Successful Laparoscopic Management of Zinner Syndrome in a Pediatric Patient.

Zinner syndrome, a rare congenital anomaly affecting males, is characterized by atresia of the ejaculatory duct, seminal vesicle cysts, and ipsilateral renal agenesis. This case report details a 2-year-old boy successfully treated with laparoscopic excision of a dilated vas deferens and seminal vesicle cyst. The rarity of Zinner syndrome in pediatric patients underscores the importance of understanding its diagnosis and minimally invasive surgical management.

Makeshift Shunts in Extrahepatic Portal Vein Obstruction in Pediatric Population.

Background and Objectives: More than 20% of patients with extrahepatic portal vein obstruction (EHPVO) may be deemed as nonshuntable due to lack of a suitable vein. The role of "makeshift shunts" or "lesser shunts" assumes importance in such cases. In this report, the authors have shared their experience with the makeshift shunts in the management of portal hypertension in children with emphasis upon anatomic considerations, resolution of symptoms, outcomes after surgery, and shunt patency. Materials and Methods: During the period 1983-2018, 138 children with portal hypertension were managed under the care of a single surgeon (VB). Of them, 134 were EHPVO. Children with EHPVO were treated with splenectomy and proximal lienorenal shunt (n = 107), splenectomy and devascularization (n = 21), and makeshift shunts (n = 6). Makeshift shunts comprised (i) side-to-side right gastroepiploic vein (Rt-GEV) to left renal vein (LRV) shunt (n = 1), (ii) superior mesenteric vein (SMV) to inferior vena cava (IVC) shunt using a spiral saphenous venous graft (n = 1), (iii) side-to-side inferior mesenteric vein (IMV) to LRV shunt (n = 2), (iv) side-to-side IMV to IVC shunt (n = 1), (v) end-to-side IMV to IVC shunt (n = 1), and (vi) side-to-side IMV to LRV shunt (n = 1) in a case of crossed fused renal ectopia. Results: Following the creation of portosystemic shunt, a decline in portal pressure was demonstrated in all six patients. There was resolution of symptoms including hematemesis, melena, and anorectal variceal bleed. None of the patients demonstrated the features of hepatic encephalopathy. The associated portal cavernoma cholangiopathy (n = 1) also resolved following Rt-GEV to LRV shunt. Shunt patency was documented for the entire duration of follow-up (1.5-4 years) in five of six patients; the sixth patient demonstrated shunt block at 6-month follow-up but without recurrence of symptoms. Conclusions: Makeshift shunts offer a viable alternative to standard portosystemic shunting in pediatric patients with a nonshuntable vein. The selection of such shunts is, however, subject to surgeon's preferences and has to be individualized to local anatomy.

Comparison of analgesic efficacy of ultrasound-guided quadratus lumborum block versus erector spinae plane block in children undergoing elective open pyeloplasty - A randomized, double-blinded, controlled study.

Background and Aims: Comparison of analgesic efficacy of ultrasound-guided transmuscular quadratus lumborum block (QL-3) and erector spinae block (ESP) in children undergoing open pyeloplasty was done in this study. Material and Methods: This was a randomized, double-blinded, controlled study conducted in a tertiary care center, operating rooms, post-anesthesia care unit (PACU), and paediatric surgical ward. Sixty children of age 1-6 years, with American Society of Anesthesiologists (ASA) status I or II, undergoing elective open pyeloplasty were included in the study. Patients were randomized into two groups: group I (QL block-3) and group II (ESP). Both blocks were performed under USG guidance using 0.5 ml/kg of 0.25% ropivacaine after induction of general anesthesia. Postoperative Modified Objective Pain Score (MOPS), perioperative hemodynamic parameters, perioperative time for first rescue analgesia, total rescue analgesia, and incidence of complications were recorded. Statistical tests were applied as follows: (i) quantitative variables were compared using independent t-test/Mann-Whitney test (when the data sets were not normally distributed) between the two groups, and repeated measure analysis of variance (ANOVA)/Friedman test was used for comparison between different time intervals within the same group and (ii) qualitative variables were correlated using the Chi-square test/Fisher's exact test. A P value of <0.05 was considered statistically significant. Results: Pain was assessed using MOPS in the postoperative period at 0, 30 min, 1, 2, 4, 6, 12, and 24 h. Overall, the pain scores were low and showed a decreasing trend toward baseline as time progressed. Group I showed lower score, but was statistically significant only at the sixth hour. Highest mean score was 2.4 ± 2.01 in group I and 2.67 ± 2.32 in group II. Perioperative hemodynamic parameters were comparable. Total rescue analgesia during the perioperative period was not statistically significant (intraoperative P = 0.075 and postoperative P = 0.928). Also, 63.33% patients in group I and 63% patients in group II required rescue analgesia in the postoperative period and were comparable. Mean ± standard deviation (SD) for first rescue analgesia time was 6.32 ± 12.57 in group I and 16.67 ± 31.25 in group II, but not significant. The distribution in group II was skewed, hence the larger value for group II, but when compared to group I, this was statistically not significant. Conclusion: Both ultrasound-guided ESP block and QL block using 0.25% ropivacaine 0.5 ml/kg provided adequate analgesia during the first 24 h post-surgery in children undergoing open pyeloplasty. The fentanyl requirement during the first 24-h postoperative period was also decreased.

Benign paediatric liver tumours: The radiological maze demystified.

The trajectory from the clinical identification of a benign liver mass to establishing a precise diagnosis is perplexing and arduous. Related contributory factors are that such lesions are encountered infrequently and that there is a nonavailability of dedicated paediatric radiologists in the developing world. The objective of this study was to review the spectrum of benign liver lesions in children and their typical imaging features. Cross-sectional imaging of all paediatric patients (< 18 years) with liver lesions (single and multiple) performed in the institute from 01 January 2018 to 01 January 2019 as well as those acquired at outside institutions and referred to the institute for management was included. Ultrasound was done as the first line of investigation in all the cases with suspicious liver masses and retrospectively performed in referral cases in whom CT or MRI was already done. Images were analysed by two senior radiologists. Most of the cases were diagnosed based on clinical, biochemical and imaging findings, and biopsy was only performed in equivocal cases. Most of the benign liver lesions in the paediatric age group were hepatic haemangioma and mesenchymal hamartomas. A simplified clinical-radiologic paradigm should be established for benign liver lesions in children to assist in reaching the correct diagnosis. Contribution: The article demonstrates the salient radiological findings of various benign liver lesions in the paediatric age group and the role of demographic, clinical and biochemical findings, which plays a substantial role in the diagnosis and avoids unnecessary biopsies.

Management of Stoma Prolapse Using Polypropylene Mesh Strip: A Novel and Easy Technique.

ABSTRACT: Stomal prolapse is one of the most common complications of enterostoma. Stoma if persistent and significant often requires revision of stoma. The techniques described often require laparotomy with resection or internal fixation of the bowel. Such procedures may make further laparotomy more difficult. We herein describe the use of a relatively simple procedure for the correction of stomal prolapse.

Anthropometric Outcomes in Survivors of Pediatric Solid Tumors.

Nutritional status is an important aspect of childhood cancer, with a bearing on the disease and subsequent survivorship. We sought to evaluate the long-term anthropometric outcomes in a cohort of pediatric solid tumor survivors treated between 1994 and 2016. Anthropometry was noted at the time of presentation, after completion of therapy, and at the last follow-up. The z-scores for weight-for-age, height-for-age, and BMI-for-age were calculated using WHO growth charts for ages <5 years and the Indian Academy of Pediatrics growth charts for age >/= 5 years. BMI was calculated for adult survivors. We included 317 survivors, comprising 48, 81, and 188 survivors of Hepatoblastoma (HB), Malignant Germ cell Tumor (MGCT), and Wilms Tumor (WT) respectively. The median age at diagnosis was 24.5 (IQR 59-13.2) months, with a follow-up ranging from 5 to 19.54 years. The z-scores of the collective cohort and individual cohorts of HB, MGCT, and WT showed an improving trend from diagnosis to the last follow-up. The difference in the prevalence of malnutrition was found to be statistically significant when any two-time points were compared. Of the 28 adult survivors, 43% were noted to be underweight. Thus, anthropometric measures improve during follow-up, however, up to 15% of children persist in being malnourished.

Role of Magnetic Compression Anastomosis in Long-Gap Esophageal Atresia: A Systematic Review.

Background: Magnetic compression anastomosis (MCA) is an alternative technique for patients with long-gap esophageal atresia (EA). It allows for preservation of the native esophagus. We aimed to systematically summarize the current literature on MCA in EA. Methods: Studies where neonates with EA were treated with MCA devices were included, while studies on esophageal stenosis were excluded. All clinical studies, including comparative studies, case series, and case reports, were eligible for inclusion. Methodological quality assessment was performed using a validated tool. Results: Twelve studies with a total of 42 patients were included in this review. There was a wide variation among these studies with regard to the time of initiation of MCA (1 day to 7 months), procedure time (13-320 minutes), and magnet characteristics (strength, size, and shape of the magnets used). The time to achieve anastomosis ranged from 1 to 12 days. Stricture at the anastomotic site was reported in almost all the patients, which required multiple endoscopic dilatations (median no. of dilatations/patient = 9.8). Stent placement for refractory stricture was required in 9 (21%) patients, and surgery for stricture was required in 6 (14%) patients. Long-term outcomes included esophageal dysmotility (n = 3) and recurrent pulmonary infections (n = 3) were reported in only four studies. Conclusion: As per the findings of this review, neonates with long-gap EA undergoing MCA would invariably require multiple sittings of endoscopic dilatations (median no. of dilatations/patient = 9.8). Also, there is a wide variation among the included studies in terms of the procedure of MCA. Future studies with a standardized procedure for achieving MCA are needed to determine additional outcomes in this fragile patient population.

Role of Procalcitonin as a Biomarker in Early Identification of Adverse Events Following Esophageal Atresia Surgery.

Introduction: Surgical complication following esophageal atresia repair is one of the several factors known to influence the final outcomes. Early identification of such complications may help in timely institution of therapeutic measures and translate into improved prognosis. Objective: The objective of this study was to evaluate the role of procalcitonin in early prediction of the adverse events after surgery in patients of esophageal atresia and the temporal relationship with clinical manifestations and other inflammatory biomarkers such as C-reactive protein (CRP). Materials and Methods: This was a prospective study on consecutive patients of esophageal atresia (n = 23). Serum procalcitonin and CRP levels were assessed at baseline (prior to surgery) and on postoperative days (POD) 1, 3, 5, 7, and 14. The trends in the biomarker values and temporal relationships of deviation in trend with the clinical and conventional laboratory parameters and patient outcomes were analyzed. Results: Baseline serum procalcitonin was elevated (n = 23; 1.7 ng/ml: min: 0.07 ng/ml-max: 24.36 ng/ml) in 18/23 (78.3%) patients. Procalcitonin nearly doubled on POD-1 (n = 22; 3.28 ng/ml: min: 0.64 ng/ml-max: 16.51 ng/ml) followed by a gradual decline. CRP was also elevated on POD-1 (three times the baseline) and depicted a delayed peak at POD-3. POD-1 procalcitonin and CRP levels correlated with survival. POD-1 procalcitonin cutoff at 3.28 ng/ml predicted mortality with a sensitivity and specificity of 100% and 57.9% (P = 0.05). Serum procalcitonin and CRP were higher for patients who sustained complications, so was the time required for hemodynamic stabilization. Procalcitonin (baseline and POD-5) and CRP (POD-3 and POD-5) values correlated with the clinical course after surgery. Baseline procalcitonin cutoff at 2.91 ng/ml predicted the possibility of a major complication with a sensitivity of 71.4% and a specificity of 93.3%. POD-5 procalcitonin cutoff at 1.38 ng/ml predicted the possibility of a major complication with a sensitivity of 83.3% and a specificity of 93.3%. Patients who sustained major complications depicted a change in serum procalcitonin trend 24-48 h ahead of clinical manifestation of an adverse event. Conclusions: Procalcitonin is a good indicator to identify the adverse events in neonates after surgery for esophageal atresia. The procalcitonin levels in patients who sustained a major complication depicted a reversal in trend 24-48 h of clinical manifestation. POD-1 procalcitonin correlated with survival while the baseline and POD-5 serum procalcitonin predicted the clinical course.

'Waterproofing layers' for urethrocutaneous fistula repair after hypospadias surgery: evidence synthesis with systematic review and meta-analysis.

OBJECTIVE: To summarize the available evidence and to quantitatively evaluate the global results of different waterproofing layers in substantiating the UCF repair. MATERIAL AND METHODS: After defining the study protocol, the review was conducted according to the PRISMA guidelines by a team comprising experts in hypospadiology, systematic reviews and meta-analysis, epidemiology, biostatistics and data science. Studies published from 2000 onwards, reporting on the results of UCF closure after hypospadias repair were searched for on PUBMED, Embase and Google Scholar. Study quality was assessed using Joanna Briggs Checklist (JBI) critical appraisal tool. The results with different techniques were compared with the two samples independent proportions test with the help of Microsoft Excel, MedCalc software and an online calculator. RESULTS: Seventy-three studies were shortlisted for the synthesis; the final analysis included 2886 patients (71 studies) with UCF repair failure in 539. A summary of various dimensions involved with the UCF repair has been generated including time gap after last surgery, stent-vs-no stent, supra-pubic catheterization, suture material, suturing technique, associated anomalies, complications, etc. The success rates associated with different techniques were calculated and compared: simple catheterization (100%), simple primary closure (73.2%), dartos (78.8%), double dartos flaps (81%), scrotal flaps (94.6%), tunica vaginalis (94.3%), PATIO repair (93.5%), biomaterials or dermal substitutes (92%), biocompatible adhesives (56.5%) and skin-based flaps (54.5%). Several techniques were identified as solitary publications and discussed. CONCLUSIONS: Tunica vaginalis and scrotal flaps offer the best results after UCF closure in the synthesis. However, it is not possible to label any technique as ideal or perfect. Almost all popular waterproofing layers have depicted absolute (100%) success sometimes. There are a vast number of other factors (patient's local anatomy, surgeon's expertise and technical perspectives) which influence the final outcome.

Unjumbling the TWIST score for testicular torsion: systematic review and meta-analysis.

OBJECTIVE: A systematic review and meta-analysis of the studies evaluating the utility of the Testicular Work-up for Ischemia and Suspected Torsion (TWIST) score in establishing or excluding the diagnosis of testicular torsion (TT) is herewith presented in an attempt to quantify the available evidence. METHODS: The study protocol was outlined in advance. The review has been conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA). The PubMed, PUBMED Central, PMC databases & Scopus followed by Google (Scholar & search engine) were systematically interrogated with the keywords TWIST score, testis and testicular torsion. Fourteen sets of data (n = 1940) from 13 studies were included; data from 7 studies (giving a detailed score-wise break-up) (n = 1285) were dis-integrated and re-integrated to tweak the cut-offs for low and high risk. RESULTS: For every 4 patients presenting to the Emergency Department (ED) with acute scrotum, one patient will eventually be diagnosed with TT. The mean TWIST score was higher in patients with testicular torsion (5.13 ± 1.53 vs 1.50 ± 1.40 for those without TT). TWIST score can be used to predict testicular torsion at cut-off of 5 with a sensitivity, specificity, PPV, NPV, and accuracy of 0.71 (0.66, 0.75; 95%CI), 0.97 (0.97, 0.98; 95%CI), 90.2%, 91.0%, and 90.9% respectively. While the slider for cut-off was shifted from 4 to 7, there was a rise in specificity and PPV of the test with a corresponding decline in sensitivity, NPV, and accuracy. The sensitivity witnessed a sharp decline from 0.86 (0.81-0.90; 95%CI) @ cut-off 4 to 0.18 (0.14-0.23; 95%CI) @ cut-off 7. The area under the SROC curve for cut-off 5 was more than that for cut-offs 4, 6 & 7. TWIST cut-off of 2 may be used to predict the absence of testicular torsion with a sensitivity, specificity, PPV, NPV, and accuracy of 0.76 (0.74, 0.78; 95%CI), 0.95 (0.93, 0.97; 95%CI), 97.9%, 56.5%, and 80.7%, respectively. While the cut-off is lowered from 3 to 0, there is a corresponding rise in the specificity and PPV, while the sensitivity, NPV, and accuracy are compromised. The sensitivity witnesses a sharp decline from 91 to 35%. The area under the SROC curve for cut-off 2 was more than that for cut-off @ 0, 1 or 3. The sum of sensitivity and specificity of TWIST scoring system to ascertain the diagnosis of TT is more than 1.5 for cut-off values 4 & 5 only. The sum of sensitivity and specificity of TWIST scoring system to confirm the absence of TT is more than 1.5 for cut-off values 3 & 2 only. CONCLUSION: TWIST is a relatively simple, flexible, and objective tool which may be swiftly administered even by the para-medical personnel in the ED. The overlapping clinical presentation of diseases originating from the same organ may prevent TWIST from absolutely establishing or refuting the diagnosis of TT in all the patients with acute scrotum. The proposed cut-offs are a trade-off between sensitivity and specificity. Yet, the TWIST scoring system is immensely helpful in the clinical decision-making process and saves time-lag associated with investigations in a significant majority of patients.

Looking Beyond Toxicities: Other Health-related Morbidities Noted in Childhood Solid Tumor Survivors.

Aim: In addition to the well-known toxicities of treatment, survivors of pediatric solid tumors can also develop other health-related conditions. They may either be an indirect consequence of therapy or could be unrelated to their prior history of malignancy. We aim to evaluate the nontoxicity related health conditions in survivors of pediatric solid tumors. Materials and Methods: The study included a cohort of hepatoblastoma (HB), Wilm's tumor (WT), and malignant germ cell tumors (MGCT) survivors registered at pediatric surgical-oncology clinic from 1994 to 2016. Follow-up was done according to standard protocols and children were evaluated at each visit for any health-related conditions. Results: Of the survivors, 318 survivors, comprising of 48, 81, and 189 survivors of HB, MGCT, and WT, respectively, were included in the analysis. We found 20.8% of patients with HB, 11.1% of patients with MGCT, and 16.4% of patients with WT to report nontoxicity-related health issues. A high prevalence of surgical conditions (3.4%), secondary malignancies (1.2%), gynecological conditions in girls (16.9%), tuberculosis (1.2%), gallstone disease (0.9%), pelvi-ureteral junction obstruction (0.9%), and neurological issues (0.9%) was noted. Two presumed survivors had died, one due to a late recurrence and the other due to a secondary malignancy. Conclusions: A high prevalence of medically or surgically manageable conditions makes it imperative to keep these children under follow-up to address any health-related conditions they may subsequently develop.

Fifty Most Influential Articles on Anorectal Malformations: A Tribute through Bibliometric Ranking.

Background: With the global assimilation of "publish and perish" culture into institutional academics, there has been an exponential rise in the publication numbers. There are ~2500 PUBMED entries related to "anorectal malformation (ARM)." The young clinician in his pursuit to translate experimental research to bedside often finds himself lost "in the midst of plenty." This bibliometric analysis has been conducted to codify the seminal work on ARM for future reference and pay tribute to the most impactful articles. Materials and Methods: Thomson Reuters Web of Science citation indexing database and research platform was used to retrieve the most cited articles in ARM using appropriate search strings. The characteristics (name of authors, the total number of authors, the title of publication, journal of publication, year of publication, etc.,) of the 50 top-cited articles were analyzed. Results: The analysis revealed that the Journal of Paediatric Surgery was leading the choice of journal for publication. While most of the publications originated from the United States of America, Alberto Pena was the most influential author. The most studied topic was on associated malformations, and the most common study design was cohort studies. Conclusion: The approach of citation analysis provided us an opportunity to retrieve the most influential articles on ARM. The trends in research in ARM have also been analyzed, spreading over five decades.

Evaluation of Interstitial Cells of Cajal-Like Cells at the Pelviureteric Junction in Children with Pelviureteric Junction Obstruction: A Prospective Study and Review of Literature.

Introduction: The etiopathogenesis of pelviureteric junction obstruction (PUJO) has been debated. Recently, the role of interstitial cells of Cajal-like cells (ICC-LC)has been studied and reported to be the cause of this functional obstruction. We studied the histopathology and ICC-LC density at PUJ and compared it with that of PUJ of the control group and distal ureteric margin of the study group. Methods: A prospective study was conducted which included PUJO patients in the study group and the renal tumor patients in the control group. Histopathological examination (muscle hypertrophy and fibrosis) and immunohistochemistry (ICC-LC density) were done. The muscle hypertrophy, fibrosis, and ICC-LC density at the PUJ in both the groups were compared. A similar comparison was performed between the findings at the PUJ and the distal margin in the study group. Results: The study and control groups included 37 PUJO patients and 13 Wilms tumor patients. The ICC-LC density at PUJ in the study group was significantly lower than that in the control group (P < 0.001) and that at the distal resected margin of the study group (P < 0.001). Significantly increased muscle hypertrophy (P < 0.001) and fibrosis (P = 0.002) were seen at PUJ in the study group compared to the control group. No significant association was noted between the ICC-LC density and muscle hypertrophy at PUJ and the distal resected margin in the study group. Conclusion: A significant decrease in the density of ICC-LC and increased fibrosis and muscle hypertrophy at PUJ in children with PUJO play a role in the etiopathogenesis of the disease.

Publication landscape of Posterior urethral valve- A scientometric study.

To assess the global research landscape in the field of Posterior urethral valve (PUV), a scientometric study was conducted to determine the quantity and quality of the research output and get insight into the key research areas and pattern of collaboration in the research community. METHODS: Primary data was acquired from the Scopus® database. Various parameters like the total research productivity, citations, citations per publication, citations per year, citations per publication per year, the scientific output of countries, institutions, authors, and journals were calculated. Key research areas were inferred by examining the top-cited articles (citations >150). Collaborative maps were generated by calculating co-authorship links amongst countries and authors using VOSviewer software. RESULTS: One thousand seven hundred fourteen results were found published from 1913 to 2019, receiving a total of 27,253 citations, authored by 5138 authors from 125 countries. The top three countries were the United States of America (USA), India, and the United Kingdom (UK). The leading institutes were the Children's Hospital Boston, USA (n = 48), All India Institute of Medical Sciences (AIIMS, Delhi), India (n = 38), and Great Ormond Street Hospital, UK (n- = 27). The journals which published the most articles on PUV were the Journal of Urology (n = 278), Journal of Pediatric Urology (n = 121), followed by Urology (n = 86). Leading authors were Glassberg, K. L and Bajpai M (n = 17, each), Canning DA & Kajbafzadeh, AM (n = 16, each), followed by Peters CA with 14 manuscripts. The top-cited documents (n = 10) received 2104 citations and were published between 1986 and 2010. The key areas of interest found were "long-term outcomes" (n = 4) and "fetal surgery" (n = 2). The most productive countries were also the most collaborative countries (the USA and UK). CONCLUSION: In the research landscape of PUV, the USA, Children's Hospital Boston, and Glassberg, K. L, along with Bajpai, M were the top contributors in terms of the country, organization, and authors, respectively. The "Journal of Urology" and "Pediatric Nephrology" stands out in terms of bibliometric parameters considered. The hotspot topics based on the number of citations received were "long-term outcomes" and "fetal surgery" in PUV. The most productive countries were also the most collaborative countries (the USA and UK).

Serum Matrix Metalloproteinase 7 as a Diagnostic and Prognostic Biomarker for Extrahepatic Biliary Atresia.

Background: Differentiation of neonatal cholestasis into neonatal hepatitis (NH) and extrahepatic biliary atresia (EHBA) is essential to formulate the treatment plan; promptness is indispensable for optimal outcomes. The clinical and nonoperative algorithms lack precision; the gold standard investigations (liver biopsy or per-operative cholangiogram) are invasive. There is a need for a noninvasive test which is both, sensitive and specific and has a high likelihood ratio. Aim: To study the (diagnostic) role of matrix metalloproteinase 7 (MMP-7) as a serum biomarker to differentiate between EHBA and NH and evaluate the prognostic significance in EHBA based on its correlation with liver histopathology and serological predictors of liver fibrosis - Aspartate-to-Platelet Ratio Index (APRI) and Fibrosis-4 (FIB-4). Materials and Methods: This was a prospective study conducted upon patients of neonatal cholestasis presenting with acholic stools (n = 46) with equal number of controls (n = 45) with no liver pathology. Observational parametric included disease-specific workup and serum MMP-7 levels (all participants); liver biopsyl and APRI-FIB-4 (EHBA). Results: (Diagnostic) Serum MMP-7 levels were significantly elevated in EHBA (n = 25; 28 ng/mL) as compared to those in NH (n = 21; 1.88 ng/mL) and normal infants (n = 45; 1.2 ng/mL) (P < 0.001 for both). Serum cutoff at 4.99 ng/mL differentiated EHBA-NH with a high sensitivity (96%), specificity (90.5%), and a negative predictive value (95%), with the number needed to misdiagnose being 23. (Prognostic) Inflammatory activity and fibrosis-stage on liver histopathology (METAVIR-and-Ishak scores) correlated with MMP-7 levels. APRI and FIB-4 scores also depicted a strong correlation with each other, age of the patient, and liver fibrosis. Conclusions: MMP-7 has a diagnostic value in differentiating EHBA from NH and may also be used as a prognostic biomarker in the follow-up of these patients. MMP-7 levels in controls may be used as a baseline for future studies.

Electronic Medical Record System-Based Teleconsultations in Pediatric Surgery: An Initiative from a Tertiary Care Public-Funded Hospital in North India to Alleviate the COVID-19 Imposed Hospital Visit Restrictions.

Background: There has been a dramatic effect of the coronavirus disease 2019 pandemic on the daily health-care services. The era of physical consultations is slowly being replaced with teleconsultation, and this current pandemic has tipped the scales further. This study highlights the preliminary experience in providing teleconsultation to pediatric surgical patients at a tertiary care hospital in north India. Materials and Methods: A retrospective analysis of the electronic medical record system records of the patients undergoing teleconsultation at the authors' department between the June 26, 2020 and the September 26, 2020 was performed. The data were categorized on the basis of the type of consultation (urgent, semi-urgent, or routine) and the type of intervention. A comparison with the data from the corresponding months of 2019 was also performed. Results: A total of 261 teleconsultations were conducted during the study period, with a success rate of 69% (181/261). Of these, 96% (171/178) were follow-up patients and 56% (99/178) presented with genitourinary complaints. After triaging, only 10% (18/178) of the patients required urgent medical/surgical attention. Conclusion: In the current as well as postpandemic phase, teleconsultation can act as a potent triaging tool and can help in better utilization of resources alongside helping in the maintenance of social distancing by decreasing the number of physical visits to the hospital.

Pediatric Neuroblastoma - Impact of Nutritional Status on Complications and Outcomes.

Aim: The aim is to study the impact of nutritional status on outcomes and treatment-related complications in patients of neuroblastoma. Materials and Methods: Anthropometry (height, weight, weight for height [in <5 years], mid-arm circumference [MAC] [in <5 years], and body mass index [BMI]), hemoglobin level, and serum albumin level were recorded. Results: Twenty-four neuroblastoma patients, 16 males and 8 females, with a mean age of 42.7 months were treated and followed up over a period of 0.03-170 months. Of 24 patients, 16 survived (complete response - 9, partial response - 2, no response - 2, progressive disease - 3). Height <2 standard deviation [SD] was seen in 12.5% (3/24). Mortality and complications were found in 66.7% (2/3) and 100% (3/3), respectively. Weight <2SD was seen in 37.5% (9/24). Mortality and complications were found in 33.3% (3/9) and 67% (6/9), respectively. Weight for height <2 SD was seen in 35% (7/20). Mortality and complications were found in 42.9% (3/7) and 71.4% (5/7), respectively. MAC <2 SD was seen in 15.8% (3/19). Mortality and complications were found in 66.7% (2/3) and 66.7% (2/3). BMI <2SD was seen in 29.2% (7/24). Mortality and complications were found in 42.8% (3/7) and 28.6% (2/7). Anemia was seen in 62.5% (15/24), of which nine survived. Complications were seen in 58.3% (14/24) (P = 0.001). Hypoproteinemia was seen in 20.8% (5/24). Mortality and complications were 40% (2/5) and 80% (4/5). Conclusion: Neuroblastoma has poor outcome in Indian children, and there does not appear to be a major role played by nutrition in determining the response to treatment and survival.

Comparison of Cisplatin Monotherapy and PLADO in the Management in Children with Standard-Risk Hepatoblastoma in a Resource-Challenged Nation.

Background: Recent SIOPEL studies have shown cisplatin monotherapy to be equally effective in management of Standard risk Hepatoblastoma (SRHB)as compared to PLADO. Aims and Objectives: To study the chemotherapy, response and outcomes in children with SRHB. Material and Methods: A retrospective study was conducted and all children with SRHB who presented to us from June 2007 to December 2017 were included. All patients with standard risk hepatoblastoma who had received at least 2 cycles of chemotherapy were included. Data regarding the demographics, PRETEXT stage, chemotherapy, response to chemotherapy and outcomes were recorded. Kaplan Meier survival analysis was performed to calculate 5 year overall survival (OS) and event free survival (EFS). Results: Thirty two children were included in the study. The disease was PRETEXT I in 5 (15.6%), II in 9 (28.1%) and 18 (56.2%). Nineteen children (59.4%) received Cisplatin monotherapy and of these 6 patients (all PREXT III) had poor response and the chemotherapy was upgraded to PLADO. The remaining 13 (40.6%) received upfront PLADO chemotherapy. Only 31 patients could be operated. Tumor recurred in 5 patients, 2 who had upfront PLADO and 3 patients had been upgraded to PLADO. The 5 year OS and EFS was 100% in the monotherapy group (n=13), 92% and 69% in the upfront PLADO group (n=13), and 62% and 22% in the upgraded to PLADO group (n=6). Patients with PRETEXT III disease in whom chemotherapy was upgraded to PLADO had significantly lower survival (p=0.036) compared to those who received upfront PLADO chemotherapy. Conclusion: Two thirds of patients with PRETEXT stage III who received cisplatin monotherapy showed poor response and were upgraded to PLADO chemotherapy. These patients had a significantly poorer outcome compared to the rest of the cohort. PRETEXT stage III standard-risk hepatoblastoma may benefit from PLADO chemotherapy instead of cisplatin monotherapy.

Delayed Presentation of Jejunal Atresia with Multiple Enteric Fistula: A Case Report and Review of Literature.

A 6-month-old boy presented with features of intestinal obstruction. Laparotomy revealed Type IIIa jejunal atresia. The proximal and distal bowel loops were in continuity through multiple fistulae between the adjoining bowel loops. To the best of our knowledge, it is the first report of congenital Type IIIa intestinal atresia surviving beyond the neonatal age without surgery.

Utility of Pentraxin-3 as a biomarker for diagnosis of acute appendicitis: a systematic review and meta-analysis.

PURPOSE: To systematically summarize all relevant data and to define the current evidence on the utility of Pentraxin-3 (PTX3) as a biomarker for acute appendicitis (AA) in children. METHODS: This review was conducted in accordance with the PRISMA guidelines. PubMed, Embase, Scopus, and Web of Science databases were systematically searched for studies comparing the levels of PTX3 in patients with AA vs healthy controls or non-specific abdominal pain (NSAP). Mean differences were calculated for all outcomes and the inverse variance method was used for weighted mean difference. The methodological quality of the included studies was assessed using the Downs and Black scale. RESULTS: Five comparative studies were included. Significantly elevated levels of PTX3 in cases with AA vs healthy controls (WMD: 9.56, 95% CI 7.24-11.88, p < 0.00001), and patients with AA vs NSAP (WMD: 8.05, 95% CI 6.81-9.29, p < 0.00001) were demonstrated. Similarly, in separate meta-analyses, the levels of PTX3 were significantly elevated in children with AA vs healthy controls (WMD: 11.18, 95% CI 10.03-12.34, p < 0.00001), and children with AA vs NSAP (WMD: 8.35, 95% CI 6.88-9.82, p < 0.00001). CONCLUSIONS: PTX3-levels are elevated in AA, but differentiation between perforated and non-perforated appendicitis demands other methods.