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.

Exosomal long non-coding RNA MALAT1: a candidate of liquid biopsy in monitoring of Wilms' tumor.

PURPOSE: Wilms' tumor (WT) is a rare kidney cancer that primarily affects children. Exosomes are extracellular vesicles that cargo nucleic acids, proteins,etc. for cellular communication. Long non-coding RNAs (lncRNAs) have utility as biomarkers for cancer diagnosis, prognosis, and disease monitoring. We hypothesize that expression of lncRNA, metastasis-associated lung adenocarcinoma transcript-1(MALAT1), is dysregulated and possibly trafficked within exosomes to influence the tissue microenvironment for metastasis and recurrence of WT. METHODS: We investigated the expression of MALAT1 in thirty WT samples by qPCR. Exosomes were isolated using a precipitated and affinity-binding-based kit, and characterized using TEM, NTA, and DLS. RESULTS: Mean number of exosomes was 9.01×108/mL in primary culture, 1.64×108/mL in urine, and 4.65×108/plasma:400µl. Average yield of total RNA was 1.28µg (primary-culture supernatant:1ml), 1.47µg (Urine:1ml), 1.65µg (Plasma:400 µL). We quantified MALAT1 in exosomes derived from these sources in patients of WT. Expression of MALAT1 was significantly downregulated (p=0.008) in WT samples. CONCLUSION: This is the first study that demonstrated the presence of lncRNA MALAT1 in various invasive and non-invasive samples of patients with WT(primary tissue culture, urine, and plasma samples).

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.

Diffuse Esophageal Leiomyomatosis and the Surgical Challenges Encountered.

Diffuse esophageal leiomyomatosis (DEL) is a rare condition characterized by the growth of multiple benign smooth muscle tumors (leiomyomas) in the esophagus. These tumors can cause obstruction and functional impairment of the esophagus, leading to symptoms such as difficulty swallowing, chest pain, and weight loss. The disease can present with leiomyomas or glomerular nephropathy in other body parts, hearing defects, astigmatism, or myopia (Alport's syndrome). However, isolated cases are also reported in the literature. Surgical management of DEL presents several challenges due to the location and extent of the tumors. Herein, we describe a girl afflicted with DEL and required multiple surgeries for management, highlighting the unique challenges faced by the surgical team in restoring esophageal function and improving the patient's quality of life.

An Innovative Technique to Prevent Air Leaks from Cervical Esophagostomy during Face Mask Ventilation.

During face mask ventilation, invariably, some air enters the esophagus rather than the airways; this results in gastric insufflation while the esophagus is intact or loss of tidal volume in patients with a cervical esophagostomy (Cx-esophagostomy). Objective: The objective of the study was to report the indigenous use of an electrocardiography (ECG) electrode in successfully occluding the Cx-esophagostomy to prevent inadvertent loss of tidal volume. Materials and Methods: Twenty-nine observations were recorded on 27 patients (mean age: 10.9 months; male:female = 2.6:1). The oral cavity, pharynx, and Cx-esophagostomy were cleared of saliva. Peristomal area was cleaned with dry gauze and dried with alcohol. Latex-free, 50 mm, ECG electrode was pasted symmetrically over the Cx-esophagostomy. The efficacy of the electrode was assessed using a five-point algorithm: (i) collapse of reservoir bag, (ii) chest expansion, (iii) ballooning of electrode, (iv) cotton-wisp test, and (v) change in compliance with manual compression of the electrode. Subgroup analysis was conducted for laterality and health of Cx-esophagostomy. Results: Study cohort: Cx-esophagostomy was located on the left in 58.26% (n = 17/29) patients. The health of Cx-esophagostomies was graded from I to IV (n = 9, 16, 3, and 1, respectively). The collapse of the reservoir bag and chest rise indicated successful ventilation. Ballooning of the electrode with each breath was observed in 79% (23/29) patients. Partial separation of the electrode from the skin (exaggerated with each breath) was observed in 10.3% (3/29) patients (Group I: n = 1, Group III: n = 1, and Group IV: n = 1). This was related to poor local preparation or pooling of saliva (n = 1; Group I: resolved with procedural re-application of a fresh electrode) and underlying skin condition (n = 2; Groups III and IV: resolved only temporarily with re-application). The cotton-wisp test was positive in two additional patients (1 each in Groups II and III) and was related to the pooling of saliva. No change in compliance was observed with manual compression of the electrode. No difference in success was observed between left and right Cx-esophagostomies. Overall success with this technique was 82.8% with a potential for 100% in those with peristomal health graded I-III. Conclusions: The ECG electrode is a technically simple, cost-effective, and widely available device, instrumental in addressing the problem of air leak through Cx-esophagostomy during face mask ventilation before intubation.

Is Virtual Reality Effective in Decreasing Pain during Clinical Procedures among Children: A Systematic Review and Meta-analysis.

Background: Virtual reality (VR) refers to a computer-generated simulation or environment that can be experienced and interacted with by a person through special electronic equipment, such as a headset with a screen and motion-tracking sensors. VR has been used as a distraction technique to alleviate pain and discomfort during medical procedures or as an alternative to traditional pain medication. This article explores VR's effectiveness in reducing pain in pediatric patients undergoing painful clinical procedures. Methodology: This systematic review and meta-analysis was performed as per the preferred reporting item for systematic review and meta-analysis guidelines. A systematic literature search was conducted for all articles published till December 2022 in four different databases: PubMed, Scopus, Embase, and LILACS, which yielded 6886 studies. Studies showing the effect of VR on pediatric patients undergoing clinical procedures were included in the study. Risk of bias (ROB) 2.0 was used to assess the ROB. Outcome data were pooled using a random-effects model and reported as standardized mean difference (SMD) with a 95% confidence interval (CI). The meta-analysis was performed on RevMan5.4. Subgroup analysis was done for the different clinical procedures. Results: A total of 2477 participants from 34 studies were included in the meta-analysis. Children in whom VR was used had a significantly lower pain score as compared to the control group with an SMD of 0.87 (CI 95% -1.20, -0.53; (P < 0.00001). Subgroup analysis based on the type of intervention did not reveal any significant difference (P = 0.19). Heterogeneity for overall pain using a random effect model with I 2 was significant (P < 0.00001). Conclusion: This systematic review suggests that the use of VR distraction in children undergoing painful procedures is associated with significantly lower pain scores. Hence, VR can be a promising intervention for reducing pain experienced by pediatric patients during medical procedures.

Genetic Markers of Spina Bifida in an Indian Cohort.

Objective: To identify the genetic markers of spina bifida through a systematic survey of the exome in an Indian cohort. Materials and Methods: Three consecutive patients (P1: 1 year, male; P2: 2.8 years, male; and P3: 10 years, female) with spina bifida (lumbosacral meningomyelocele) underwent whole-exome sequencing (libraries: SureSelect Human All Exon V8; sequencing: 2 * 150 bp paired-end run, 100×) with NovaSeq 6000. Data analysis was performed using SMART-One™ (secondary analysis) and SMARTer™ (tertiary analysis) for automated quality check, alignment (GRCh38/hg38), variant calling, annotation (ClinVar, OMIM, avsnp150, 1000 Genomes v5b, ExAC v0.3, gnomAD v4.0, and esp6500vi2all v0.0.25), v0.0.25), interpretation. The pathogenic and likely pathogenic (ClinVar/ InterVar), non-synonymous, exonic markers (read depth ≥ 5) were matched with the Familial Neural Tube Defects (Version 1.10) panel (FNTD panel). Results: Pathogenic variants overlapping with the FNTD panel were MTRR, CC2D2A, and ZIC2 in P1 and P2, TGIF1 in P1 only, and none in P3. Novel pathogenic/likely pathogenic variants common to all three patients were PRUNE1, PKD1, PDZD2, and DAB2 in the homozygous state as well as in the heterozygous state, PLK1 and NLGN2. The possible role of such markers in etiopathogenesis was explored through a literatur search. Conclusions: The genetic landscape of the spina bifida in an Indian cohort is diverse compared to that reported from other parts of the world. A comprehensive catalog of single-nucleotide variants in the etiopathogenesis of the spina bifida on a background of the Familial Neural Tube Defects Panel has been generated.

Dear Laparoscopic Surgeons: Caution with the Use of Glutaraldehyde!!!

Background: The occupational hazards of glutaraldehyde are well known; the possibility of harm to the patients has been highlighted in the form of isolated reports only. Objective: To synthesize evidence for contact mucosal injury or injury due to intraperitoneal instillation of glutaraldehyde following its use during laparoendoscopy. Materials and Methods: The current review is Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) compliant. PubMed, PubMed Central, and Google Scholar were interrogated for animal and human studies upon the harmful effects of glutaraldehyde during laparoscopy and proctosigmoido-colonoscopy. Results: Thirty-five studies substratified into animal experiments (n = 2), glutaraldehyde-induced colitis (G-iC) postendoscopy (n = 30), and laparoscopy (n = 3) were included. Rats suffered mucosal injury following colonic injection of glutaraldehyde which was time- and concentration-dependent quantum and developed bloody diarrhoea. Omental and renal injury was observed due to glutaraldehyde instillation during simulation of intra-peritoneal insufflation in rats; the serum leucocytes, CRP and creatinine were also elevated. G-iC following colonoscopy was related to contact mucosal injury due to failure (human or machinery) to rinse the chemical off the instrument surface or as a case of mistaken identity (glutaraldehyde was mistaken for saline or another reagent). The incubation period was <24 h in 90% of patients; clinical presentation was related to the initiation of intense inflammation. Intraperitoneal instillation during laparoscopy also leads to intense inflammation. One patient was asymptomatic, the second required laparotomy and peritoneal irrigation for 48 h, while the third case underwent multiple laparotomies for enteric cutaneous and enteroenteric fistulae, adhesions, and multiple intra-abdominal abscesses. Hydronephrosis consequent to retroperitoneal fibrosis has been reported. Conclusions: The use of glutaraldehyde in the patient care facilities should be discontinued; when unavoidable, the standard processes of disinfection and rinsing must be stringently followed.

Advancing Precision in Penile Length Measurement: Evidence-based Synthesis of Stretched Penile Length INdicator Technique (SPLINT).

Purpose: The purpose of this study was to synthesize evidence and propose a technique for estimation of stretched penile length (SPL) applicable to children. Materials and Methods: This review has been conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. PubMed, Embase, and Scopus databases were queried on penile length (PL) measurement techniques in humans published from 1990 onward. The devices and the techniques used for PL measurement, state of the penis, the habitus of the participants, proximal and distal landmarks for measurement, handling the foreskin and pubic pad of fat, optimal stretching of the penis, and other factors (including the environment) which should be accounted for in the technique were identified from the study cohort (90895 participants across 145 included studies). Results: PL has been represented through flaccid (33.79%), stretched (81.3%), and erect (12.41%) PLs as well as the greatest corporeal length (1.4%). Following devices have been used to measure the PL in the study cohort: rulers [54.68%], calipers [9.7%], measuring tapes [14.5%], coloured measuring strips [2.06%], spatulas/tongue depressors [11.03%], FitKit, syringe [1.4%], cotton swab (and ruler), titan cylinders, slide gauge and ultrasonography [1.37%]. The factors relevant to SPL measurement have been incorporated into the proposed SPL INdicator Technique (SPLINT) which is essentially a holistic extension of the "Conventional PL Measurement" technique. Conclusions: There is a wide range of heterogeneity in the technique for estimation of PL across the study cohort; the underlying factors have been identified along with the respective variables, and the SPLINT for SPL has been described.

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.

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.

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.

'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.

Comparison of High Spermatic Vessel Ligation and Low Spermatic Vessel Ligation in an Undescended Model of Rat Testis.

Aims: The aim of this study was to compare the immediate and long-term outcomes after high spermatic vessel ligation (HSVL) and low spermatic vessel ligation (LSVL) in a high undescended testis (UDT) model in rats. Materials and Methods: A prospective randomized controlled study was conducted on 24 male Wistar rats. The rats were randomly divided into three groups. Group A underwent a sham laparotomy and acted as the control. Group B underwent HSVL of both testicular vessels. Group C underwent LSVL of both testicular vessels. Each group was again subdivided into two subgroups. One sub-group underwent blood collection and testicular biopsy of both testes 24 h after the procedure to demonstrate immediate changes. Other subgroups underwent blood sample collection and testicular biopsy of both testes on day 50 following the procedure for hormonal changes and long-term changes. Results: All the testes in HSVL showed atrophy (100%) in the long term, whereas LSVL showed atrophy in 12.5% of testes, even though both groups showed adequate neovascularization. Testes in HSVL showed poor bleeding on incision at both 24 h and day 50. On histology, 75% of testes in HSVL showed complete necrosis, and 50% in LSVL showed partial necrosis at 24 h. On day 50, all the testes in HSVL (100%) showed complete necrosis with dystrophic calcification, whereas all the testes in LSVL showed normal histology with good maturation of seminiferous tubules. There was no significant difference in testosterone levels between both groups. Conclusions: Both immediate and long-term changes following LSVL showed an increase in blood flow to the testis after ligation through collaterals and reverses early ischemic changes to the testis. Given the higher testicular atrophic rate after HSVL, LSVL or at least low ligation can be preferred for the management of high intra-abdominal UDT.

Bibliometric Analysis and Visualization of the Extrahepatic Portal Venous Obstruction Publication Landscape.

Introduction: A scientometric analysis was conducted to characterize the global research publications in extrahepatic portal venous obstruction (EHPVO), and state-of-the-art visualization graphics were generated to provide insight into specific bibliometric variables. Materials and Methods: The Web of Science database was accessed for research productivity and bibliometric variables of countries, institutions, authors, journals, and content analysis of top-20 cited documents were performed. Collaborative networks and co-occurrence of keywords map were generated using VOSviewer software. Results: Two hundred and sixteen records were retrieved with an annual growth rate of 2.53%. India is the leading country in productivity (n = 4339), followed by the USA and China. Post Graduate Institute of Medical Education and Research, Chandigarh, was the top productive institute. Sarin SK was the most prolific author, having the highest citations received and h-index. The hotspot topics were "portal hypertension," "cirrhosis," "children," "biliopathy/cholangiopathy," "liver fibrosis," and "liver transplantation" as per keyword co-occurrence networking. J Gastroenterol Hepatol had the most publications of EHPVO research as well the h-index. Regarding collaborative network mapping, the USA and Primignani M were the significant nodes among country and author, respectively. Conclusion: EHPVO research publication volume is low but is gradually progressing with dominant contributions from Indian institutes and authors. Most highly cited articles are of low level of evidence, and multi-institutional collaborative research can be the way forward.

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.

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.

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.

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.

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.