Management and Outcomes of Children with Malignant Germ Cell Tumor.

OBJECTIVES: To assess the clinico-pathological features, management and outcomes, amongst extracranial malignant germ cell tumors (MGCTs) in children treated primarily at a tertiary care center in a resource-challenged nation. METHODS: The prospectively maintained data for children below 14 y of age treated for extracranial MGCT from May 1994 to January 2023 was analyzed for patient characteristics, management, event-free survival (EFS) and overall survival (OS) and factors effecting survival. Events was defined as death, recurrence and progression. Multivariate logistic regression analysis was performed to identify the factors independently predicting unfavorable outcomes. RESULTS: One hundred and seventy-seven children (37% males) with a median (IQR) age at presentation of 30 mo (range 2-168 mo) were included. The cohort consisted of 87 (49%) extra-gonadal and 90 (51%) gonadal cases. Disease was metastatic at presentation in 48 (27%) with lungs being the most common site. Neoadjuvant chemotherapy (NACT) was given to 119 (67%) and finally 162/177 (92%) had undergone resection of the primary tumor. Endodermal sinus tumor (EST) was the commonest histological subtype in 141 children (73%). Twenty-two (12%) patients had died giving a 5-y OS of 84.7% (95% CI 78.3- 91.1). Recurrence occurred in 25 patients, and an additional 5 patients had progression giving a 5-y EFS of 69.9% (95% CI 62.5- 77.3). Stage III (p = 0.05), Stage IV (p = 0.006) and extra-gonadal site (p = 0.05) were significantly associated with poorer EFS. CONCLUSIONS: Children with MGCT have a favorable outcome with 5-y OS of 84.7% and EFS of 69.9%. Stage III and IV disease and extra-gonadal sites were independent predictors of a poor outcome.

Ewing sarcoma among children 5 years of age or younger: Is it a different disease?

INTRODUCTION: Children ≤5 years of age with Ewing's sarcoma (ES) possibly have a distinct disease biology, data on which are scarce. We evaluated clinical features, outcomes, and prognostic factors of ES among children with age ≤5 years. METHODS: Children with ES registered between 2003 and 2019 were included. Baseline clinical and treatment details were retrieved from medical records. Prognostic factors were identified using multivariable Cox regression. Clinical features and outcomes of children ≤5 years were compared with those greater than 5 years by chi-square and log-rank tests. Propensity score-matched (PSM) analysis was done to evaluate the impact of age on survival in the metastatic and localized subgroups. RESULTS: Out of the 859 patients, 86 (10%) were ≤5 years of age (median age 4 years, 60 males [69.8%]). The most common location was the extremities (37.2%), followed by thorax (27.9%) and head and neck (H&N) (22.1%); baseline metastases were seen in 25 patients (29.8%). The median event-free-survival (EFS) and overall survival (OS) were 25.6 and 68.7 months, respectively. Metastatic disease predicted inferior OS (hazard ratio [HR] = 2.54, p = .018) and EFS (HR = 2.47, p = .007], symptom duration ≤3 months predicted an inferior OS (HR = 2.17, p = .048). Compared to age greater than 5 years, younger children had more H&N and less pelvic primaries (p < .001) and lesser baseline metastases (p = .037). PSM analysis did not reveal any significant impact of age on OS in the metastatic (HR = 1.59, p = .29) or localized cohort (HR = 1.77, p = .09). CONCLUSIONS: Children with ES ≤5 years of age have a distinct favorable clinical presentation. However, age is not an independent prognostic factor for survival outcomes when adjusted for confounders.

A comparative study of smooth muscle cell characteristics and myofibroblasts in processus vaginalis of pediatric inguinal hernia, hydrocele and undescended testis.

BACKGROUND: Congenital inguinal hernia, hydrocele and undescended testis (UDT) are associated with patent processus vaginalis. The smooth muscles present in the processus vaginalis aid in the descent of the testis and undergo programmed cell death after testicular descent leading to obliteration. The persisting amount of smooth muscle in the processus vaginalis influences the clinical outcome as inguinal hernia, hydrocele or UDT. Therefore, a study was conducted to evaluate the processus vaginalis in these three conditions to observe the presence and phenotype of smooth muscle cells and the presence of myofibroblasts. MATERIALS AND METHODS: The processus vaginalis sacs in patients with inguinal hernia, hydrocele and UDT were examined using light microscopy for the presence and distribution of smooth muscle cells and immunohistochemical staining for vimentin, desmin, and α-smooth muscle actin (SMA) to identify the smooth muscle phenotype. Transmission electron microscopy was also performed in all the sacs to observe the presence of myofibroblasts. RESULTS: Seventy-eight specimens of processus vaginalis (from seventy-four patients), distributed as 47%, 27%, and 26% as inguinal hernia, hydrocele and UDT respectively, were included in the study. The sacs from inguinal hernia and hydrocele had significantly more presence of smooth muscles distributed as multiple smooth muscle bundles (p < 0.001). Desmin and SMA staining of smooth muscle cells was observed in significantly more sacs from hydrocele, followed by inguinal hernia and UDT (p < 0.001). The sacs from UDT had a significant presence of striated muscles (p = 0.028). The sacs from inguinal hernia had a significant presence of myofibroblasts, followed by hydrocele and UDT (p < 0.001) and this significantly correlated with the light microscopy and immunohistochemical features. The processus vaginalis sacs from four female patients did not differ statistically from the male inguinal hernia sacs in any of the above parameters. CONCLUSION: The processus vaginalis sacs in pediatric inguinal hernia, hydrocele and undescended testis differ in the presence, distribution and phenotype of smooth muscles and the presence of myofibroblasts. The clinical presentations in these entities reflect these differences.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS): challenges in diagnosis and management.

Megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS) is a rare, congenital functional intestinal obstruction, characterised by megacystis (bladder distention in the absence of mechanical obstruction), microcolon and intestinal hypoperistalsis (dysmotility).We are reporting a case of a female child with normal antenatal course who presented with recurrent episodes of abdominal distension since the second day of life and underwent negative exploratory laparotomy on multiple occasions. She also had urinary retention with a grossly distended bladder, requiring drainage by clean intermittent catheterisation. Surgical procedures for bowel decompression, including gastrostomy and ileostomy, were carried out without success. Genetic analysis revealed a mutation in the human smooth muscle (enteric) gamma-actin gene (ACTG2 gene), clinching the diagnosis of MMIHS. The patient was managed with parenteral nutrition and prokinetic medications and tolerated jejunostomy feeds for a brief period before she succumbed to the illness.Female neonates or infants presenting with abdominal distension and dilated urinary tract should be investigated for MMIHS early on. A timely diagnosis will enable the early involvement of a multidisciplinary team to provide the best options available for management.

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.

Study of Intrahepatic Biliary Architecture in Patients Operated for Extrahepatic Biliary Atresia using Magnetic Resonance Cholangiopancreatography.

Aim: The aim is to study intrahepatic biliary architecture in patients following Kasai's portoenterostomy for extrahepatic biliary atresia using magnetic resonance cholangiopancreatography (MRCP). Materials and Methods: It is a prospective observational study in a cohort of patients who have survived with a complete jaundice-free period for at least 1 year. MRCP was done to look for various intrahepatic architectural changes during their last visit. Findings were correlated with liver functions and growth and development. Results: Twenty-one patients were included in the study. The male-to-female sex ratio was 1.6:1, the median age at surgery was 75 days (18-140 days), and the median age at magnetic resonance imaging (MRI) was 4 years (18 months-18 years). More than 2 years of follow-up was in seven patients. Left lobe hypertrophy was observed in six patients, right lobe hypertrophy was in three patients, intrahepatic biliary tract dilatation was in five patients, and altered signal intensity between intrahepatic ducts was seen in five patients. In addition, hypointense foci in the spleen were seen in one patient. Twelve children had normal weight for their age, ten children had the normal height for their age, and gamma-glutamyl transferase was elevated in all children. Only four children had a history of fever and jaundice. Conclusions: The patients who survive after successful surgical intervention have near-normal liver gross architecture as on MRI/MRCP. Occasional findings of dilatation and lobar atrophy/hypertrophy are possible, especially in those with a history of cholangitis. The survivors also have acceptable growth parameters and normal liver functions.

Defining the Indications of PATIO Technique for Urethrocutaneous Fistula Repair.

Introduction: Despite the advancements in technique and technology, urethrocutaneous fistula (UCF) formation continues to be the most common complication after hypospadias repair. Objective: The objective of the current synthesis is to define the indications of PATIO technique for UCF repair. Materials and Methods: The review was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. PubMed, Scopus, Ovid, Embase, Web of Science, and Google Scholar were interrogated for studies presenting primary data upon UCF repair by the PATIO technique. Data analysis was performed on MedCalc and R software. Results: Eighteen studies were identified relevant to the current context: inversion of UCF tract has been described in 13 and ligation in 5. There were 2 duplications (abstract and manuscript). The overall success for PATIO is 88.2% (314/356). The success rate was variable between classic PATIO (inversion at 87.2%), ligation-inversion at 86.9%, and ligation alone at 88.9%. The success rate was not improvised upon by supplementing inversion of UCF tract with ligation (p = 0.957) or addition of a waterproofing layer (p = 0.622). PATIO has been used for single or multiple UCFs post hypospadias repair, genital piercing, and genitoplasty in cis- or transgender population for UCF up to 5 mm in size. The success rates were best for UCF <2 mm and worst for those approaching 5 mm. The results were, however, unaffected by the location of UCF along the penile shaft. Besides, the use of urethral catheter is optional and may be eliminated with shorter hospitalization. Conclusions: PATIO repair may be considered for repair of UCFs (a) with diverse etiologies, (b) located anywhere along the penile shaft included coronal UCF, (c) preferably <4 mm in size, (d) single or multiple in number; multiple PATIOs may be done in the same setting, (e) in patients unwilling for prolonged hospitalization, (f) in patients unwilling for a urethral catheter, and (g) in hypospadias cripples wherein mobilization of distant tissues such as tunica vaginalis flap or a buccal mucosal graft may be required for supplementing the UCF repair.

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.

Traumatic Isolated Right Lobe Devascularization of the Liver: An Unusual Case.

Isolated liver lobe devascularization is a very rare case, with conflicting literature regarding management. We describe a very unusual case of traumatic isolated right lobe devascularization of the liver with its attendant management challenges. An eight-year-old boy with a history of road traffic accidents presented with abdominal pain. Although the child was hemodynamically stable on presentation, extended focused assessment with sonography in trauma was positive. Contrast-enhanced computed tomography (CECT) scan of the torso revealed a nonenhancing right lobe of the liver involving segments 5-8 and the gross hemoperitoneum. Nonoperative management was tried. There were persistent high-grade fever spikes, for which prophylactic antibiotics were started, but the fever workup was negative. Abdominal drains were inserted to drain fluid and relieve distress. Output was noted to be bilious on day 21 of injury. Diagnostic laparoscopy on day 22 revealed hypertrophied left lobe of the liver with an absent (autolyzed) right lobe. The subsequent ward course was uneventful, and the child was discharged in stable condition. Thus, the indication of surgery in such cases is clinical deterioration, not radiological findings. Management should be in a dedicated trauma center with immediate operating room availability.

Preoperative Triple-Phase Three-Dimensional-Multi-Detector Computed Tomography Imaging of the Hepatic Vascular Tree: An Accurate Road Map Prior to Tumor Resection in Hepatoblastoma.

Objective: To evaluate the hepatic vasculature/tumor relations in hepatoblastoma patients with three-dimensional (3D) reformatted images after triple-phase multi-detector computed tomography (MDCT) and to compare these with the surgical findings to judge the accuracy of this investigation. Materials and Methods: The study was carried out in hepatoblastoma patients after appropriate neo-adjuvant chemotherapy, prior to resection. Images were postprocessed at a dedicated workstation for multi-planar reformations, maximum intensity projection, curved planar reformations, and volume-rendered technique reconstructions. The reporting was done as per a specific protocol by both the radiologist and surgeon (per-operative findings) and the accuracy of MDCT ascertained as per concordance between the surgical and imaging findings. Results: Fourteen children (13 boys, 1 girl) underwent surgery. Clinically, relevant information regarding vascular, tumor involvement, and interface with vessels was provided by the study in all cases. Although all tumors were deemed resectable on preoperative imaging, one procedure was abandoned due to an unanticipated portal cavernoma. While a few anatomical variations were unexpectedly encountered during surgery, there was good concordance overall between findings on imaging and surgical exploration. Conclusions: MDCT with 3D reformatting provides accurate virtual representations of the hepatic tumor. This allows simulation of surgical resection with decreased risk of vascular injury and postoperative liver failure.

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.

Bibliometric analysis of pediatric glaucoma.

Purpose: The research activity in pediatric glaucoma (PG) was qualitatively and quantitatively evaluated using a scientometric approach. Methods: The "Web of Science" database was accessed for primary bibliometric data regarding PG using search terms "pediatric glaucoma," "paediatric glaucoma," "congenital glaucoma," and "childhood glaucoma." The data was analyzed for total research productivity, citations, and scientific output in terms of journals, countries, institutions, and authors. The results were further characterized for coauthorship links and visualized by VOS viewer software. Also, the top 25 cited articles were reviewed with the above bibliometric characteristics. Results: One thousand two hundred and sixty-nine items were obtained from our search query from 1955 to 2022; these received 15,485 citations, originated from 78 countries. The top-3 contributing countries were the United States of America (n = 369), India (n = 134), and China (n = 127). LV Prasad Eye Institute (n = 58), Duke University (n = 44), and King Khalid Eye Specialist Hospital (n = 42) were the top-3 productive institutes. The top-3 prolific authors were Mandal AK (n = 53), Freedman, SF (n = 36), and Sarfarazi, M (n = 33). Journal wise, "Investigative Ophthalmology" (n = 187), "Journal of Glaucoma" (n = 92), and "Journal of AAPOS" (n = 68) were the journals in which the most articles were published. The top-25 cited documents received 3564 citations and were published between 1977 and 2016. The key areas of interest were basic sciences (genetics of childhood glaucoma) and surgical management. Conclusion: United States of America, LVPEI, Mandal AK, and "Investigative Ophthalmology" were the top rankers as far as the productivity and publications related to PG are concerned. Articles on molecular genetics in PG have received interest among the ophthalmology community.

The comparison of interrupted and continuous suturing technique in Snodgrass urethroplasty in patients with primary hypospadias: A systematic review and meta-analysis.

Background: Hypospadias is among the common congenital anomalies in boys. Snodgrass urethroplasty is one of the most popular techniques for correcting distal and mid hypospadias. Although there is consensus among pediatric surgeons on using absorbable sutures for urethroplasty, there are no established guidelines about the suturing techniques (interrupted suturing [IS] or continuous suturing [CS]) for neourethra creation in Snodgrass urethroplasty. This analysis aims to compare the reported outcomes of both the urethroplasty suturing techniques. Materials and Methods: This systematic review and meta-analysis was conducted as per the preferred reporting items for systematic review and meta-analyses guidelines. A systematic, detailed search was carried out by the authors in the electronic databases - MEDLINE, PubMed Central, Scopus, Google Scholar, and Clinical Trial Registry. Studies were selected and compared based on primary outcomes - development of urethrocutaneous fistula (UCF), meatal stenosis, and secondary outcomes - wound infection, urethral stricture, and operative time. Statistical analysis was performed using a fixed-effect model, pooled risk ratio, and I2 heterogeneity. Results: Five randomized studies with a total of 521 patients met our inclusion criteria. Pooled analysis for total complications, UCF, meatal stenosis, and wound infection showed no significant difference between the CS and IS groups. Subgroup analysis of patients with the use of polyglactin sutures showed a decrease in total complications and UCF in the IS group. Conclusion: There is no difference in total complication rates among the CS and the IS group with the use of absorbable sutures in Snodgrass urethroplasty; however, there is a decrease in the incidence of total complications and UCF in the IS group when polyglactin was preferred over polydioxanone suture for urethroplasty.

Postoperative necrotising enterocolitis following incarcerated inguinal hernia repair.

The occurrence of postoperative necrotising enterocolitis (NEC) is rare. In comparison with the commonly known inciting factors for the development of NEC, surgical stress as a cause of NEC is relatively uncommon. We report a case of incarcerated inguinal hernia in a preterm infant who clinically deteriorated in the postoperative period because of NEC following herniotomy. The patient's clinical, biochemical and radiological features were suggestive of NEC, and the patient responded to the appropriate treatment based on the diagnosis. To conclude, incarcerated inguinal hernia should always be considered a causative factor for postoperative NEC.

'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.

Pediatric Appendicitis Score or Ultrasonography? In Search of a Better Diagnostic Tool in Indian Children with Lower Abdominal Pain.

OBJECTIVES: To prospectively assess the performance of pediatric appendicitis score (PAS) in diagnosing acute appendicitis in the children with lower abdominal pain and correlated with ultrasound findings; and to assess the impact of the PAS on clinical outcome and its efficacy in differentiating between complicated and uncomplicated appendicitis. METHODS: A prospective study was done which included cases of lower abdominal pain. Appendectomy was done for PAS ≥ 6, and diagnosis was confirmed on histopathology. A receiver operator characteristic (ROC) curve was created to assess the PAS performance. The sensitivity, specificity, and accuracy of ultrasonography in diagnosing appendicitis were assessed, and analysis of agreement between ultrasonography and PAS score was done by kappa statistics. RESULTS: Of 260 cases with lower abdominal pain, 205 were suspected of having appendicitis. One hundred fifty-nine had PAS ≥ 6. There were 2/159 (1.26%) cases of negative appendectomies and 2/46 (4.34%) cases of missed appendicitis. The mean PAS was significantly higher in patients with appendicitis than in those without appendicitis. The area under the ROC curve was 0.9925. Sensitivity, specificity, and positive and negative predictive value of PAS were 98.74%, 95.65%, 95.7% and 95.65%, respectively. Complicated appendicitis had significantly more PAS, fever, and cough tenderness than uncomplicated appendicitis. The sensitivity and specificity of ultrasonography were 86.79% and 17.39%, respectively. Agreement between ultrasonography-proven appendicitis and PAS-dependent appendicitis was weak. CONCLUSION: PAS has high efficacy in diagnosing acute appendicitis. Clinical outcome was more favorable with the use of PAS. Ultrasonography should be used judiciously and in combination with clinical judgment.

Response to Neoadjuvant Chemotherapy and Outcomes in Children With Wilms Tumor With Caval Thrombus: A Single Center Experience.

AIM: The aim of our study is to present our experience in the management and outcome of Wilms tumor with intracaval thrombus. MATERIALS AND METHODS: All children with Wilms tumor with intracaval thrombus who presented to us from July 2000 to December 2017 were reviewed retrospectively. We evaluated the tumor stage, management, and outcomes in these patients. RESULTS: Thirty-four patients were included in the study. The median age of presentation was 48 months (11 to 84 mo). Preoperative chemotherapy was given in 32 (94%), with a median duration of 8 weeks. Intracaval thrombus completely resolved in 9 (26%) children after neoadjuvant chemotherapy. Surgical intervention for residual inferior vena cava (IVC) thrombus was performed in 32 patients. The median follow-up was 30 months (5 to 150 mo). At the last follow-up, 24 patients (70%) were alive and disease free. The 5-year overall survival (OS) and event-free survival were 67% (95% confidence interval, 50% to 84%) and 59% (95% confidence interval, 42% to 76%). The OS in children with nonmetastatic disease (94%) was significantly higher than those with metastases (29%; P <0.01). The OS in children with complete resolution of IVC thrombus (100%) was significantly higher than those with persistent thrombus (48%; P =0.025). Analysis of survival outcomes in children with nonmetastatic disease (stage III) revealed no significant difference on comparison with cohort with stage III disease with absence of IVC thrombus. The P -value was 0.224 and 0.53 for 5-year OS and event-free survival, respectively. CONCLUSION: The management of Wilms tumor can be complicated by the presence of caval thrombus. Patients with metastasis have a significantly poor outcome. Patients in whom, there is complete resolution of intracaval thrombus on neoadjuvant chemotherapy have a significantly higher OS.