Background: Intestinal malrotation is a congenital anomaly resulting from abnormal or incomplete rotation and fixation of the midgut during embryogenesis. It commonly presents in the neonatal period (75%) with sudden onset bilious vomiting and rarely beyond infancy (<10%). Aim: The aim of the study was to highlight the clinical features, radiological findings, and treatment outcomes of patients with malrotation presenting beyond infancy. Materials and Methods: Eleven consecutive cases of delayed presentation of malrotation presented over a period of 5 years (2017–2021). Data were analyzed retrospectively. Results: Out of the 11 patients, four were female and seven were male. The age of patients ranged from 14 months to 18 years. Patients beyond infancy present usually with diffuse pain abdomen compared to neonates which present with sudden onset bilious vomiting and therefore difficult to diagnose. Five patients had associated abnormalities such as intussusception or nutcracker syndrome or mesenteric cyst or jejunal stricture or mesenteric lymphadenopathy along with malrotation. Patients underwent ultrasonography, upper gastrointestinal contrast study, and contrast-enhanced computed tomography abdomen to confirm the diagnosis. All patients underwent the Ladd procedure with four requiring resection anastomosis and one requiring excision of the mesenteric cyst. Eight out of eleven patients had favorable outcomes, two develop adhesive intestinal obstruction and required re-exploration, and one had persistent complaints of hematochezia. Conclusion: Malrotation beyond infancy is a rare diagnosis. Malrotation in older children is usually not suspected because of the wide range of symptoms. A high index of suspicion on ultrasound or computed tomography is required to demonstrate the reversal of superior mesenteric artery and superior mesenteric vein position and related conditions. Early intervention and treatment can prevent catastrophic events such as intestinal volvulus and intestinal ischemia in these patients.

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Background and Aims: In this cost-effectiveness model, we compared the cost-effectiveness of commonly used urinary biomarkers with conventional diuretic renogram (DR) in diagnosing ureteropelvic junction obstruction (UPJO). We hypothesized that urinary biomarkers are as effective as DR. Methods: We used incremental cost-effectiveness ratio (ICER) as a tool for our cost-effectiveness analysis model. The cost of biomarker assay and renogram were sourced from the same center while the accuracy data of DR and urinary biomarkers from the relevant pubications. Results: As the accuracy of individual biomarker increased, the ICER also got better. As a panel of biomarker was introduced, the ICER went to the negative range suggesting cost saving as well. ICER of most urine biomarkers is currently less expensive and less effective. When a biomarker panel was applied ICER became more expensive and effective. With higher samples, test running cost is likely to go down in future and thus biomarkers are likely become less expensive and more effective. Conclusions: Individual urine biomarkers are currently less expensive and less effective compared to DR in predicting UPJO. In future, biomarker panel is likely to be more cost-effective and reduce the need for invasive renogram thus reducing the radiation exposure.

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Background: Severe pruritus caused by progressive familial intrahepatic cholestasis (PFIC) and Alagille syndrome (AGS) is refractory to medical treatment. Surgical interruption of the enterohepatic circulation is considered the mainstay of alleviating distressing symptoms and delaying cirrhosis. Aim and Objectives: This study aims to evaluate the short-term effect of partial external biliary diversion (PEBD) on pruritus, liver disease progression, patient's growth, and quality of life. Material and Methods: This prospective cohort study enrolled children with PFIC and AGS from July 2019 to July 2021, whose guardians consented to the PEBD procedure. A standard surgical approach was performed by a single surgeon. Outcomes were measured subjectively and objectively pre- and post-procedure using the pruritus 5-D itching score, Paediatric Quality of Life Inventory scale (PedsQL), growth parameters, bile acids level, and liver function tests. Patients' follow-up period ranged from 6 to 12 months. Results: Seven patients had PEBD procedure; five with PFIC and two with AGS. A significant improvement was detected in the 5-D itching score (p-value < 0.001), PedsQL (p-value < 0.001), and bile acids level (p-value 0.013). The preexisting growth failure was ameliorated. The downward trend in the bilirubin level was not significant. No influential difference in the other liver function tests occurred. No intra-operative complications encountered. Only one case had a post-operative stoma prolapse which was managed surgically. Conclusion: PEBD procedure could be considered as an effective and safe treatment options for intractable pruritus in patients with PFIC or AGS, providing preserved synthetic liver functions.

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Introduction: Invasive urodynamics (UDS) is a standard investigation in children. Studies measuring voiding pressures in children use varied nomenclatures and quote a wide range of voiding pressures. Thus, voiding pressures in children are not considered reliable and they do not find any place in the pediatric diagnostic armamentarium. On the contrary, adult studies have well-defined nomograms and standard values which make voiding studies indispensable in the diagnosis of voiding dysfunctions in adults. The difference primarily lies in the uniformity of parameters assessed in adults and the contrasting heterogeneity in the pediatric literature. Objective: The objective of this study was to study the voiding parameters observed during UDS in boys. Study Design: We retrospectively reviewed the pressure flow data obtained during conventional invasive UDS in 106 neurologically normal boys (6 months–16 years) who had different indications for urodynamics. The values of Pdetmax and PdetQmax were analyzed and compared with the existing data of pressure flow studies in children. Results: Pdetmax decreased with age whereas PdetQmax was independent of age. The difference between the values of Pdetmax and PdetQmax was more in the younger kids. The wide range of voiding detrusor pressure (Pdet) in the existing pediatric literature is similar to the values of Pdetmax observed in our study, whereas the value of PdetQmax is much lower. Discussion: The values of Pdetmax observed in this study are similar to the values of “maximum Pdet during voiding” documented in previous studies and are determined by detrusor contractility and functional/dynamic contraction of outflow during voiding. PdetQmax has been documented in very few pediatric studies and is significantly less than Pdetmax. Further prospective studies are needed to corroborate UDS findings with radiologic/cystoscopic findings to create nomograms of voiding parameters in children. Conclusion: Existing literature on pediatric voiding studies mentions voiding pressures during variable phases of void (usually Pdetmax) and the values have been very heterogeneous, making voiding pressure-flow studies unreliable in children. PdetQmax values are much lower than values quoted as “standard” pressures and are age independent. The use of PdetQmax instead of PdetMax may make voiding pressures in children more reproducible and informative.

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Background: Posterior urethral valve (PUV) is obstructive uropathy that may lead to chronic kidney disease (CKD) and end-stage renal disease (ESRD) in children. Glomerular filtration rate (GFR) measurement remains the gold standard for renal function measurement. However, due to its less availability and cumbersome, it is not commonly used, and GFR is estimated utilizing various endogenous filtration markers. Objective: This study includes pediatric patients with PUV. We aimed to compare the measured GFR (mGFR) with various creatinine-based estimated GFR methods (eGFR). Materials and Methods: A single-center retrospective study included 62 treated cases of PUV, postvalve fulguration. The mGFR measured by 99mTc-diethylenetriaminepentaacetate in vitro method and compared with eight eGFR (Schwartz, Cockcroft-Gault [CG], Counahan-Barratt [CB], CKD Epidemiology Collaboration [CKD-EPI], full-age spectrum [FAS] age, FAS height (FAS Ht), Schwartz-Lyon [SL], and Ht independent). Patients were subdivided into different CKD grades and compared with various eGFR. Discussion: PUV is a common cause of CKD in children and needs special consideration as there is growth retardation associated with it. It decreases creatinine production and thus fallacies in eGFR measurement. There is a requisite to identify and closely monitor the subset of patients with baseline decreased renal function and therefore at risk of developing ESRD. Results: A total of 62 patients were included. Mean age and serum creatinine levels were 8.02 ± 5.53 years and 1.15 ± 0.95 mg/dl (range: 0.4–4.5), respectively. The mean mGFR was 61.6 ± 31.80 mL/min/1.73 m² and a positive variable correlation was 0.46–0.77 between mGFR and eGFR. Based on mGFR, there were 14 (22.6%), 21 (33.8%), 13 (20.9%), 9 (14.5%), and 5 (8.1%) patients in Grades I–V, respectively. The correct classification of the CKD grades was noted in 25 (40.3%), 16 (25.8%), 32 (51.6%), 16 (25.8%), 25 (40.3%), 27 (43.5%), 26 (41.9%), and 28 (45.2%) patients by Schwartz, CG, CB, CKD-EPI, FAS age, FAS Ht, SL, and Ht-independent equation. The eGFR overestimates GFR at the lower level and underestimates at higher levels. Conclusion: Our results confirm the considerable limitations of various creatinine-based clearance methods for estimating actual GFR. The creatinine clearance-based eGFR should not replace the measurement of the GFR. An initial measure of the mGFR followed by serial follow-up with the eGFR equation may be done. The most accurate eGFR equations are CB for Grade II, SL or Ht independent for Grade III, FAS age for Grade IV, and SL for Grade V CKD.

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Vulvar lichen sclerosus et atrophicus is an inflammatory disease which can affect the pediatric age group, although rarely. Its prevalence is underestimated. It may have genital or extragenital manifestations. If left untreated, it may progress to complications which hamper the quality of life. The treatment of choice is topical clobetasol propionate. Here, we present a case of prepubertal vulvar LS, which was refractory to topical clobetasol but responded to topical tacrolimus application.

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Congenital tracheoesophageal fistula (TEF) is a result of the failed fusion of tracheoesophageal ridges during the 3^rd week of gestation. Commonly, TEF is associated with esophageal atresia, which is detected and treated in newborns. Isolated TEF is rare with an overall incidence of 1 in 80,000 live births. Very rarely, they can present in adulthood. This report is adding one such patient at the age of 17 years who presented with the first episode of aspiration pneumonia and a history of retrosternal discomfort and regurgitation since childhood to the limited world experience.

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Background: Kasai portoenterostomy (KPE) is the initial treatment for biliary atresia (BA). Even after initial jaundice clearance, a significant number of children presented with the reappearance of symptoms due to ongoing fibrosis involving porta and intrahepatic ducts. Mitomycin-C (MMC) is an antifibrotic agent, and the study hypothesized that local application of MMC at porta can decrease fibrosis, which can improve jaundice clearance and lead to better native liver survival (NLS). Materials and Methods: This prospective randomized control trial included children with BA, who were allocated to groups A or B. The patients in both groups underwent standard KPE; in addition, a 5 French infant feeding tube (IFT) was placed near the porta through the Roux limb in Group B children. During the postoperative period, MMC was locally instilled over the porta in Group B children through IFT. Postoperative jaundice clearance and NLS were assessed and compared. Results: A total of 27 children were enrolled in the study, 16 in Group A and 11 in Group B. Both groups were comparable preoperatively. Although the NLS was not statistically significant in Group B, the survival was quite higher, that was 91%, 81%, and 73% at 6 months, 1 year, and 2 years, respectively, compared to 63%, 50%, and 38% in Group A. Conclusion: Children in Group B clinically showed an early jaundice clearance and a better trend of serial bilirubin levels as well as longer NLS than Group A, but it was not statistically significant. The procedure was technically easy, and no complication was encountered related to surgical technique or MMC instillation.

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Aim: Enhanced recovery after surgery (ERAS) are multimodal perioperative pathways that have shown improved outcomes. ERAS after colostomy reversal has shown promising results in adults and few pediatric studies. We report our experience using ERAS for a colostomy reversal. Materials and Methods: A retrospective analysis of children in whom ERAS was used during colostomy reversal between May 2016 and 2019 was carried out. ERAS protocol in our study included avoiding mechanical bowel preparation (MBP), oral liquid diet upto 3 h preoperatively, usage of regional anesthesia, minimal handling of bowel intraoperatively, using nonopioid analgesics for pain relief, early initiation of feeding on the first postoperative day, early discharge once full feeds are established. Outcomes analyzed are the duration of hospital stay and complications, including readmissions. Requirement for opioids and anti-emetics are noted. The outcomes are compared with traditional care pathways (TCP), which use MBP, overnight fasting, opioid analgesia, and delayed feeding. A total of 48 are included in the study, with 13 cases using ERAS and TCP in 35 cases. Statistical Analysis Used: Nonparametric Mann–Whitney U-test was used. Results: In the ERAS group, the mean length of hospital stay (LOS) postoperatively was 3.7 days (2–5 days) as opposed to 7.2 days (5–11 days) in TCP. There was only one child with complications in the ERAS group, while 9 cases in TCP had complications, though none of them required operative intervention. There was the requirement of anti-emetic in only one child in the ERAS group. Conclusion: ERAS for colostomy reversal is feasible in the pediatric population. For successful implementation, all personnel involved in the care of the child need to be educated about the protocol. It reduces LOS and complications.

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A 4-year-old male child presented with complaints of abdominal pain and vomiting along with yellowish discoloration of the eyes. Investigations were suggestive of acute pancreatitis and double gall bladder (GB) with dilated common bile duct (CBD) with intraluminal calculi and Type II choledochal cyst. He underwent surgical resection of double GB with dilated CBD with hepatico-docho-jejunostomy. On follow-up, the patient was asymptomatic. Our case highlights the importance of preoperative diagnosis to deal with increased operative difficulty and complications.

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Background: Cases of hypospadias present for poor stream or cosmetic appearance. The main aim is to provide a visibly normal phallus. Preputial reconstruction is technical. A properly planned reconstruction based on anthropometry may improve the result. We are presenting our experience of reconstruction based on glans anthropometry. Aim: The aim of the study was to evaluate the importance of glans anthropometry in preputial reconstruction in cases of hypospadias. Materials and Methods: All cases of hypospadias operated between June 2014 and March 2022 were included. Glans width was measured at the base. The marking sutures for preputial reconstruction were taken at distance thrice the glans width at base. Those requiring religious circumcision along with repair, associated significant chordee, catheter came out before 2 weeks, or history of any previous penile surgery were excluded. All the cases were subjected to urethroplasty, meatoplasty, and preputioplasty. The results obtained were analyzed. Results: One hundred and forty-eight out of 159 cases formed the study group. There were 31 glanular, 42 distal penile, 58 mid–penile, and 17 proximal penile hypospadias. Mean glans width at base was 16 mm (range: 11–21 mm). Mean distance of marking suture at prepuce was 38 mm (range: 33–63 mm). Mean follow-up was 12 months (range: 1–36 months). Mean age at presentation was 23 months (range: 14–72 months). Mean operating time was 50 min (range: 45–60 min). Fistula at the base of preputioplasty was seen in four. Dehiscence of preputioplasty was seen in six. Meatal stenosis was seen in three cases. Conclusion: Preputial reconstruction improves the cosmetic appearance of the hypospadiac penis. Reconstruction based on glans anthropometry improves the result and avoids complications.

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Aim: The aim of this study was to present the long-term institutional experience and outcomes of our Malone antegrade continence enema (MACE) procedure using the proximal appendix without any antireflux procedure. Materials and Methods: A single-center prospective study of 29 children undergoing a simplified Malone antegrade continence enema (SMACE) procedure from 2006 to 2017 was conducted using the appendix, whole or split. The mean follow-up period was 11.5 years (ranging from 5 to 16 years). Results: In 25 children, the proximal appendix was used, and in 4 cases, the whole appendix was used as a conduit. On follow-up, the MACE channel has been working well in 29/29 patients. Among the complications, seven patients had stomal stenosis, which was managed by home dilatation. There was no reflux of stools seen in any of the patients. Conclusion: The SMACE procedure, without incorporating an antireflux mechanism, is technically simpler and saves operative time. Most importantly, the results are satisfactory and comparable with procedures using antireflux techniques.

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Hypertensive crisis is rare in children. Among the rarest causes leading to this acute crisis, is bilateral adrenal tumour as a part of a syndrome, Von Hippel Lindau syndrome. The treatment is based on the excision of the adrenal tumour followed by long term surviellence. The authors present a case where conventional imaging with ultrasound and contrast enhanced CT scan demonstrated a right side adrenal tumour with raised catecholamines.The small left side tumour got missed on conventional imaging and got picked up on DOTA scan. The persistence of hypertension in post operative period can be related to such hidden functioning tumours. In view of the size of tumour & raised dopamine associated with high incidence of malignancy, robotic surgery was used for bilateral adrenal tumour excision which confirmed the diagnosis of Pheochromocytoma (PCC).

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Colonic atresia (CA) is an uncommon type of intestinal atresia commonly associated with other anomalies, while biliary atresia (BA) is also rare but usually an isolated anomaly. The pathogenesis for either of the anomalies is unclear. The co-occurrence of both pathologies has not been mentioned in the literature. We here discuss the management of CA with BA and the review of pertinent literature.

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Thoracoscopic surgery was not previously accepted in the neonatal population due to inappropriate instrumentation and lack of experience. However, our experience in the last few decades has slowly yet steadily established its safety and efficacy. The major advantages that thoracoscopy offers are early recovery and fewer long-term complications. However, we are aware that this comes at the cost of a steep learning curve and the potential challenge of facing certain complications which may compel a conversion to open. There is a paucity of literature regarding intraoperative complications of neonatal thoracoscopy and its management. Conversion to open thoracotomy is appropriate, keeping patient safety in mind, and any decision made to continue management of a complication thoracoscopically is technically demanding. Iatrogenic bronchial injury is one such rare complication of thoracoscopy with a limited mention in literature. We describe below a 25-day-old patient with a bronchogenic cyst who sustained injury to the left bronchus during thoracoscopic cyst excision, which was successfully repaired thoracoscopically.

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Anorectal malformations (ARMs) are common congenital anomalies in neonates. Colonic perforation is very rare in ARMs. Delay in diagnosis of neonate with ARM results in colonic perforations and life-threatening morbidity. Colonic perforation due to ARM may not be completely avoided; however, early diagnosis and management are essential in assuring better outcomes.

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Urethral duplication is a rare anomaly with varying clinical manifestations. About 300 cases have been reported till date and are often associated with genitourinary (GU) and gastrointestinal (GI) tract anomalies. A 6-day-old neonate presented with an absent anal opening and a tiny perineal fistula having slight meconium staining. He did well after anoplasty. Low anorectal malformation (ARM) has been reported as the GI anomaly associated with urethral duplication. However, an ectopic scrotum as the principal GU tract anomaly associated with urethral duplication is being reported for the first time in literature with probable embryologic explanation. The GU tract anomalies were successfully repaired surgically later in infancy.

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