Journal of Pediatric Surgery Open最新文献

{"title":"Robot-assisted thoracoscopic versus conventional thoracoscopic plication for diaphragmatic eventration in children: Comparison of mid-term outcomes","authors":"Mengxin Zhang ,&nbsp;Jingfeng Tang ,&nbsp;Yuan Liu ,&nbsp;Zhiqing Cao ,&nbsp;Kangtai Wang ,&nbsp;Guoqing Cao ,&nbsp;Xi Zhang ,&nbsp;Shao-tao Tang","doi":"10.1016/j.yjpso.2024.100157","DOIUrl":"10.1016/j.yjpso.2024.100157","url":null,"abstract":"<div><h3>Objective</h3><p>Minimally invasive surgery has been widely used for diaphragmatic eventration (DE). We aimed to compare the mid-term outcomes of robot-assisted thoracoscopic (RTP) and conventional thoracoscopic plication (CTP) for DE and to discuss the technical advantages of RTP.</p></div><div><h3>Methods</h3><p>From January 2015 to July 2022, the clinical outcomes of 30 RTP patients and 35 CTP patients were retrospectively analyzed. The baseline data, surgical outcomes and surgical technique will be compared in detail.</p></div><div><h3>Results</h3><p>The baseline data of two groups were comparable. No intraoperative conversion or complications occurred. The total operation time and intraoperative blood loss were similar between two groups, but RTP group had a significantly shorter diaphragmatic plication time compared to CTP group (25.77±3.55 min vs 44.49±4.43 min, <em>p</em> &lt; 0.001). The total surgical cost of RTP group was higher than that of CTP group (48,593.06±2752.33 RMB vs 25,181.23±5681.51 RMB, <em>p</em> &lt; 0.001). No significant differences were found in postoperative hospital stay, diclofenac suppository dosage, unplanned readmission rate within 30 days postoperatively, recurrence rate, or postoperative complications within 1 year postoperatively between RTP and CTP groups. Sixty patients had preoperative symptoms resolved or significantly improved with normal diaphragmatic position. However, two patients in CTP group encountered recurrence, and one patient in CTP group and two patients in RTP group had a diaphragm descent in only one intercostal space.</p></div><div><h3>Conclusion</h3><p>RTP is a feasible and effective minimally invasive option for the treatment of DE, with mid-term outcomes comparable to CTP, which has a great advantage in terms of easier suturing and free knotting. DE can serve as an ideal disease type to train robots for thoracoscopic surgery by young doctors.</p></div><div><h3>Level of evidence</h3><p>Level III.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100157"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S294971162400042X/pdfft?md5=dd9053abb546cc2e32d9b859710cde44&pid=1-s2.0-S294971162400042X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141630718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pedi-Bots: Innovations and progress in robotic pediatric general surgery","authors":"Krysta M Sutyak ,&nbsp;KuoJen Tsao","doi":"10.1016/j.yjpso.2024.100156","DOIUrl":"10.1016/j.yjpso.2024.100156","url":null,"abstract":"<div><p>Robotic Assisted Surgical Systems for abdominal surgery began human trials in adults in the 1990s [<span>1</span>,<span>2</span>]. The first commercially available system was approved for use in Europe and the United States by 2000 [<span>2</span>,<span>3</span>]. Almost 25 years later, robotic surgery is commonplace in most areas of general surgery in adults and is utilized nationally and internationally throughout most other surgical specialties. The first robotic pediatric surgery, a Nissan fundoplication, was completed in 2000 [<span>4</span>]. In relatively similar time periods where robotic surgery has become widespread in the adult population, its use has remained limited in pediatric patients. This is most often attributed to limitations secondary to the patient's size relative to the size of the robot and instruments[<span>5</span>,<span>6</span>]. In this article, we aim to review the robotic technology currently available in pediatrics, the advantages of robotic surgery, the use and safety of robotics in pediatric general surgery, and the challenges or limitations of its use, highlighting how surgeons and centers are utilizing this technology and implementing robotic practices despite these obstacles.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100156"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000418/pdfft?md5=b5e2b12710654b14d599da7858503d4d&pid=1-s2.0-S2949711624000418-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141408300","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Emergency Department Thoracotomy: A National Trauma Databank analysis","authors":"Taylor Glassman ,&nbsp;Sathyaprasad Burjonrappa","doi":"10.1016/j.yjpso.2024.100154","DOIUrl":"https://doi.org/10.1016/j.yjpso.2024.100154","url":null,"abstract":"<div><h3>Introduction</h3><p>Emergency Department Thoracotomy (EDT) warrants further investigation in the pediatric population due to concerns about its futility. Our study evaluates how patient demographics, injury characteristics, and physiologic status influence pediatric EDT outcomes in blunt and penetrating trauma.</p></div><div><h3>Methods</h3><p>This retrospective study analyzed patients ≤19 years who received EDT within 1 hour of arrival to the ED from the National Trauma Data Bank. Cases were stratified by blunt and penetrating trauma. Descriptive statistics and univariate analysis were used to identify predictors of survival post-EDT.</p></div><div><h3>Results</h3><p>Of the 1,075 pediatric patients who underwent Emergency Department Thoracotomy (EDT), 79.6 % experienced penetrating trauma and 20.4 % blunt trauma, with survival rates at 25.9 % and 9.1 %, respectively. Survivors of penetrating trauma typically presented with more stable vital signs, such as pulse rate, oximetry, and Glasgow Coma Scale (GCS) scores, and lower Injury Severity Scores (ISS), all correlating with survival (p &lt; 0.05). Survivors of blunt trauma were distinguished by higher respiratory rates and temperatures (p &lt; 0.05). Risk factor analysis indicated that age-adjusted bradycardia, age-adjusted hypotension, and pre-hospital cardiac arrest were significant predictors of mortality across trauma types (penetrating trauma: OR = 18.7, 6.2, and 20.7; blunt trauma: OR = 12, 2.69, and 6.4, all p &lt; 0.05). Notably, pre-hospital respiratory support was associated with a reduced risk of mortality for both types of trauma (OR = 0.32 for penetrating, and OR = 0.23 for blunt, p &lt; 0.05).</p></div><div><h3>Conclusions</h3><p>The study affirms the significance of pre-hospital care, showing that stable vitals and respiratory support enhance pediatric thoracotomy outcomes. Ongoing research into pediatric-specific trauma protocols are needed.</p></div><div><h3>Type of Study</h3><p>Retrospective cohort</p></div><div><h3>Level of Evidence</h3><p>III</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100154"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S294971162400039X/pdfft?md5=e6da275be2ccb8802f406569f3485361&pid=1-s2.0-S294971162400039X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141594142","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editorial Board Page","authors":"","doi":"10.1016/S2949-7116(24)00049-2","DOIUrl":"10.1016/S2949-7116(24)00049-2","url":null,"abstract":"","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100164"},"PeriodicalIF":0.0,"publicationDate":"2024-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000492/pdfft?md5=23bc44533f72d7ff8393a6e7a7e13ac2&pid=1-s2.0-S2949711624000492-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141843507","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pilot study: Significance of I-FABP2 in the diagnosis of acute abdominal episodes in children","authors":"Szymon Gryboś ,&nbsp;Viera Karaffová ,&nbsp;Milan Kuchta ,&nbsp;Peter Krcho","doi":"10.1016/j.yjpso.2024.100146","DOIUrl":"10.1016/j.yjpso.2024.100146","url":null,"abstract":"<div><h3>Background</h3><p>Pediatric abdominal conditions, including necrotizing gastrointestinal diseases, pose significant diagnostic challenges due to clinic symptoms and limited diagnostic tools. Intestinal fatty acid binding protein 2 (I-FABP2) has emerged as a potential biomarker for intestinal damage, but its efficacy in diagnosing pediatric intestinal necrosis remains under explored.</p></div><div><h3>Methods</h3><p>A prospective study was conducted on 55 pediatric patients presenting with abdominal pain and suspected intestinal necrosis or intestinal perforation. Clinical, laboratory, and radiological data were collected, and intraoperative assessment of bowel necrosis was performed. Gene expression of I-FABP2 in peripheral blood was measured using Real-Time RT-PCR, and correlations with surgery and laboratory parameters were analyzed.</p></div><div><h3>Results</h3><p>Intraoperative assessment revealed a moderate sensitivity (61.1%) and specificity (73.7%) of I-FABP2 in identifying bowel necrosis. Positive predictive value (PPV) was high (81.5%), indicating a high likelihood of the condition when I-FABP2 is positive. However, the negative predictive value (NPV) was limited (50%), suggesting challenges in confidently excluding necrosis based on negative I-FABP2 results. Correlations were observed between I-FABP2 expression and elevated levels of C-reactive protein (CRP), interleukin-6 (IL-6), and white blood cell count (WBC), indicating its association with inflammatory processes.</p></div><div><h3>Conclusions</h3><p>While I-FABP2 shows promise as a biomarker for pediatric intestinal necrosis, its diagnostic utility may be enhanced when considered alongside other clinical parameters. Further research and validation studies are warranted to refine its clinical application and improve diagnostic accuracy in pediatric gastrointestinal conditions.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"8 ","pages":"Article 100146"},"PeriodicalIF":0.0,"publicationDate":"2024-06-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000315/pdfft?md5=21a1c6f80de62c71255bfa1aade16414&pid=1-s2.0-S2949711624000315-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142058488","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Immunohistochemistry study of PHOX2B expression in Hirschsprung's disease allied disorders","authors":"Huan Wang,&nbsp;Xiulei Sun,&nbsp;Likun Yu,&nbsp;Yiyuan Xu","doi":"10.1016/j.yjpso.2024.100148","DOIUrl":"10.1016/j.yjpso.2024.100148","url":null,"abstract":"<div><h3>Objective</h3><p>This study aimed to investigate the significance of PHOX2B expression in Hirschsprung's disease allied disorders (HAD).</p></div><div><h3>Methods</h3><p>Eight specimens of HAD were surgically resected and collected at Beijing Children's Hospital Affiliated to Capital Medical University Heilongjiang Hospital in recent years. Additionally, three specimens from patients with immature ganglion development in the intestinal wall and six control specimens from normal colons were also collected. PHOX2B, S-100, and CR antibodies were used for immunohistochemical staining to observe their expression at the mucosal, submucosal, and intrinsic muscular layer ganglia.</p></div><div><h3>Results</h3><p>In the control group,the nuclei of the submucosal and myenteric ganglion cells showed strong or weak PHOX2B staining pattern,the mature ganglion cells showed weak PHOX2B staining,and the immature ganglion cells showed strong staining;S-100 only stained nerve fibers but not ganglion cells; CR stained nerve fibers in the mucosal layer and submucosal layer and also stained ganglion cells. These three antibodies showed different expression patterns in poorly developed enteric ganglia and immature enteric ganglion cells.</p></div><div><h3>Conclusion</h3><p>PHOX2B is only expressed in the nucleus of ganglion cells, has discriminatory significance in identifying mature and immature ganglion cells, and is an essential marker for diagnosing HAD.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100148"},"PeriodicalIF":0.0,"publicationDate":"2024-06-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000339/pdfft?md5=10649e1203efdabd43cc634633648df5&pid=1-s2.0-S2949711624000339-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141401420","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Post-reduction observation and recurrence of pediatric intussusception in New Zealand: A national multicenter retrospective study","authors":"Brodie M. Elliott ,&nbsp;Jonathan M. Wells ,&nbsp;Shona Naera ,&nbsp;Andrew Weston ,&nbsp;Jacqueline Copland ,&nbsp;Shirin Gosavi ,&nbsp;Kristine Jung ,&nbsp;Udaya Samarakkody ,&nbsp;Georges K. Tinawi ,&nbsp;Samuel Haysom ,&nbsp;Rieke L. Meister ,&nbsp;Christopher I. Cassady ,&nbsp;Stephen Evans","doi":"10.1016/j.yjpso.2024.100155","DOIUrl":"https://doi.org/10.1016/j.yjpso.2024.100155","url":null,"abstract":"<div><h3>Background</h3><p>Intussusception is the most common cause of preschool intestinal obstruction. International data suggest that following uncomplicated enema reduction, 4-hour observation with prompt return to oral diet is safe. We aimed to investigate the rate and timing of intussusception recurrence across Aotearoa New Zealand, compared to the cost of routine post-reduction observation.</p></div><div><h3>Methods</h3><p>We performed a national 15-year retrospective, multicenter cohort study of all children treated for intussusception between 01 Jan 2007 and 01 Jan 2022 across AoNZ. A robust clinical dataset was collected using a REDCap proforma. We also investigated the financial and time costs of current in-hospital observation practices.</p></div><div><h3>Results</h3><p>During this period, primary enema reduction without general anesthetic was successful in 339 cases, requiring a median of 2 attempts (1–8). The median age was 0.8 years; 70.8% were male, and 17.1% were Māori. The median length of hospital stay was 25.9 h, primarily comprised of post-reduction observation (21.6 h). Intussusception recurrence within 30 days occurred in 29 children (8.5%) at a median time of 24.7 h post-reduction. Of the 19 cases recurring before discharge, 12 (3.5%) recurred between 4 and 24 h. The median observation period for those with successful primary enema reduction was 21.6 h; if reduced to 4 h, savings of NZD 883,632 (USD 529,825) and 7342 ward hours were possible.</p></div><div><h3>Conclusions</h3><p>Nationally, the intussusception recurrence rate after uncomplicated enema reduction was 8.5%, but only 3.5% occurred between the 4–24 hour period post-reduction. Expediting discharge by avoiding routine overnight observation appears safe and economically viable.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100155"},"PeriodicalIF":0.0,"publicationDate":"2024-06-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000406/pdfft?md5=c60ac96156584f54e099bc3c49f4db4d&pid=1-s2.0-S2949711624000406-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141323365","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Vacuum assisted closure therapy in children with complicated abdominal wounds, with or without viscero-cutaneous fistulae","authors":"","doi":"10.1016/j.yjpso.2024.100153","DOIUrl":"10.1016/j.yjpso.2024.100153","url":null,"abstract":"<div><h3>Background</h3><p>Management of complicated abdominal wounds is challenging in children. Vacuum-assisted closure proved to be beneficial. We aimed to report the outcome of VAC over 5 years’ practice in complicated abdominal wounds with or without viscero-cutaneous fistulae.</p></div><div><h3>Methods</h3><p>Retrospective review of children managed by VAC over 5 years, from January 2017 to December 2022. Included patients had complicated abdominal wounds. Customized VAC devices with continuous negative pressure were used for all patients. The applied pressure was tailored according to the patient's age and the wound condition.</p></div><div><h3>Results</h3><p>Nineteen patients were included, their ages ranged from 3 months to 15 years. Peritonitis was the main pathology in 12 patients, abdominal wall infection in 4, and a combination of both in 3. VAC was applied over closed wounds in 10 patients and over open wounds in 9; 6 of them had cutaneous fistulae. The median duration of VAC application over closed wounds was 7.5 days, while it was 10 days in open wounds. VAC promoted healing in closed and narrow-gapped wounds, while it was successfully used as a bridging therapy in 4 patients with wide-gapped wounds. Patients with viscero-cutaneous fistulae were successfully managed without further surgical intervention. VAC was complicated by fascial dehiscence with evisceration and incisional hernia in two patients.</p></div><div><h3>Conclusion</h3><p>VAC could be beneficial to complicated abdominal wounds in pediatric age. It was helpful in managing small viscero-cutaneous fistulae.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"8 ","pages":"Article 100153"},"PeriodicalIF":0.0,"publicationDate":"2024-06-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000388/pdfft?md5=00eb53d8a7f51a71ae6910fd05bd3d26&pid=1-s2.0-S2949711624000388-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141407159","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Attending physicians and trainees' perspective of patient safety culture in operating room: A mixed methods study","authors":"Nutan B. Hebballi ,&nbsp;Molly P. Kilcullen ,&nbsp;Maryam Broussard ,&nbsp;Madelene J. Ottosen ,&nbsp;Eric J. Thomas ,&nbsp;Eduardo Salas ,&nbsp;KuoJen Tsao","doi":"10.1016/j.yjpso.2024.100152","DOIUrl":"https://doi.org/10.1016/j.yjpso.2024.100152","url":null,"abstract":"<div><h3>Background</h3><p>Regulatory quantitative assessments are routinely undertaken to understand the patient safety culture within an organization. However, adjunctive qualitative approaches to explore low-scoring areas of safety culture in the operating room (OR) are lacking and may provide additional insight. Thus, we explored OR patient safety culture perceptions of pediatric surgical providers, specifically among attending physicians and trainees.</p></div><div><h3>Methods</h3><p>A cross-sectional survey was pilot-tested by pediatric surgical attending physicians and surgical trainees from one academic hospital OR. The “Safer Culture” survey was developed using validated survey measures of concepts from the Safer Culture framework and modified by cognitive interviews with subject-matter experts. The survey was electronically administered, and the results were analyzed descriptively, and percent positive scores were computed. Semi-structured interviews with a purposive sample of participants were then conducted and thematically analyzed.</p></div><div><h3>Results</h3><p>Of the 144 participants, 67 completed the survey (response rate: 46.5 %). <em>Safety competence</em> (&gt;95 %) and <em>individual commitment &amp; prioritization of safety</em> (&gt;90 %) were rated highest by attending physicians and trainees. Trainees rated <em>policies &amp; resources for safety</em> and <em>cohesion</em> the lowest (&lt;60 %). Attending physicians and trainees rated <em>just culture</em> the lowest (&lt;56 %). Interview data revealed three overarching themes explain the lowest ratings: 1) feedback and fear influence incident reporting, 2) lack of accountability and learning affect just culture, and 3) individual and group preparedness impact safe surgical care.</p></div><div><h3>Conclusions</h3><p>Safer Culture survey identified low-performing issues affecting OR safety culture by pediatric surgical providers. Their perceptions specified areas to focus initiatives to improve OR safety culture.</p></div><div><h3>Levels of evidence</h3><p>Level III.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"7 ","pages":"Article 100152"},"PeriodicalIF":0.0,"publicationDate":"2024-06-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000376/pdfft?md5=ecbe8f4567cbbdf6cbc5c319c2deabde&pid=1-s2.0-S2949711624000376-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141315023","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Non-Wilms renal tumors: Twenty years experience in a referral center","authors":"Cristina Garcés-Visier ,&nbsp;Przemyslaw Maruszewski ,&nbsp;Ana Lourdes Luis-Huertas ,&nbsp;Pedro Borrrego-Jimenez ,&nbsp;Daniel Azorín ,&nbsp;Alberto Martín-Vega ,&nbsp;Manuel Espinoza-Vega ,&nbsp;Blanca Herrero-Velasco ,&nbsp;Jose Lorenzo Alonso-Calderón","doi":"10.1016/j.yjpso.2024.100151","DOIUrl":"10.1016/j.yjpso.2024.100151","url":null,"abstract":"<div><h3>Background</h3><p>Non-Wilms Renal Tumors (NWRT) constitute less than 10% of all renal tumors diagnosed in childhood. We studied our experience in the diagnosis and management of these tumors to compare our results with the current literature.</p></div><div><h3>Study design</h3><p>This is a retrospective observational study which includes all patients aged 0-18 years with histopathological diagnosis of NWRT treated in our center during the period 2000-2022.</p></div><div><h3>Results</h3><p>We identified 10 patients with diagnosis of: cystic nephroma(3), congenital mesoblastic nephroma(1), renal cell carcinoma(2), clear cell sarcoma of the kidney (2), renal Ewing's sarcoma(1) and malignant rhabdoid tumor of the kidney(1). Sixty percent (60%) were female. The median age at diagnosis was 3.25 years (IQR 1.5-10). Median age at diagnosis excluding CN was 6 years old (IQR 2.62-10.75). Molecular alterations were detected in 60% of the cases. Hematuria (40%) and palpable abdominal mass (40%) were the most frequent presenting symptoms. In total, 62.5% patients were misdiagnosed during the preoperative period as Wilms' tumor (WT), based on imaging data. <del>A</del> Radical nephroureterectomy was performed in all cases and staging lymphadenectomy in 70%. We recorded a major complication in one patient, who suffered a contralateral lower renal pole infarction due to section of a polar vessel during surgery. This patient had no preoperative vascular study. The recurrence-free survival rate was 90% with a median follow-up of 6.4 years (IQR 2-13.9).</p></div><div><h3>Discussion</h3><p>Radiological imaging has fundamental importance in the diagnosis of renal tumors, especially to identify children who might benefit from initial surgical treatment or the indication of biopsy for preoperative histopathological confirmation before initiating cytotoxic treatment. However, there are no pathognomonic imaging findings that clearly differentiate between WT and other renal tumors, nor among the heterogeneous group of NWRT[<span><span>3</span></span>]. In our series, 62.5% of patients were misdiagnosed as WT based on imaging features.</p><p>We found different molecular alterations in 60% of our patients (Table 1). None of them predispose to the development of bilateral tumors nor correlates with predisposing syndromes of metachronous tumors.</p><p>Apart from its retrospective design, this study is limited by small number of cases and a long study period.</p></div><div><h3>Conclusions</h3><p>Correct differential diagnosis of NWRT is necessary for an adequate therapeutic approach. The molecular-genetic profile is an important step in the diagnosis of NWRT that allows for the use of targeted therapies in refractory patients. Detailed anatomical study by vascular mapping minimizes the risk of iatrogenic damage during tumor resection.</p></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"8 ","pages":"Article 100151"},"PeriodicalIF":0.0,"publicationDate":"2024-05-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S2949711624000364/pdfft?md5=83215d0377f5c8331eba5eab9dcffb06&pid=1-s2.0-S2949711624000364-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141959401","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}