{"title":"Current and future state of the management of Hirschsprung disease.","authors":"Jessica L Mueller, Ryo Hotta","doi":"10.1136/wjps-2024-000860","DOIUrl":"10.1136/wjps-2024-000860","url":null,"abstract":"<p><p>The enteric nervous system (ENS) consists of a network of neurons and glia that control numerous complex functions of the gastrointestinal tract. Hirschsprung disease (HSCR) is a congenital disorder characterized by the absence of ENS along variable lengths of distal intestine due to failure of neural crest-derived cells to colonize the distal intestine during embryonic development. A patient with HSCR usually presents with severe constipation in the neonatal period and is diagnosed by rectal suction biopsy, followed by pull-through procedure to surgically remove the affected segment and reconnect the proximal ganglionated intestine to the anus. Outcomes after pull-through surgery are suboptimal and many patients suffer from ongoing issues of dysmotility and bowel dysfunction, suggesting there is room for optimizing the management of this disease. This review focuses on discussing the recent advances to better understand HSCR and leverage them for more accurate and potentially less invasive diagnosis. We also discuss the potential future management of HSCR, particularly cell-based approaches for the treatment of HSCR.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"e000860"},"PeriodicalIF":0.8,"publicationDate":"2025-03-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11962771/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143773585","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Value of preoperative APRI and FIB-4 in assessing short-term prognosis after Kasai portoenterostomy.","authors":"Bingliang Li, Yingyu Jia, Hongxia Ren","doi":"10.1136/wjps-2025-001002","DOIUrl":"10.1136/wjps-2025-001002","url":null,"abstract":"<p><strong>Objective: </strong>To explore the value of preoperative Aspartate Aminotransferase-to-Platelet Ratio Index (APRI) and Fibrosis-4 score (FIB-4) in predicting short-term prognosis of children with biliary atresia (BA) undergoing Kasai portoenterostomy (KPE).</p><p><strong>Methods: </strong>Clinical data from children who underwent KPE were analyzed. Patients were divided into two groups based on their 2-year native liver survival after KPE. General information and laboratory findings were collected before KPE. The difference in liver fibrosis between the two groups was analyzed. The predictive efficacy of each index for short-term prognosis of children with BA was evaluated using the receiver operating characteristic curve.</p><p><strong>Results: </strong>The APRI and FIB-4 in the good prognosis group were lower than those in the poor prognosis group (<i>p</i>=0.008 and 0.023, respectively), and postoperative jaundice clearance rate was higher (<i>p</i>=0.002). In the poor prognosis group, gamma-glutamyl transpeptidase levels in the F3+F4 fibrosis subgroup were significantly higher than those in the F1 subgroup (<i>p</i>=0.038). The area under the curve (AUC) for preoperative APRI in predicting short-term prognosis was the highest at 0.667, with a cut-off value of 1.190. The AUC for preoperative FIB-4 was predicted to be 0.642. The combination of preoperative APRI and alanine aminotransferase showed a higher AUC for prognosis prediction compared with either marker alone.</p><p><strong>Conclusions: </strong>Preoperative APRI and FIB-4 may havepredictive values for short-term prognosis. The predictive value of APRI and FIB-4 combined with liver function indicators for the short-term prognosis of children is superior to that of a single indicator, but the results are not satisfactory.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"e001002"},"PeriodicalIF":0.8,"publicationDate":"2025-03-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11956308/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143754730","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Suhaib Abdulfattah, Iqra Nadeem, Yashaswi Parikh, Sami Shaikh, Sonam Saxena, Aznive Aghababian, Katherine Fischer, Sameer Mittal, Arun K Srinivasan, Aseem R Shukla
{"title":"Management of urachal anomalies in children: insights from a retrospective cohort study.","authors":"Suhaib Abdulfattah, Iqra Nadeem, Yashaswi Parikh, Sami Shaikh, Sonam Saxena, Aznive Aghababian, Katherine Fischer, Sameer Mittal, Arun K Srinivasan, Aseem R Shukla","doi":"10.1136/wjps-2024-000977","DOIUrl":"10.1136/wjps-2024-000977","url":null,"abstract":"<p><strong>Introduction: </strong>While large and symptomatic urachal anomalies (UAs) often lead to surgical excision, urachal malignancy is rare, rendering prophylactic excision unwarranted. We hypothesize that in the pediatric population, the presentation of an infected UA is the predominant etiology leading to surgical intervention.</p><p><strong>Methods: </strong>We retrospectively identified patients with UA from July 2012 to December 2021 evaluated in our urology outpatient. Inclusion criteria included patients ≤18 years old and confirmation of UA on ultrasound (US). Exclusion criteria were patients diagnosed with clinical mimickers on US, excision by general pediatric surgery service, or excision done concomitantly as part of another urological procedure.</p><p><strong>Results: </strong>We identified a total of 78 patients with UA. Of those, 35 (44.9%) underwent excision. The observation cohort was younger (5 months <i>vs.</i> 73 months, <i>p</i>=0.002), more likely to be asymptomatic (65.1% <i>vs.</i> 85.7%, <i>p</i>=0.038), and more likely to have UA characterized as a 'remnant' on US (72.1% <i>vs.</i> 48.6%, <i>p</i>=0.034). Univariate analysis showed that infected or symptomatic UA, or those characterized as hyperemic, cystic, or as a diverticulum on US were more likely to be excised. Multivariable analysis shows that patients with a 'urachal cyst' classification (<i>p</i>=0.008) and infectious presentation (<i>p</i>=0.046) were more likely to undergo surgical intervention. Excision was accomplished laparoscopically (80.0%) or robotically (11.4%). No excised UA was suspicious for malignancy.</p><p><strong>Conclusion: </strong>We present a large pediatric cohort with UA and found that infectious symptoms at presentation and those classified as 'urachal cyst' based on US were more likely to prompt surgical excision as compared with other factors.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"e000977"},"PeriodicalIF":0.8,"publicationDate":"2025-03-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11931935/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143701718","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Short-term outcomes and risk factors for mortality in neonatal cardiac surgeries with cardiopulmonary bypass: a 5-year single-center report.","authors":"Xianghong Zhang, Tingting Wen, Jiajie Fan, Yunxiang Qiu, Jiangmei Wang, Liyang Ying, Jiangen Yu, Xiangming Fan, Shengwen Song, Shanshan Shi, Xiangming Fang, Qiang Shu","doi":"10.1136/wjps-2024-000968","DOIUrl":"10.1136/wjps-2024-000968","url":null,"abstract":"<p><strong>Background: </strong>Open-heart surgeries carry significantly greater risks in neonates than in older children. The current study aimed to analyze the short-term outcomes and risk factors for mortality in neonates undergoing cardiac surgeries.</p><p><strong>Methods: </strong>This retrospective analysis included neonates who underwent cardiac surgeries with cardiopulmonary bypass (CPB) at our center between 2019 and 2023. Perioperative and operative factors were collected, and in-hospital outcomes and risk factors for mortality were identified.</p><p><strong>Results: </strong>We analyzed the medical records of 190 neonates, including 111 males and 79 females. The median age at the time of surgery was 15 days, with a median weight of 3.2 kg. The overall mortality rate was 11.6%. Major postoperative complications included infection (48.9%), low cardiac output (48.4%), hepatic insufficiency (24.9%), acute kidney injury (22.1%), unplanned reintervention (13.1%), intracranial hemorrhage (3.7%), and cerebral infarction (0.5%). Multivariable analysis identified prolonged CPB time (odds ratio (OR)=1.018, <i>p</i>=0.014), Risk Stratification for Congenital Heart Surgery (RASCH-2) categories of ≥4 (OR=11.927, <i>p</i>=0.026), extracorporeal membrane oxygenation (ECMO) duration (OR=1.606, <i>p</i>=0.036), and peritoneal hemodialysis (OR=9.252, <i>p</i>=0.014) as significant predictors for mortality.</p><p><strong>Conclusions: </strong>Despite advancements in recent decades, continued vigilance is required to further reduce mortality rates among neonates undergoing cardiac surgeries.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 Suppl 1","pages":"e000968"},"PeriodicalIF":0.8,"publicationDate":"2025-03-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11927473/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143693494","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Conditions that mimic Hirschsprung's disease, but that are not Hirschsprung's disease.","authors":"Shun Onishi, Satoshi Ieiri","doi":"10.1136/wjps-2024-000918","DOIUrl":"https://doi.org/10.1136/wjps-2024-000918","url":null,"abstract":"<p><p>Hirschsprung's disease (HSCR) is widely recognized in pediatric surgery. This condition has been elucidated, and therapeutic approaches have been developed. However, even when ganglion cells are present in the rectum, some patients still experience symptoms such as bowel obstruction, intestinal dilatation, and chronic constipation, which are similar to those observed in HSCR. A consensus regarding the terminology for these diseases is yet to be established. This group of diseases was defined as 'allied disorders of Hirschsprung's disease' (ADHD). They are classified into two categories based on pathology: (1) Abnormal ganglia, including immaturity of ganglia, hypoganglionosis, and intestinal neuronal dysplasia; and (2) Normal ganglia, including megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS), segmental dilatation, internal anal sphincter achalasia, and chronic idiopathic intestinal pseudo-obstruction (CIIP). Hypoganglionosis, MMIHS, and CIIP are particularly severe and not curable by surgery. Guidelines were compiled to facilitate an accurate clinical diagnosis and provide appropriate treatment strategies for each disease. A full-thickness rectal biopsy with H&E and acetylcholinesterase staining is often required for a differential diagnosis. Patients are now able to survive longer with enteral nutrition combined with long-term intravenous nutrition and decompression of the gastrointestinal tract. However, all treatment strategies are symptomatic. It is necessary to improve the results of small intestine transplantation and to develop new therapies using regenerative medicine.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"e000918"},"PeriodicalIF":0.8,"publicationDate":"2025-03-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11907078/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143651036","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Medium- and long-term outcomes of tunneled labial mucosa tube grafts for the repair of primary severe hypospadias and failed hypospadias.","authors":"Hengyou Wang, Xiang Yan, Guangjie Chen, Chang Tao, Dehua Wu, Wei Ru, Daxing Tang","doi":"10.1136/wjps-2024-000976","DOIUrl":"10.1136/wjps-2024-000976","url":null,"abstract":"<p><strong>Objective: </strong>This study aimed to evaluate the medium and long-term outcomes of tunneled labial mucosa tube graft urethroplasty in the treatment of primary severe hypospadias and failed hypospadias.</p><p><strong>Methods: </strong>We retrospectively reviewed the medical records of the patients who underwent tunneled labial mucosa tube graft urethroplasty at a single center between 2007 and 2020. Data collected included patient demographic, operative and post-operative characteristics. The surgical procedure was performed in two stages. Binary logistic regression model was used to identify risk factors for postoperative complication.</p><p><strong>Results: </strong>A total of 82 patients who were followed for at least 12 months were included in this study. Among these, 56 patients received primary repair, while 26 had previously undergone failed reconstruction. The mean age at surgery was 5.0 years. Postoperative urinary fistula occurred in 36 patients, with 24 requiring surgical repair and 12 resolving spontaneously. Urethral stricture developed in 26 patients, with 20 undergoing surgical intervention and 6 managed with urethral dilation. Preoperative surgical history was identified as an independent risk factor for postoperative complications (<i>p</i>=0.015).</p><p><strong>Conclusions: </strong>Although the complication rate associated with tunneled labial mucosa tube graft urethroplasty is relatively high compared with other techniques, it remains a viable option for patients with insufficient replacement materials, particularly for primary severe hypospadias. For cases of failed hypospadias repair, this procedure should be considered selectively based on patient-specific factors.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"e000976"},"PeriodicalIF":0.8,"publicationDate":"2025-03-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11881169/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143568312","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Tribute to reviewers (January 1, 2025 to December 31, 2025).","authors":"","doi":"10.1136/wjps-2025-reviewers","DOIUrl":"https://doi.org/10.1136/wjps-2025-reviewers","url":null,"abstract":"","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"8 1","pages":"ereviewers"},"PeriodicalIF":0.8,"publicationDate":"2025-02-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11836849/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143469017","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Haley Etskovitz, Rosa S Kim, Sarah Ziqi Wang, Prathima Nandivada
{"title":"Surgical management of short-segment Hirschsprung disease.","authors":"Haley Etskovitz, Rosa S Kim, Sarah Ziqi Wang, Prathima Nandivada","doi":"10.1136/wjps-2024-000916","DOIUrl":"10.1136/wjps-2024-000916","url":null,"abstract":"<p><p>Hirschsprung disease (HSCR) is the most common congenital motility disorder of the intestine, characterized by the absence of ganglion cells in the myenteric and submucosal plexuses, leading to functional bowel obstruction. Short-segment Hirschsprung disease (SS-HSCR) accounts for the majority of cases, with surgical resection being the cornerstone of treatment. Despite advances in surgical techniques, considerable variability exists in practice regarding the timing of surgery, the choice of technique, and the length of aganglionic rectal cuff to resect. This review synthesizes the current evidence surrounding surgical management of SS-HSCR, discussing technique-specific outcomes and areas for future research, with a focus on optimizing patient care and functional outcomes.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"7 4","pages":"e000916"},"PeriodicalIF":0.8,"publicationDate":"2024-12-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11752025/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143024949","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Felix Oyania, Kara Faktor, Sarah Ullrich, Doruk Ozgediz, Meera Kotagal
{"title":"Delayed diagnosis of anorectal malformations: a call for standardization of the current definitions.","authors":"Felix Oyania, Kara Faktor, Sarah Ullrich, Doruk Ozgediz, Meera Kotagal","doi":"10.1136/wjps-2024-000960","DOIUrl":"10.1136/wjps-2024-000960","url":null,"abstract":"","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"7 4","pages":"e000960"},"PeriodicalIF":0.8,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11683927/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142907738","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Paulo Sérgio Lucas Da Silva, Renato De Oliveira, Nikkei Tamura, Leonardo Camargo, Emerson Yukio Kubo
{"title":"Outcome of anatomical versus non-anatomical lung resection for necrotizing pneumonia in children.","authors":"Paulo Sérgio Lucas Da Silva, Renato De Oliveira, Nikkei Tamura, Leonardo Camargo, Emerson Yukio Kubo","doi":"10.1136/wjps-2024-000943","DOIUrl":"10.1136/wjps-2024-000943","url":null,"abstract":"<p><strong>Objective: </strong>We aimed to evaluate the characteristics, complications and outcomes of necrotizing pneumonia (NP) requiring surgical intervention.</p><p><strong>Methods: </strong>We conducted a retrospective study of all children who underwent surgical therapy for NP from January 2010 to December 2023. Patients were analyzed based on two surgical approaches: anatomic resection (AR) or non-AR (NAR).</p><p><strong>Results: </strong>A total of 66 patients (median age: 36 months) required a surgical intervention for NP. A total of 37 patients received AR, 29 received NAR. The AR procedures were segmentectomy (<i>n</i>=29), lobectomy (<i>n</i>=8), bilobectomy (<i>n</i>=1) whereas NAR included wedge resection (<i>n</i>=13) and necrosectomy (<i>n</i>=16). The most common reasons for surgery were failure to respond to treatment (43.9%) and sepsis/septic shock (42.4%). A significantly greater proportion of patients in the AR group underwent surgery due to sepsis (<i>p</i>=0.023). There was no difference in the proportion of patients experiencing complications between the AR group (40.5%) and the NAR group (27.5%) (<i>p</i>=0.266). The majority of complications in both groups (68.0%) were categorized as minor, with 59.0% of cases occurring in patients who underwent AR. Prolonged air leak was the most frequent complication in both groups. There was no difference in the postoperative hospital stay, or duration of mechanical ventilation between the groups. There were no deaths.</p><p><strong>Conclusions: </strong>Surgical intervention for NP may result in complications in one-third of patients, mostly minor and unlikely to significantly impact outcomes. Surgery should be tailored to the extent of parenchymal involvement.</p>","PeriodicalId":23823,"journal":{"name":"World Journal of Pediatric Surgery","volume":"7 4","pages":"e000943"},"PeriodicalIF":0.8,"publicationDate":"2024-12-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11683895/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142907741","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}