Choledochal cyst of the cystic duct is a rare anomaly; broadly classified, the pathology may either present as a solitary anomaly or with concomitant involvement of other parts of the biliary tree. In the absence of standard guidelines for surgical planning, the definitive management is based on the experience shared in the literature in the form of case reports and case series. The available options should be discussed with the patient while explaining the possible short- and long-term complications. The authors herewith present two cases of choledochal cyst of the cystic duct along with a review of literature relevant to their etiopathogenesis and its implications for the definitive management.

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Objective: The objective of this study was to compare mini-percutaneous nephrolithotomy (PCNL) performed by standard and Miniperc techniques in pediatric patients. Materials and Methods: This was a retrospective study conducted at our institution between January 2012 and December 2017. The outcomes of pediatric renal stones treated by mini-PCNL done by Miniperc and standard techniques were compared in terms of the drop in the hemoglobin, stone-free rate, and analgesic requirement in the first 24 h. Results: A total of 57 children (age: 1–16 years), who underwent mini-PCNL by Miniperc equipment (n = 23) and standard equipment (n = 34), were included in this study. The postoperative mean drop in hemoglobin was significantly higher in mini-PCNL done by standard compared to the Miniperc technique. The stone-free rate was 95.65% in the Miniperc group and 94.12% in the standard mini-PCNL group. The need for analgesics was significantly lower in the Miniperc group compared to the standard mini-PCNL group (P = 0.0002). In the Miniperc group, the majority of the patients required only one dose of analgesics, whereas, in the standard mini-PCNL group, around 44% of the patients required three or more than three doses of analgesics to reduce postoperative pain. Conclusion: Both the techniques were safe and efficacious in the management of pediatric renal stone and stone clearance. However, the Miniperc technique resulted in significantly less pain and a lower dosage of analgesics.

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Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.

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Aims: The aim of this study was to evaluate the factors associated with the failure of nonoperative reduction of intussusception in children. Methods: A retrospective study was conducted in a tertiary care pediatric surgery hospital. The children admitted to the department of pediatric surgery between November 2013 and February 2020 with the diagnosis of Intussusception were included. Results: A total of 106 (67%) children underwent pneumatic reduction. Eighty-nine (84%) children had a successful reduction. A higher rate of failed reduction was found in children who presented at or after 48 h of the onset of symptoms (P = 0.03) and abdominal distension at presentation (P < 0.002). On multiple logistic regression analysis, the children presenting at or after 48 h of the onset of symptoms (odds ratio [OR] = 11.3; P = 0.039) and abdominal distension at presentation (OR = 4.46; P = 0.021) were found to be associated with increased risk of failure of nonoperative reduction. The variables age <1 year, weight <10 kg, pain abdomen, vomiting, bilious vomiting, fever, bleeding per rectum, and palpable mass were not associated with the failed nonoperative reduction. The variables, presentation at or after 48 h of the onset of symptoms (OR = 2.812; P = 0.045) and abdominal distension at presentation (OR = 8.758; P = 0.000) were found to be associated with an increased need for surgery. Conclusion: The risk factors for failed nonoperative reduction of intussusception include a presentation at or after 48 h of the onset of symptoms and the presence of abdominal distension at presentation. The delayed presentation was associated with the increased need for surgery and increased chances of intestinal nonviability.

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Introduction: Tumors located at the hepatocaval junction with the involvement of hepatic veins conventionally warrant major liver resections. Appreciation of sizeable inferior right hepatic vein can allow preservation of uninvolved inferior segments of the liver. Operative Technique: Preoperative and intraoperative identification of sizeable inferior right hepatic and other accessory or communicating veins in combination of intra-operative ultrasound guidance for resection to preserve inferior segments of the liver with adequate venous drainage. Application of upper transversal resections for pediatric liver tumors at the hepatocaval confluence was illustrated with description of operative technique and patient selection. Conclusions: Parenchymal preserving surgeries are possible for pediatric liver tumors located at the hepatocaval confluence by identification of accessory draining veins to the inferior segments. This along with meticulous parenchymal dissection with ultrasound guidance to preserve all uninvolved veins is key to safe upper transversal resections.

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The aim of the study was to determine the effectiveness and complications of ultrasound (US)-guided percutaneous removal of foreign bodies (FBs). Eleven patients were evaluated for US-guided percutaneous removal of FBs in the upper and lower extremities between April 2018 and March 2020 at the pediatric surgery department of a tertiary-level referral hospital. Elongated FBs, without damage to the adjacent neural and vascular tissue, were included. FBs with open wound, those with glass piece, and irregular-shaped and very superficial palpable FBs were excluded.

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Phaeochromocytoma is a rare childhood adrenal medullary catecholamine secreting tumour, arising from the chromaffin cells of the sympathetic origin derived from the neural crest cells. Only a few cases have been reported in the literature so far. We report an uncommon presentation in a 5-year-old boy with intracerebral haemorrhage, hemiplegia, and paroxysmal hypertension. Magnetic resonance imaging of the brain showed chronic and acute hemorrhagic infarcts in the left cerebral hemisphere and no vascular anomaly seen on cerebral venogram. Computed tomography scan abdomen showed a mass in the lesser sac and urinary catecholamines were elevated. The patient underwent exploratory laparotomy and the mass was excised in toto. Histopathological examination (HPE) confirmed the diagnosis. This case illustrates the need for keeping in mind atypical presentations of phaeochromocytoma especially in children; as it is a treatable cause of hypertension and early diagnosis with adequate management can prevent morbidity and fatal outcomes.

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Acquired Rectovaginal Fistula (RVF) is rare in infants. Interleukin10/ Interleukin 10 receptor deficiencies are monogenic disorders presenting as aggressive forms of infantile onset inflammatory bowel disease with perianal abscess and fistula. Genetic studies assist in confirming the diagnosis. We present a two month old infant with rectovaginal fistula, severe colitis, failure to thrive and recurrent infections in whom colonoscopy revealed irregular colonic ulcers, and genetic studies confirmed an IL10RB mutation. Hematopoietic Stem cell transplantation is the definitive therapy for this disorder which the child underwent. We report this infant with an acquired RVF with extraintestinal features due to IL10RB mutation to highlight the importance of thinking beyond the local anatomy and looking into the genetic domain.

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Priapism due to pseudoaneurysm of the cavernosal artery is very rare. Herein, we describe the magnetic resonance imaging and digital subtraction angiographic findings of pseudoaneurysm in the right corpus cavernosum in a 7-year-old boy who presented with pain in the penis and lower abdomen and priapism with an obscure history of trauma. This was managed by endovascular embolization of the aneurysm. One-year follow-up has shown him to be normal without any symptoms and normal erection.

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Large neck masses involving the airway can lead to hypoxia or the demise of the newborn in case the airway is not secured in time. A planned ex utero intrapartum treatment (EXIT) enables to access the airway by various means under optimal conditions. Advancements in imaging and well-orchestrated teamwork enable to improve the survival by EXIT procedure.

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Calyceal diverticuli are rare entities which are difficult to diagnose radiologically as they mimic various other pathology.Close follow up is essential even in asymptomatic individuals to prevent complications. The authors present a giant calyceal diverticulum in a solitary kidney in a child that was managed by open surgery.

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Iatrogenic urethral foreign bodies are rare conditions in clinical practice, and the relevant data are limited to adult cases in the literature. In this paper, we presented a pediatric case in which a fragment of Foley catheter balloon remained in the posterior urethra.

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Thyroglossal cyst is the most common congenital neck anomaly in children with equal sex incidence, but papillary carcinoma of the thyroglossal cyst in children is rare with only 10 cases reported so far. Even in adults, the incidence of malignancy in the thyroglossal cyst is only 1%. Most cases are diagnosed following surgery on histopathological examination; there is no consensus on the management owing to this rarity and indolent but unpredictable behavior of thyroid cancers. Here, we present one such rare case.

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The rarity, diverse clinical presentation, relatively featureless sonography, and diagnostic dilemma associated with pilomatrixomas may trigger anxiety and call for unwarranted investigations. The authors have shared their experience in two consecutive cases to generate awareness for such lesions.

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Aim: The aim of this study was to review the compliance to the provisional date of elective surgery allotted to pediatric surgery outpatients at our center. Patients and Methods: This retrospective study was conducted in the department of pediatric surgery at our center. The children who were brought to the outpatient department with a previously allotted date of admission for elective surgery (DAES) and the final operation theater (OT) lists of elective surgery between July 2007 and September 2018 under one senior consultant (VB), were reviewed. Results: A total of 2409 cases were allotted a DAES during the study period. Out of these, 12% (288/2409) were defaulters with a male gender preponderance (82%). Most of the defaulters (>49%) belonged to the day-care surgery group, followed by simple reconstructive (32%), major reconstructive (18%), and infectious sequelae groups (<1%). The difference in the proportion of defaulters from both the reconstructive groups was statistically significant (P = 0.0001). On a further system-wise subcategorization of children in the day-care group, the genitourinary subgroup constituted the majority (80%). Children who had to undergo staged procedures showed a significantly better compliance (P < 0.0001) to provisional DAES as compared to those belonging to the single-stage group. Conclusions: Compliance to provisional DAES is poor among the children scheduled for day-care surgeries and those undergoing simple reconstructive surgeries in a single stage.

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Purpose: The purpose of the study was to survey the opinion of delegates attending national pediatric urology meeting regarding live case demonstrations (LCDs) to see whether these can be replaced with taped case demonstrations (TCDs) in future. Methods: A questionnaire-based survey was conducted at the end of the 3-day annual conference and live operative workshop in pediatric urology. Apart from general data such as age of the respondent and type of practice setting, four key questions on LCDs with yes/no responses included: would you have attended this meeting if there were no LCDs? Are unedited videos (TCDs) as effective as LCDs for teaching? Do you think LCDs should be continued? Would you allow your child to be operated in LCD by an expert? For question 3, the outcomes were compared between junior surgeons (<45-year-old) and senior surgeons (>45-year-old). Results: On analysis, 88/140 (62%) respondents (95% confidence interval [CI]: 54%–70%) said that they would not have attended the meeting if there were no LCDs; 70/139 (50%) respondents (95% CI: 42%–58%) felt that TCDs may be an effective alternative to LCDs; 129/144 (90%) respondents (95% CI: 83%–94%) felt that LCDs should be continued. For question 4, 101/129 (79%) said they would allow their child to be operated in LCD by an expert, while 28 (21%) did not agree for their child to be operated in LCD. There was no significant difference between junior and senior surgeons regarding support for LCDs (P = 0.15). Conclusions: In the Indian scenario, LCDs were favored as an essential part of pediatric urology meeting, attract more participation, and are likely to be important for the success of the meeting.

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Objective: Congenital pulmonary airway malformation (CPAM) is a rare developmental lung disease. The aim of this study is to analyze the histomorphological spectrum of CPAM in a series of 15 cases. Materials and Methods: A retrospective descriptive study of 15 cases of CPAM was carried out from 2013 to 2018 in our hospital, and cases were classified based on the Stocker's classification. Results: The age ranged from 4 days to 9 years (66.6% were infants). The left lung was most commonly involved (66.6%). The most common lobe was the left upper lobe (60%), followed by right lower lobe (20%). Grossly, cysts measured 0.2–5 cm, filled with mainly serous fluid with few having hemorrhagic and brownish mucoid secretions. On microscopy, single to multiple noncommunicating cysts of size 0.2–5 cm were seen, lined by ciliated columnar epithelium (60%), pseudostratified ciliated columnar epithelium (26.7%), mucin-secreting columnar epithelium (6.7%), and flattened epithelium (6.7%). Few cases showed smooth muscle (20%) and cartilage (13.3%) in the cyst wall. Chronic inflammation (73.3%) with dense histiocytic infiltrate (13.3%) was also seen. Emphysematous changes were also observed (13.3%). Cytomegalovirus inclusions (6.7%), zygomycete fungus (6.7%), and red hepatization (6.7%) were observed. The most common type was type II (60%), followed by type I (33.3%) and type IV (6.7%). Conclusion: Type II was the most common variant in this study. A careful observation should be done to look for fungal hyphae or viral inclusions.

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Introduction: Pyloric atresia is a rare cause of congenital gastric outlet obstruction. It is often associated with epidermolysis bullosa (EB). Rarity and experience with 11 cases are the reason for this publication. Aims and Objectives: The aim and objective of this study is to present our experience of 11 cases of congenital pyloric atresia and correlate with available literature. Materials and Methods: This was retrospective cohort of 11 cases correlative comparative study. Data of all the 11 cases from 1982 to 2019 were collected, reviewed, and analyzed. The parameters studied included age, gender, antenatal diagnosis, postnatal diagnosis, preoperative management, intraoperative findings, postoperative course outcome, associated anomalies, and any genetic studies if done. All these parameters were compared with published data. Results: There were 11 cases in the present series with six boys and five girls. Most of them presented at varying periods from birth to day 1 of life. Eight cases of type 1 pyloric atresia, two cases of type 2 pyloric atresia, and one case of type 3 pyloric atresia constituted the cohort. Five out of 11 cases were associated with EB. Two out of six cases with isolated pyloric atresia and four out of five cases with EB died. Discussion: Congenital pyloric atresia may be isolated or associated with EB. Three varieties of pyloric atresia were described. Association with EB increases the mortality. Conclusions: Review and analysis of 11 cases of pyloric atresia compared with published literature is being reported.

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Background: Benign renal tumors are extremely rare and were studied here. This series also includes a renal teratoma in a horseshoe kidney, probably only the second in the pediatric literature. Materials and Methods: Retrospective review of children with benign renal tumors operated between 2006 and 2018 at one center. Results: Twelve patients (M:F ratio 10:2), age range 3 weeks (31-week gestation) to 13 years presented with large palpable renal swelling (n = 12) and hematuria (n = 3). Computed tomography (CT) scan showed features typical of the tumor. Final histopathology (age group [mean]) showed: multilocular cystic nephroma (MLCN) - n = 5 (41.7%), (11–16 months [13.6]); congenital mesoblastic nephroma (CMN) - n = 4 (33.3%) (classic 1, cellular 3) (0.75–5 months [2.125]); mature cystic teratoma - n = 1 (8.3%): (48 months, in a horseshoe kidney), and angiomyolipoma (AML) - n = 2 (16.7%) (144 months [sporadic] and 156 months [tuberous sclerosis]) One patient with cystic teratoma with no calcification on CT scan received pre-operative chemotherapy as fine-needle aspiration cytology (FNAC) reported malignant small blue cell tumor. Nephroureterectomy with Gerota's fascia could be done easily in all without intraoperative complications. Delay in presentation in MLCN and CMN led to increased symptoms and CT scan changes. All patients did well in 1.5–12 years (median 3 years) follow-up including cellular mesoblastic nephroma. Conclusions: Benign renal tumors often occur in specific age groups but may overlap that of Wilms tumor. Proper interpretation of clinical presentation, CT scan, and FNAC findings help in avoiding preoperative chemotherapy. Upfront nephroureterectomy is curative. Histopathological findings decide further treatment. Children with AML and tuberous sclerosis need lifelong follow-up.

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Introduction: Intestinal fatty acid-binding protein (I-FABP) is located in the apex of mature enterocytes and released into circulation; once the injury of enterocyte happens, its circulating levels are considered an early and sensitive marker of intestinal ischemia as in necrotizing enterocolitis (NEC); because of its small molecular weight, it can be detected in urine. Aims: The aim was to study the usefulness of both serum and urine I-FABP in early diagnosis of NEC and to correlate the serum and urinary levels. Settings and Design: This study was case–control design. Methods: Simultaneous serum and urine samples obtained at the onset of symptoms, in 40 preterms with suspected NEC, with gestational age ± 27.70 weeks and birth weight ± 1.11 kg, i.e., 20 preterms diagnosed at Stage I, 12 preterms at Stage II, and 8 preterms at Stage III, were compared with age- and weight-matched preterms with no NEC. Statistical Analysis: The collected data were tabulated, coded, and then analyzed using the computer program Statistical Package for the Social Science (SPSS version 22). Results: Serum levels of I-FABP in NEC cases were significantly higher than the control group, with a mean of 6005.77 ± 6384.77 and 1480.79 ± 1276.48 pg/ml, respectively (P < 0.001). Urine levels of I-FABP in NEC cases were significantly higher than the control group, with a mean of 5009.22 ± 3941.64 and 2677.62 ± 2257.29 pg/ml, respectively (P = 0.04). Both serum and urine I-FABP levels not only in Stage II are significantly higher than Stage I but also in Stage III are significantly higher than Stage I and II (P < 0.001, P = 0.03, respectively), which showed significant positive correlation with stages of NEC (r = 0.618; P < 0.001; r = 0.306; P = 0.049, respectively). Both serum and urine I-FABP levels had a highly significant positive correlation with each other (r = 0.406 P < 0.0001). Receiving operating characteristic curve showed an area under the curve of 0.92 and 0.81 for serum and urine I-FABP, respectively. Conclusions: Whether serum or urinary I-FABP is valuable in the diagnosis and prediction of NEC and strongly correlated with the disease severity and with each other.

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Introduction: Incidence of pediatric urolithiasis has increased over the last few decades. Procedures such as extracorporeal short wave lithotripsy, percutaneous nephrolithotripsy, and ureterorenoscopic lithotripsy are not widely available for pediatric age group in many developing countries. It is desirable that advantages of minimally invasive surgery be offered to selected cases with urolithiasis. Materials and Methods: All patients with pediatric upper tract urolithiasis managed laparoscopically from January 2015 to April 2020 were retrospectively reviewed. Results: A total of 38 patients were included. The mean age of the patients was 8 ± 2.85 years. Thirty-four patients (renal and upper ureteric) were managed through retroperitoneal approach, while those with lower ureteric calculi (n = 4) were approached transperitoneally. A total of eight patients required conversion to open technique. The stone clearance rate was 79% by laparoscopic approach alone. There were no procedure-related complications. Conclusion: Our study suggests that laparoscopic management for pediatric upper tract urolithiasis is a radiation-free, single-time curative treatment and is feasible in centers where facilities for other endoscopic procedures are unavailable.

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