA in a 7-year-old boy which was diagnosed by angiography and managed with angiographic embolization followed by surgical excision.

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Vascular malformation of the gastrointestinal (GI) tract along with metachronous lesions elsewhere in the body is extremely rare. We report an unusual case of a female child, with a history of venous malformation excision over the right leg presenting with severe iron-deficiency anemia. Although venous malformations of the small bowel are a rare cause of occult GI bleed, in the presence of cutaneous vascular lesions, there should be a high index of suspicion of a vascular malformation, along with the other causes of GI bleed in children.

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Colon duplication (CD) is a rare congenital abnormality with varied presentations. We present a neonatal case with complete tubular CD terminating uniquely as a common channel, a single rectovestibular fistula. We summarize surgical management options in CD as well as factors that will help to decide the best surgical treatment in individual cases.

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Esophagogastric fistula is a rare complication related to severe gastroesophageal reflux disease, previous surgery, or malignancy. We describe an unusual case of esophagogastric fistula associated with paraesophageal hernia in a 5-year-old girl. This is the first report in pediatric patients.

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