Journal of Pediatric Surgery Open最新文献

{"title":"The safety of cuffed, tunneled central venous catheters across access sites in pediatric patients: a single institution’s experience","authors":"Andrew N Hendrix ,&nbsp;Ankur Makani ,&nbsp;Andrew D Costa ,&nbsp;Timothy Pitts ,&nbsp;Bhairav Shah","doi":"10.1016/j.yjpso.2025.100218","DOIUrl":"10.1016/j.yjpso.2025.100218","url":null,"abstract":"<div><h3>Introduction</h3><div>Few studies exist comparing immediate and late complication rates between internal jugular (IJ), subclavian, and femoral vein placement of cuffed, tunneled central venous catheters (ctCVCs). Our recent systematic review showed that IJ placement is performed 96.9 % of the time, however, at our institution, femoral placement is performed at a higher rate. The purpose of this study is to investigate the safety and efficacy of femoral placement of ctCVCs in pediatric patients. We hypothesized that femoral access would demonstrate fewer immediate complications related to insertion without increased rates of late complications.</div></div><div><h3>Methods</h3><div>A retrospective review of 202 pediatric patients with ctCVCs placed at our institution between 2019 and 2024 was performed. Variables included patient demographics, location of line, type of line, size of lumen, number of lumens, line duration, and anesthesia type. Primary outcomes included immediate complications, central line associated bloodstream infection (CLABSI), and late mechanical complications.</div></div><div><h3>Results</h3><div>Immediate, CLABSI, and mechanical complication rates were 2.5 %, 10.9 %, and 18.8 %, respectively. Patients undergoing ctCVC placement in the IJ vein were 29.5 times more likely to receive general anesthesia and fluoroscopic guidance compared to femoral access (OR = 29.5, p &lt; 0.001) with no significant difference in immediate complications. Propensity-matched femoral placements had significantly lower CLABSI rates (0 % vs. 20.59 %; p = 0.0156) compared to IJ and subclavian.</div></div><div><h3>Conclusions</h3><div>Our findings suggest that femoral placement of ctCVCs may be a safe and effective alternative to IJ and subclavian placements, with a decreased risk of CLABSI as well as a decreased need for general anesthesia and fluoroscopic guidance, particularly for younger or lower weight pediatric patients.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100218"},"PeriodicalIF":0.0,"publicationDate":"2025-07-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144563033","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cost analysis of four surgical techniques for pediatric inguinal hernia repair in a Dutch hospital","authors":"Roxanne Eurlings ,&nbsp;Merel L. Kimman ,&nbsp;Lloyd Brandts ,&nbsp;Ruben G.J. Visschers ,&nbsp;Wim G. van Gemert","doi":"10.1016/j.yjpso.2025.100217","DOIUrl":"10.1016/j.yjpso.2025.100217","url":null,"abstract":"<div><h3>Introduction</h3><div>Pediatric inguinal hernia repair (IHR) can be performed using open repair, laparoscopic approaches such as percutaneous internal ring suture (PIRS) or conventional laparoscopic IHR (LIHR) with N-/Z-suture, and robot-assisted IHR (RIHR). While extensive literature on clinical outcomes exists, cost data remain scarce. This study aims to determine the real costs of these four techniques.</div></div><div><h3>Methods</h3><div>A retrospective cohort of consecutive patients with pediatric IHR in the past five years (2019–2023) was analyzed. Patients were grouped by technique. Individual one-year costs were collected, including pre-operative, per-operative, postoperative and complication costs. Kruskal-Wallis test was performed to compare costs between techniques. Multivariable-adjusted linear regression analyses were performed to identify factors associated with (non-OR) costs.</div></div><div><h3>Results</h3><div>184 patients were included in four groups: PIRS (<em>n</em> = 56), LIHR (<em>n</em> = 56), Open (<em>n</em> = 50), RIHR (<em>n</em> = 22). Total OR cost for PIRS were significantly lower than for the other laparoscopic techniques (€1915 vs. €2414 for LIHR; <em>p</em> = 0.001 and €3340 RIHR; <em>p</em> = 0.000), but not for the open technique (€1915 vs. €1971; <em>p</em> = 1.000). They were the highest for RIHR, probably due to prolonged anesthesia time, which is explained by docking of the robotic system. Other costs of the treatment journey (excluding OR costs) were significantly associated with gestational age (4.2 % increase per week the patient was born earlier; <em>p</em> = 0.001), age at surgery (7 % increase per year the patient was younger; <em>p</em> = 0.001), reducible incarceration (40.7 % increase; <em>p</em> = 0.037) and recurrence (124.5 % increase; <em>p</em> = 0.001). Hospitalization costs were significantly higher for the open technique (38.8 % increase; <em>p</em> = 0.030).</div></div><div><h3>Discussion and conclusion</h3><div>This retrospective analysis shows that the PIRS technique is related to lower OR costs. Other (non-OR) costs are highly dependent on patient characteristics and clinical outcomes. Prospective randomized studies are needed to confirm these findings and guide optimal decision making concerning surgical technique with an adequate cost-effectiveness study.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100217"},"PeriodicalIF":0.0,"publicationDate":"2025-06-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144330298","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Identification of clinical and laboratory factors predictive of long term-native liver survival after Kasai portoenterostomy","authors":"Takashi Kobayashi,&nbsp;Yoshiaki Kinoshita,&nbsp;Toshiyuki Ohyama,&nbsp;Yuhki Arai,&nbsp;Yu Sugai,&nbsp;Koichi Saito,&nbsp;Yu Hamasaki","doi":"10.1016/j.yjpso.2025.100216","DOIUrl":"10.1016/j.yjpso.2025.100216","url":null,"abstract":"<div><h3>Purpose</h3><div>This study explored pre- and post-operative predictive factors for long-term native liver survival (NLS) in patients with biliary atresia (BA) after Kasai hepatoportoenterostomy (KPE).</div></div><div><h3>Methods</h3><div>We retrospectively reviewed 50 consecutive patients with BA between January 1990 and December 2023 at our institute after excluding those with splenic malformations. We analyzed the prognostic factors at pre-KPE, 30 days and 6 months after KPE for a long-term NLS.</div></div><div><h3>Results</h3><div>Of the 50 patients, 25 survived with their native liver, and 25 showed native liver failure over a median 109 (2–395) months. A multivariable analysis showed that early age (≤56 days old) at KPE was a predictive factor for an NLS (odds ratio [OR]: 5.43, 95 % confidence interval [CI] 1.04–28.30; <em>p</em> = 0.045). Serum total bilirubin (≤3.5 mg/dl) at 30 days (OR: 9.31, 95 % CI 1.57–55.07; <em>p</em> = 0.014) and bile acid (≤49.6 nmol/ml) at 6 months after KPE were predictive factors for an NLS (OR: 35.33, 95 % CI 1.82–686.03; <em>p</em> = 0.019).</div></div><div><h3>Conclusion</h3><div>KPE for BA should be performed early (≤56 days old) for an NLS. Serum total bilirubin ≤3.5 mg/dl at 30 days and bile acid ≤49.6 nmol/ml at 6 months after KPE were predictive factors for an NLS.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100216"},"PeriodicalIF":0.0,"publicationDate":"2025-06-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144335817","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Minimally invasive excision combined with epidermal autografting and poly-lactic acid skin substitute improves outcomes in pediatric partial thickness burns","authors":"Julia Maxey ,&nbsp;Mallory Wampler ,&nbsp;Djoni Elkady ,&nbsp;Adrienne DeVault ,&nbsp;Kelly Williamson ,&nbsp;Mimi Borrelli ,&nbsp;Richard Lou ,&nbsp;Anjay Khandelwal","doi":"10.1016/j.yjpso.2025.100215","DOIUrl":"10.1016/j.yjpso.2025.100215","url":null,"abstract":"<div><h3>Introduction</h3><div>The traditional approach to pediatric deep partial thickness burns has been a “watch and wait” attitude with frequent dressing changes, primarily due to evidence that pediatric burns will often heal, and that early debridement leads to removal of viable tissue. However, there is still significant morbidity with delayed healing, increased pain and dressing changes, prolonged hospital stays, added cost and hypertrophic scarring. Dermabrasion is a minimally invasive excisional technique that may preserve viable dermis while epidermal autografting (EA) utilizing an autologous skin cell suspension (ASCS) can be used for partial thickness burns to facilitate wound healing. In an effort to improve outcomes, the authors evaluated the outcomes of dermabrasion with epidermal autografting in the pediatric population.</div></div><div><h3>Methods</h3><div>A retrospective review of pediatric patients (&lt;18 years old) with superficial-mid or deep partial thickness burns was performed. From July 2021 to July 2023, 44 patients that were treated with epidermal autografting and application of poly-lactic acid skin substitute were included in the study. A propensity matched group of patients based on age and TBSA from January 2017 to June 2021 that were managed conservatively with dressing changes were included (<em>n</em> = 44). Patient information that was collected includes: demographics, mechanism of injury, percentage of total burn surface area (%TBSA), time to operating room (OR), length of stay (LOS), narcotic use, postoperative complication, and number of dressing changes requiring sedation.</div></div><div><h3>Results</h3><div>The medical charts of 88 patients [mean age: 4.61 years (range: 0.02–18)] were examined with an average %TBSA of 7.29 (range: 0.1–20). When compared to conservative treatment, epidermal autografting led to increased re-epithelialization (2x), reduced number of dressing changes (1.5 fewer), significant reduction in scarring (by 79 %).</div></div><div><h3>Conclusions</h3><div>Within the pediatric population, epidermal autografting can serve as an efficacious treatment modality for management of partial thickness burns.</div></div><div><h3>Applicability of research to practice</h3><div>Epidermal autografting can be effectively utilized within the pediatric population for partial thickness burns.</div></div><div><h3>External funding</h3><div>None</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100215"},"PeriodicalIF":0.0,"publicationDate":"2025-05-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144069941","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Editorial:Innovation and Emerging Technology in Pediatric Surgery","authors":"Satoshi IEIRI M.D., Ph.D.","doi":"10.1016/j.yjpso.2025.100214","DOIUrl":"10.1016/j.yjpso.2025.100214","url":null,"abstract":"","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100214"},"PeriodicalIF":0.0,"publicationDate":"2025-05-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144213020","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Navigating complexities: Confronting intriguing challenges in Wilms tumor with inferior vena cava (IVC) and atrial thrombus","authors":"Nabila Talat ,&nbsp;Muhammad Usama Aziz ,&nbsp;Imran Hashim ,&nbsp;Farrukh Mahmood Sattar ,&nbsp;Muhammad Bilal Mirza ,&nbsp;Wajeeh Ur Rehman ,&nbsp;Sajid Iqbal Nayyar ,&nbsp;Muhammad Saleem ,&nbsp;Warda Tahir","doi":"10.1016/j.yjpso.2025.100213","DOIUrl":"10.1016/j.yjpso.2025.100213","url":null,"abstract":"<div><h3>Objective</h3><div>The objective of the study was to highlight the rarity of Wilms tumor with metastatic thrombus in inferior vena cava and right atrium, emphasize the multi-disciplinary approach, meticulous surgical techniques, associated technical challenges, and the outcome.</div></div><div><h3>Methods</h3><div>This study was conducted in the Department of Pediatric Surgery, The University of Child Health Sciences, and The Children’s Hospital Lahore, Pakistan. Out of total 183 patients with Wilms tumor presented, 17 patients showed Wilms tumor with vascular extension. A review of their data regarding demographic details, clinical presentation, radiological findings, operative management, and post-operative course was done.</div></div><div><h3>Results</h3><div>All 17 patients of Wilms tumor with IVC thrombus were discussed in the Institutional Tumor Board meeting and after receiving neoadjuvant chemotherapy. 16 patients underwent surgical resection. One patient with distant metastasis expired before surgery. Non-anatomical hepatic resection of segment VIII has to be done in one patient. Right atrial exploration was done in 2 patients in a joint venture with a cardiac surgery team. Tragically, 2 patients passed away postoperatively. One patient had recurrent tumor thrombus for which he had extensive chemotherapy followed by thrombectomy. The remaining 13 patients are doing fine on follow-up with the longest follow-up of 5 years.</div></div><div><h3>Conclusion</h3><div>Our small number of patients describe the rarity of Wilms tumor with IVC thrombus. Neoadjuvant chemotherapy has gained success in the regression of thrombus which is followed by surgical excision. IVC Thrombectomy after taking proximal and distal vascular control is quite challenging for surgeons not frequently dealing with these cases. Cardiac surgery aid is also required in cases of intra-atrial extension of thrombus. Multidisciplinary management is essential for improved outcomes.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100213"},"PeriodicalIF":0.0,"publicationDate":"2025-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143922055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Children with neurodevelopmental disabilities have worse appendicitis outcomes","authors":"Su Yeon Lee ,&nbsp;Emily A Byrd ,&nbsp;Jonathan E Kohler","doi":"10.1016/j.yjpso.2025.100210","DOIUrl":"10.1016/j.yjpso.2025.100210","url":null,"abstract":"<div><h3>Objectives</h3><div>Appendicitis is the most common surgical emergency in children. Children with neurodevelopmental disabilities (NDD) have communication differences that can complicate their medical care. We hypothesized that children with NDD are more likely to present with more severe appendicitis and have worse clinical outcomes.</div></div><div><h3>Methods</h3><div>We used the National Surgical Quality Improvement Program Pediatric (NSQIP-P) database to study appendectomies performed between 2015 and 2020. The primary outcome was complicated appendicitis. Outcomes were compared between patients with and without the established NSQIP-P variable for “developmental delay”.</div></div><div><h3>Results</h3><div>97,236 patients met inclusion criteria. NDD patients underwent interval appendectomy at significantly higher rates compared to neurotypical patients (4.19 % vs. 2.62 %, <em>p</em> &lt; 0.0001). Of patients undergoing appendectomy during index presentation, 1688 (1.80 %) had “developmental delay” diagnosis. Complicated appendicitis (33.77 % vs. 27.79 %, <em>p</em> &lt; 0.0001) and normal appendix (4.03 % vs. 2.38 %, <em>p</em> &lt; 0.0001) were both significantly more frequent in NDD patients. NDD patients had longer length of stay (1.048, 95 % CI 1.039–1.057, <em>p</em> &lt; 0.0001). Patients with NDD had more frequent post-operative ED visits (11.32 % vs. 7.76 %, <em>p</em> &lt; 0.0001) and higher 30-day readmission rates (15.86 % vs. 8.21 %, <em>p</em> &lt; 0.0001), but lower rates of readmission related to appendectomy (76.19 % vs. 86.57 %, <em>p</em> = 0.002).</div></div><div><h3>Conclusions</h3><div>Patients with neurodevelopmental disabilities have higher rates of complicated appendicitis resulting in more complex post-operative care. These patients represent a distinct patient population that require adaptive management strategies during evaluation and treatment of appendicitis. During diagnosis, peri‑operative, and post-operative management of children with NDD and appendicitis, it is vital to incorporate appreciation of NDD in their workup.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"11 ","pages":"Article 100210"},"PeriodicalIF":0.0,"publicationDate":"2025-04-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143883202","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Pediatric temporal bone fractures: A systematic review","authors":"Karan Gandhi ,&nbsp;Chloe Pulver ,&nbsp;Peng You","doi":"10.1016/j.yjpso.2025.100208","DOIUrl":"10.1016/j.yjpso.2025.100208","url":null,"abstract":"<div><h3>Purpose</h3><div>Pediatric temporal bone fractures, pose significant risks including hearing loss, facial nerve paralysis, and intracranial complications. This study aims to address the knowledge gap in outcomes following these fractures in children.</div></div><div><h3>Methods</h3><div>A comprehensive literature search across Embase, MEDLINE (PubMed), and Web of Science was conducted following PRISMA guidelines. The primary outcome analyzed was hearing loss, with secondary outcomes including facial nerve injury, and other complications.</div></div><div><h3>Results</h3><div>This study included 15 articles with outcomes for 1044 patients. The risk of sensorineural hearing loss (SNHL) was higher in otic capsule-violating (OCV) fractures than otic capsule-sparing (OCS) fractures (OR 28.57, <em>p</em> &lt; 0.001). Facial nerve injury was more likely in OCV fractures (OR 4.59, <em>p</em> = 0.0162). Transverse fractures had higher odds of SNHL compared to longitudinal fractures (OR 5.181, <em>p</em> &lt; 0.001). OCV fractures had higher odds of facial nerve injury compared to OCS fractures (OR 4.59, <em>p</em> = 0.0162), and transverse fractures had higher odds of facial nerve injury compared to longitudinal fractures (OR 3.02, <em>p</em> = 0.0146). No significant differences in conductive hearing loss were found between fracture types. Only 57 % of patients had audiometric data available.</div></div><div><h3>Conclusions</h3><div>This study indicates no single classification system accurately predicts outcomes for all pediatric temporal bone fractures. OCV fractures do carry a higher risk of SNHL than OCS fractures. However, this should not replace a thorough clinical assessment and audiometric testing. Long-term studies are needed to improve patient care due to limited data on long-term effects.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"10 ","pages":"Article 100208"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143800587","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(25)00020-6","DOIUrl":"10.1016/S2949-7116(25)00020-6","url":null,"abstract":"","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"10 ","pages":"Article 100211"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143855509","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Portosystemic shunt in children: Outcomes from a pediatric referral center","authors":"Thanh Tri Tran ,&nbsp;Nguyen Ha Vi Trinh ,&nbsp;Phi Duy Ho ,&nbsp;Nguyen Nhat Thang Tran ,&nbsp;Nguyen An Thuan Luu ,&nbsp;Hai Trung Bui ,&nbsp;Tuan Kiet Phan","doi":"10.1016/j.yjpso.2025.100207","DOIUrl":"10.1016/j.yjpso.2025.100207","url":null,"abstract":"<div><h3>Background</h3><div>Congenital portosystemic shunts are rare vascular malformations in children that can result in complications such as abnormal liver function, liver masses, pulmonary hypertension, and hepatopulmonary syndrome.</div></div><div><h3>Methods</h3><div>Patients with portosystemic shunts were managed according to the protocol established at the center. The characteristics and treatment outcomes of patients over a 3-year period (from January 2021 to December 2023) were retrospectively reviewed and reported.</div></div><div><h3>Results</h3><div>There were 21 pediatric patients, including 13 intrahepatic cases and 8 extrahepatic cases. All intrahepatic cases were detected at birth. The extrahepatic cases were diagnosed at an average age of 6 years. Among the 13 intrahepatic cases, clinical presentations included cholestatic jaundice (7/13) and hyperammonemia (8/13). During follow-up, 10 out of 11 intrahepatic shunts spontaneously closed after 2–10 months of monitoring, while 1/11 shunt remained open after 18 months. Two patients died due to severe pneumonia and heart defects. Among the 8 extrahepatic cases, clinical presentations included liver masses in 3/8 cases, hepatopulmonary syndrome in 1/8 case, hyperammonemia in 5/8 cases, and pulmonary hypertension in 1/8 case. Four cases without intrahepatic portal system were monitored regularly, awaiting liver transplantation if necessary. One case was lost to follow-up. Three cases underwent surgery or endovascular interventions, performed in one or two stages to close the shunt, all resulting in positive outcomes (1 case with hepatopulmonary syndrome fully recovered, 1 case with hyperammonemia and coagulation disorder returned to normal, 1 case with pulmonary hypertension and hyperammonemia did not show improvement in pulmonary hypertension after 6 months, but NH3 levels returned to normal).</div></div><div><h3>Conclusions</h3><div>Intrahepatic portosystemic shunts detected at birth often close spontaneously. In contrast, extrahepatic shunts are often detected in older children with a variety of symptoms. Surgical or endovascular interventions to close the shunt are the preferred treatment options when possible, yielding good outcomes.</div></div>","PeriodicalId":100821,"journal":{"name":"Journal of Pediatric Surgery Open","volume":"10 ","pages":"Article 100207"},"PeriodicalIF":0.0,"publicationDate":"2025-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143738370","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}