Search Results (381)

Background: Hypoplastic left heart syndrome (HLHS) is defined as “a spectrum of congenital cardiovascular malformations with normally aligned great arteries without a common atrioventricular junction, characterized by underdevelopment of the left heart with significant hypoplasia of the left ventricle including atresia, stenosis, or hypoplasia of the aortic or mitral valve, or both valves, and hypoplasia of the ascending aorta and aortic arch”. Without treatment, HLHS is usually lethal in the neonate. Many hypotheses have been advanced to explain the etiology of HLHS; however, no single theory appears to fully explain the phenotypic variability seen in HLHS. Furthermore, many of these theories offer no explanations regarding the precipitating events which lead to the development of HLHS. Objective: This review considers and critically evaluates the strengths and weaknesses of the leading theories proposed to explain the pathogenesis of HLHS—including hemodynamic disturbances, primary myocardial structural defects, valvar malformations, and genetic or epigenetic alterations that may provoke developmental and anatomic abnormalities. After presenting each model, we propose a novel, comprehensive, and data-driven framework which may assist researchers in developing models for the pathogenesis of the various subtypes of HLHS. Methods: Key findings from human fetal imaging, histopathology, genetic studies, and animal models were considered, as well as the hypothetical contribution of each in observed HLHS phenotypes. The rationales for these findings as causal factors initiating individual HLHS patterns, as well as how they might contribute to HLHS in general, were critically analyzed. Results: The flow theory is strongly supported by animal models and in utero interventions that demonstrate the impact of altered hemodynamics on cardiac morphogenesis. However, the flow theory fails to identify initial causes of disturbed flow or related histological features of HLHS like endocardial fibroelastosis. The myocardial and valve-first models suggest an important role in developmental defects, but do not necessarily have a strong experimental basis that provides explanations for how they mediate HLHS. Genetic studies in patients with HLHS have identified several candidate causal mutations. However, such genetic causes of HLHS exhibit incomplete phenotypic penetrance and clinical impact. A multifactorial framework attempts to integrate these diverse mechanisms and may provide the most coherent explanation that can accommodate the heterogeneity and variable presentation of HLHS. Such a framework may identify multiple forces that drive disease but does not provide useful pathways for future research about HLHS. Conclusions: No single hypothesis has fully explained how HLHS is initiated, progresses, and presents with the clinical conditions that are encountered by cardiac surgeons and cardiologists. The most current models suggest that the spectrum of HLHS reflects acomplex interaction between genetic susceptibility, flow-dependent cardiac remodeling, and environmental factors in utero. A multifactorial model integrates these diverse mechanisms and may provide the most coherent explanation for the various phenotypic variations in HLHS. Based on our analysis of the most current data and the strengths and weaknesses of the current theoretical frameworks, we propose a novel research strategy aimed at identifying specific cardiac progenitor cell populations whose dysregulation may represent a unifying explanation for the etiology of the various phenotypes of HLHS. Based on the arguments made throughout this manuscript that evaluate the various genetic, structural, and hemodynamic models of initiation of disease, we believe that the significant phenotypic variability across the spectrum of HLHS (i.e., the different anatomic subtypes for “classic” HLHS) most likely reflects different underlying etiologies and mechanisms. At the very least, it is very likely that the timing of the insult is critical in determining anatomic subtype. Based on the published data and the arguments within this manuscript, it seems naive to think that there is a single unifying mechanism explain all forms of HLHLS. Full article

Background: Acute appendicitis in girls presenting with lower abdominal pain remains a frequent diagnostic dilemma because of the overlap in clinical presentation with gynaecological and non-surgical causes. This study aimed to evaluate the diagnostic performance of IL-6 and CD64 and to compare them with classical inflammatory markers and the Alvarado score. Methods: We conducted a prospective observational diagnostic-accuracy study over a three-year period (December 2022 to December 2025) at the First University Paediatric Surgery Clinic, General Hospital of Thessaloniki “Georgios Gennimatas”. Consecutive girls aged ≤16 years presenting with lower abdominal pain were included. The primary outcome was appendicitis (yes/no), defined by the final clinical diagnosis and, where applicable, intraoperative and/or histopathological confirmation. Diagnostic performance was assessed using ROC curves and AUCs with 95% confidence intervals estimated by the DeLong method. The prespecified primary regression model included the Alvarado score and IL-6; IL-6 was summarised on its original scale and log1p-transformed only for regression analyses to account for right-skewness. Additional multivariable models were exploratory. Results: Of 74 initially assessed cases, one was excluded (appendiceal neuroendocrine tumour, NET G1), yielding a final sample of 73 girls: 37 with appendicitis and 36 without appendicitis. IL-6 was higher in the appendicitis group (median 19.41 vs. 4.10 pg/mL) and showed moderate discrimination (AUC 0.696). CRP showed lower to borderline performance (AUC 0.595), whereas CD64 did not demonstrate useful discrimination (AUC 0.521). The Alvarado score had the highest discriminatory ability (AUC 0.885). In the subset with complete data, adding IL-6 to the Alvarado score did not materially improve discrimination. Conclusions: IL-6 showed moderate diagnostic performance as a standalone biomarker and may be useful as an adjunct, particularly when a clinical score is unavailable or unreliable. CD64 did not add meaningful diagnostic information in this setting. Larger, prespecified studies are required to determine clinically useful cut-offs and to clarify whether IL-6 offers incremental value beyond established clinical assessment. Full article

Background: Cerebral palsy (CP) is the leading cause of permanent physical disability in children. Although hip surveillance is a global standard, spinal surveillance remains inconsistent, often leading to reactive rather than proactive management of neuromuscular scoliosis. This study aims to establish an international consensus on a risk-based spinal surveillance protocol. Methods: A three-round modified Delphi process was conducted in 2024 with 15 international pediatric spine surgeons, identified through purposive sampling. The process adhered to CREDES standards and focused on establishing standards for timing, frequency, and radiographic surveillance. Consensus thresholds were defined a priori as excellent (≥80%) and good (≥73%) agreement. Results: The panel reached excellent consensus (93%) on a “Traffic Light” system based on the Gross Motor Function Classification System (GMFCS) levels. Green Group (Walkers, GMFCS I–II): Clinical surveillance. Amber Group (Poor Walkers, GMFCS III, and asymmetric hemiplegic GMFCS I–II): Annual radiographs starting at ages 3–8. Red Group (Non-Walkers, GMFCS IV–V): Six-monthly radiographs starting at ages 3–5. There was 100% consensus on the mandatory use of sitting radiographs for non-ambulatory patients to prevent masking true pelvic decompensation. Critical referral triggers were identified as a Cobb angle >20°, pelvic obliquity ≥5°, or a progression rate ≥1° per month. Conclusions: The “Traffic Light” protocol helps identify the “window of opportunity” for intervention before reaching the 90° “point of no return,” where surgical risks increase nonlinearly. This proactive approach aims to reduce surgical complications and systemic delays in specialized care. Full article

Background: In the paediatric intensive care unit (PICU), certain drugs should be avoided or administered with strict precautions and close monitoring. This is due to their potential for toxicity or adverse effects or a lack of safety data, especially for critically ill children with organ failure. Additionally, practitioners must assess the unique pharmacokinetic and pharmacodynamic properties of drugs when treating critically ill children. In this narrative review, we highlight the risks, advantages, and disadvantages of three exemplary cases of drugs for paediatric patients hospitalised in the PICU: chloramphenicol, acetylsalicylic acid, and propofol. Methods: Apart from key papers on these drugs, a retrospective analysis of the English literature on chloramphenicol, acetylsalicylic acid (ASA), and propofol was performed on PubMed for papers from January 2014 to December 2025. Results: Chloramphenicol should be avoided in neonates due to the risk of grey baby syndrome. Acetylsalicylic acid (ASA) is contraindicated in children ≤18 years with suspected viral illness because of the risk of Reye’s syndrome, but remains essential for Kawasaki disease and post-cardiac surgery antiplatelet therapy. Propofol should be avoided when used for a longer period at high doses. With proper dosing and monitoring, propofol-related infusion syndrome (PRIS) is preventable, but high-risk patients should receive alternative treatment. Conclusions: This narrative review highlights the significant risks associated with the use of chloramphenicol, ASA, and propofol in paediatric intensive care settings. Their potential for life-threatening and severe adverse reactions emphasises the need for cautious and informed use. Clinicians must carefully consider the risks and benefits of these drugs. To minimise adverse events, strict monitoring, dose adjustments, and the use of safer alternatives are essential. However, it appears that their use in well-defined circumstances in acute illness in children is still warranted. The findings of this narrative review underscore the need for further research to focus on identifying high-risk biomarkers, genetic predispositions, and safer alternatives to improve evidence-based guidelines and reduce morbidity and mortality in paediatric intensive care. Full article

Background: Minimal access surgeries are growing more common in neonatal care, but the risk of accidental injury to abdominal wall blood vessels remains a concern. This risk is increased by limited precise anatomical data specific to neonates. Therefore, this study aimed to quantitatively map the superficial and deep blood vessels of the neonatal anterior abdominal wall concerning important surgical landmarks to develop evidence-based recommendations for safer laparoscopic port placement. Methods: Thirty formalin-fixed low-birth-weight neonatal body donations (≤4 weeks old) were dissected. An anatomical grid based on palpable landmarks—including the umbilicus, xiphoid process, and anterior superior iliac spines—was utilised to measure distances to the nearest vessels via digital image analysis. In situ topography of the liver, stomach, and umbilical vessels was also documented. Results: A midline corridor of reduced vascular density was identified; minimum circumferential distances to deep vessels above the umbilicus averaged 6.84–6.88 mm. Conversely, lateral regions were highly vascular, particularly at or below the transumbilical plane, with distances to deep vessels as short as 1.08 ± 0.83 mm. The liver and stomach extended significantly below the costal margin (averaging 20.61 ± 8.29 mm and 34.18 ± 14.44 mm, respectively). Conclusions: The results establish an anatomical foundation for using the reduced vascular midline for port placement and highlight the importance of inserting secondary lateral ports under direct visualisation. Full article

Background: Non-Wilms renal tumours (NWRTs) are rare paediatric malignancies and account for a small but clinically significant proportion of childhood renal cancers. Due to their low incidence and biological heterogeneity, outcome data are limited, and management is largely extrapolated from international collaborative protocols. No national data describing the incidence and outcomes of NWRTs in children in the Republic of Ireland (ROI) have previously been published. Objective: To determine the incidence, treatment strategies, and survival outcomes of NWRTs in children in the ROI. Methods: A retrospective cohort study was conducted of all children under 16 years of age with histologically confirmed renal tumours diagnosed and treated at Children’s Health Ireland (CHI) at Crumlin between January 2005 and December 2025. As CHI Crumlin is the single national paediatric oncology centre in the ROI, this cohort represents national case ascertainment for the study period. A total of 143 paediatric renal tumours were identified; Wilms tumours (n = 118) were excluded, leaving 25 children (17.48%) with NWRTs for analysis. No cases of bilateral renal tumours were identified. Histological subtypes included renal cell carcinoma (RCC), clear cell sarcoma of the kidney (CCSK), congenital mesoblastic nephroma (CMN), malignant rhabdoid tumour of the kidney (MRTK), and anaplastic sarcoma of the kidney. Demographic characteristics, treatment strategies, and survival outcomes were analysed. Results: Twenty-five children with NWRTs were identified: CCSK (n = 9), RCC (n = 7), CMN (n = 6), MRTK (n = 2), and anaplastic sarcoma of the kidney (n = 1). At a median follow-up of 107.9 months (range 4.5–181.3 months), overall survival for the cohort was 76%. Overall survival by histology was 100% for CMN, CCSK and anaplastic sarcoma, 43% for RCC, and 0% for MRTK. Treatment strategies varied by histology, with 68% undergoing upfront surgery, 32% receiving neoadjuvant chemotherapy, 60% receiving adjuvant systemic therapy, and 44% receiving radiotherapy. Tumour recurrence occurred in 4/25 patients (16%), confined to the RCC (3) and CMN (1) subgroups. Seven Event-Free Survival events were observed, comprising three RCC relapses and one RCC progression, one CMN relapse, and two MRTK progression-related deaths. No recurrences occurred in CCSK. Conclusions: NWRTs comprised 17.5% of all paediatric renal tumours diagnosed nationally during the study period and demonstrated marked heterogeneity in outcomes according to histological subtype. CMN showed excellent survival with six out of seven requiring surgery alone, whereas MRTK remained associated with dismal outcomes despite multimodal therapy. These national data support histology-driven, risk-adapted management and highlight the importance of continued international collaboration to improve outcomes in NWRTs. Full article

Background: Pes planovalgus affects 44–54% of preschool children and represents one of the most common concerns in pediatric orthopedic practice. Aim: This narrative review synthesizes the evidence linking increased femoral antetorsion to pediatric flatfoot deformity. Methods: A comprehensive literature search was conducted in PubMed, Scopus, and Web of Science through January 2026. The initial search yielded 847 records; after screening, 52 studies were included, 29 of which are directly cited. Search terms included combinations of: “femoral antetorsion” OR “femoral anteversion” AND “flatfoot” OR “pes planovalgus” AND “children” OR “pediatric”. Results: Strong correlations exist between flatfoot and increased internal hip rotation (as a proxy for femoral antetorsion) in preschool children (r = 0.53–0.77), suggesting an association, though direct causation remains unproven. Both deformities share similar developmental trajectories with spontaneous resolution by school age. The biomechanical model proposes that elevated antetorsion reduces gluteus medius moment arms by 40–50%, necessitating compensatory hip internal rotation; however, this derives from computational models and cerebral palsy populations, with limited direct validation in typically developing children. Femoral derotation osteotomy improves the foot progression angle, though transfer efficiency is incomplete (~54% of surgical correction manifests distally). Conclusions: Femoral antetorsion and pes planovalgus are strongly associated in preschool children, though whether this represents a direct mechanistic cascade or parallel manifestations of common developmental factors remains uncertain. This understanding supports watchful waiting in preschool children and, in persistent cases, prioritizes the assessment of the entire rotational profile before intervention. Full article

Paediatric osteoarticular infections (OAIs) encompass a heterogeneous group of musculoskeletal infections associated with acute septic complications, prolonged morbidity and potentially long-term sequelae. Over the past two decades, advances in microbiological diagnostics—particularly nucleic acid amplification assays—have refined the aetiological understanding of OAIs and started a new therapeutic debate regarding the most appropriate routes of antibiotic administration. Clinicians now evaluate which children can be treated safely using oral antibiotics from the outset (oral-first), which require an initial intravenous (IV) phase before a step-down to oral therapy, and which will need IV therapy all along their care pathway. Treatment debates are particularly relevant in contexts involving constrained healthcare resources and limited hospital bed availability. This narrative review summarises the essential prerequisites for prescribing oral antibiotic therapy for paediatric OAIs and proposes a pharmacokinetic/pharmacodynamic (PK/PD) framework for guiding clinical decision-making. Key considerations include: pathogen identification and resistance profiling; contemporary bacteriological epidemiology; the comparative effectiveness of IV versus oral therapy; the availability of active oral antibiotics and their penetration into bone and joint compartments; achieving adequate systemic exposure and hitting PK/PD targets after oral administration; and the clinical limitations of oral antibiotic therapy, including patient selection criteria. We argue that oral-first and early-switch strategies are best framed as structured selection processes that integrate clinical severity and source control, pathogen/minimal inhibitory concentration constraints, the feasibility of attaining PK/PD targets orally and the reliability of follow-up. No single strategy should be seen as a universal default strategy. Full article

Background: Preoperative differentiation between uncomplicated and complicated paediatric appendicitis remains challenging. This study aimed to evaluate the diagnostic performance of routine admission biomarkers and blood cell count-derived inflammatory indices for severity stratification and to determine whether fibrinogen provides additional predictive value beyond commonly used markers. Methods: We conducted a retrospective single-centre study (2018–2025) using electronically recorded clinical data. Patients with suspected appendicitis were identified through appendicitis-related ICD-10 codes and diagnostically validated. The final analytical cohort required complete admission laboratory data, including C-reactive protein (CRP), fibrinogen, and complete blood count parameters. Derived inflammatory indices included the neutrophil-to-lymphocyte ratio (NLR) and the systemic immune-inflammation index (SII). Diagnostic discrimination and multivariable prediction models were evaluated to assess the ability of these markers to distinguish complicated from uncomplicated appendicitis. Results: Of 1518 screened records, 1132 patients met inclusion criteria (620 complicated; 512 uncomplicated). Complicated appendicitis was associated with higher inflammatory markers and longer hospital stay (all p < 0.001). CRP demonstrated the strongest univariable discrimination (area under the curve [AUC] 0.785), while fibrinogen showed lower performance (AUC 0.744). A combined model including CRP, NLR, and SII achieved good discrimination (AUC 0.812), with minimal improvement after adding fibrinogen (AUC 0.813). In multivariable analysis, log-transformed CRP and SII remained independently associated with complicated appendicitis (both p < 0.001). A rule-out probability threshold of 0.303 achieved 90% sensitivity (negative predictive value 0.803), whereas a CRP cut-off ≥92.24 mg/L showed high specificity (0.943) and positive predictive value (0.900). Conclusions: Routine admission biomarkers and inflammatory indices derived from complete blood counts can support severity stratification in paediatric appendicitis. CRP and SII provide meaningful predictive information, whereas fibrinogen contributes little additional discriminatory value beyond CRP-based models. These findings suggest that a small set of routinely available laboratory markers may assist early risk stratification, although external validation is required before clinical implementation. Full article

Background/Objectives: Paediatric renal tumours, particularly Wilms tumours, have good survival outcomes following multimodal therapy; however, long-term morbidity related to nephrectomy and adjuvant treatment remains a major concern. As treatment paradigms increasingly prioritize nephron preservation and minimization of late effects, there is growing interest in technologies that can enhance intraoperative precision. Methods: A scoping review following the PRISMA guidelines was performed. We analysed articles on fluorescence for childhood renal tumours on 1 November 2025. Case reports, opinion articles, and narrative reviews were excluded. An Ovid Medline search with search terms “Kidney neoplasm” AND “Fluorescent Dyes”, along with a Cochrane trials registry search for “kidney” AND “neoplasm” AND “Fluorescent Dye”, was performed, along with a hand search of citations. Results: The Ovid Medline search yielded 21 results, and the Cochrane trials search gave 4 results. Following review, five papers were included, of which one was an ex vivo study and one was a randomised, controlled trial that is currently recruiting. Conclusions: There is a lack of evidence around the use of near-infrared fluorescence in paediatric renal tumour surgery. This review summarizes the key current findings and future perspectives. Full article

Introduction: Understanding superficial epigastric vessel anatomy is crucial for abdominal surgeries like laparoscopy, especially in neonates, to prevent injury. While standard courses are described, variations occur. This case report highlights a unique anatomical variation in the superficial epigastric artery found during the dissection of a stillborn neonatal cadaver. Case Report: In contrast to the usual origin from the femoral artery, this variation features the inferior epigastric artery penetrating the anterior abdominal wall near the umbilicus and branching superiorly to supply the superficial abdominal wall. Conclusions: This distinctive vascular configuration, which to the best of our knowledge has not been previously described in neonatal anatomical literature, diverges from the typical symmetrical arrangement and previously reported variations. The study stresses the clinical importance of this finding, especially for laparoscopic procedures in neonates. During trocar placement, surgeons should be cognizant of such variations to reduce the risk of iatrogenic injuries, including rectus sheath hematoma. The report highlights the need for further investigation to establish the prevalence of this variation and its potential effects on surgical safety and outcomes in a broader neonatal population, which may also reflect the dynamic vascular remodeling that occurs during early developmental stages. Full article

Background and Objectives: Cerebral palsy is a debilitating and complex movement disorder affecting millions of people worldwide. Many children with cerebral palsy develop hip dysplasia, which can lead to pain, functional decline, and long-term complications. Regular hip surveillance is therefore essential to allow early intervention and prevent progression. At present, screening is performed manually by experienced clinicians, which can be time consuming and costly. This study aimed to compare the performance of artificial intelligence models with expert clinicians in detecting hip dysplasia in children with cerebral palsy. Materials and Methods: A thorough search of Embase, Ovid MEDLINE, and Web of Science was conducted from inception to July 2025. Studies evaluating AI-based detection of hip dysplasia in children aged 18 years or younger with cerebral palsy were included. Risk of bias was assessed using the QUADAS-2 tool. Results were synthesised narratively in accordance with SWiM guidelines. Results: Across the six included studies, which included over 4000 radiographs, AI sensitivity for detecting hip dysplasia ranged from 70% to 97.4%, and specificity ranged from 85% to 96%, depending on the migration percentage thresholds applied. Area under the curve values ranged from 0.923 to 0.999. Only one study performed external validation using a national surveillance dataset. Risk of bias was moderate to high in most studies due to internal validation and small datasets. Conclusions: The findings suggest that AI demonstrates potential as an adjunct for hip surveillance in children with cerebral palsy. Full article

Background: Ipsilateral concomitant ureteropelvic junction (UPJ) and ureterovesical junction (UVJ) obstruction is an uncommon but clinically important pediatric condition. Because standard imaging often detects only one level of obstruction, the coexistence of both lesions is frequently overlooked. Delayed diagnosis may result in persistent hydronephrosis, recurrent urinary tract infections, and progressive renal injury. This systematic review synthesizes current evidence regarding diagnostic challenges, management strategies, and outcomes in children with dual UPJ–UVJ obstruction. Methods: A systematic review following PRISMA 2020 guidelines was conducted and prospectively registered in PROSPERO. Major databases were searched for studies describing pediatric patients with confirmed ipsilateral UPJ + UVJ obstruction. Extracted data included clinical presentation, diagnostic pathways, imaging modalities, timing of diagnosis, surgical sequencing, and postoperative outcomes. Results: Across the 8 included studies, preoperative recognition of dual obstruction was uncommon. Most cases were diagnosed intraoperatively when retrograde stent passage failed or postoperatively when hydronephrosis persisted after an apparently adequate first procedure. Retrograde or antegrade pyelography consistently outperformed ultrasonography and diuretic renography in identifying distal pathology. Staged repair—typically beginning with pyeloplasty—emerged as the most reliable approach, as correction of the proximal obstruction alone frequently improved distal drainage. UVJ-first strategies were less effective and often required secondary pyeloplasty. Endoscopic and minimally invasive techniques showed promise in selected patients but were reported in limited numbers with short follow-up. Functional renal outcomes generally stabilized or improved following complete correction, particularly when intervention occurred early in life. Conclusions: Dual UPJ–UVJ obstruction remains a diagnostic challenge in pediatric urology. Complementing standard imaging with contrast pyelography and maintaining vigilance during intraoperative stent placement can improve detection. Available reports suggest that a staged proximal-first surgical strategy can optimize drainage and reduce the risk of unnecessary distal reconstruction. Early intervention appears beneficial for renal recovery, though long-term outcomes remain insufficiently studied. Ongoing follow-up is essential, particularly in children with recurrent urinary tract infections or persistent hydronephrosis. Full article

Objective: The association between vitamin D status and paediatric fracture risk remains controversial, with inconsistent findings across existing studies. This study aimed to evaluate the relationship between serum 25(OH)D concentrations, vitamin D sufficiency, insufficiency and deficiency, vitamin D supplementation and fracture risk in a large Southeast Asian paediatric cohort. Methods: This retrospective cross-sectional study included children under 18 years whose serum 25(OH)D concentrations were measured between 2014 and 2022. One-way ANOVA determined statistical significance between 25(OH)D concentrations in fracture and non-fracture groups. Prevalence of vitamin D insufficiency, deficiency and supplementation was compared between the two groups. Chi-square tests evaluated the association between 25(OH)D concentrations and supplementation against fracture risk. Results: A total of 4530 children were included (157 fracture cases, 4373 controls). Mean serum 25(OH)D concentration was lower in the fracture group than in the controls (27.44 ± 12.26 vs. 30.75 ± 15.21 ng/mL; p = 0.007). Sub-sufficient vitamin D status (<30 ng/mL) was more prevalent among fracture patients (p = 0.001), and suboptimal (p = 0.001), insufficient (p = 0.001), and deficient (p = 0.014) categories were each significantly associated with fractures. An association between vitamin D supplementation and fracture risk was observed. However, the dataset did not permit the determination of causality and a protective effect cannot be inferred. Conclusions: Higher serum 25(OH)D concentrations were associated with lower fracture risk, suggesting that optimisation of vitamin D status may represent a modifiable factor in paediatric bone health. Healthcare institutions should aim to maintain adequate 25(OH)D concentrations (>30 ng/mL). An association between vitamin D supplementation and fracture risk was observed; however, causality cannot be inferred from this retrospective dataset. Full article

Background: Extrahepatic portal vein obstruction (EHPVO) accounts for more than two thirds of pediatric portal hypertension. Rex shunt is the preferred surgical management, as it restores hepatopetal flow and minimizes or reverses liver dysfunction. Case Summary: We report surgical treatment of EHPVO in a 9-year-old girl using portal-Rex shunt with internal jugular vein (IJV) as a conduit and the intact proximal main portal vein instead of the superior mesenteric vein as a venous inlet. Improvement in thrombocytopenia and reduction in splenic size were achieved post-operatively. The portal-Rex shunt remains patent with good hepatopetal flow at one year post-operation. Conclusions: The success of a portal-Rex shunt to selectively bypass EHPVO rests upon careful selection of inlet and outlet veins, as well as a conduit with good patency, such as the IJV. Full article