2026 APSPGHAN声明：内镜下静脉曲张出血止血

ABSTRACT

Purpose: Endoscopic variceal hemostasis is a critical, life-saving intervention frequently performed by pediatric gastroenterologists. To ensure safe and effective practices, the Asian Pacific Society for Pediatric Gastroenterology, Hepatology and Nutrition Endoscopy Scientific Subcommittee developed a consensus-based position statement. Methods: A comprehensive literature review was conducted with an emphasis on pediatric evidence. Nineteen draft statements were formulated and refined through electronic votes and virtual meetings. Statements with <80% agreement were revised until consensus was reached. Levels of evidence certainty were evaluated to support the final recommendations. Results: Nineteen position statements, covering screening, acute management, and surgical options, were endorsed. Although noninvasive tools such as elastography and platelet-based indices may assist in risk stratification, endoscopy remains the diagnostic and therapeutic gold standard. Endoscopic variceal ligation was identified as the preferred modality for both prophylaxis and treatment in children ≥10 kg, outperforming sclerotherapy and non-

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

pghn

Shuichiro Umetsu ID https://orcid.org/0000-0002-6129-5127 Ajay Sharma ID https://orcid.org/0000-0003-2321-8275 Yao-Jong Yang ID https://orcid.org/0000-0002-2164-138X Nagina Shahzadi ID https://orcid.org/0000-0009-2160-342X Ruey Terng Ng ID https://orcid.org/0000-0003-1656-5797 Rohan Malik ID https://orcid.org/0000-0003-3450-9668 Maribel Vitug-Sales ID https://orcid.org/0000-0001-7033-4485 Katsuhiro Arai ID https://orcid.org/0000-0002-6440-4640 Way Seah Lee ID https://orcid.org/0000-0001-9163-2828 Kyung Mo Kim ID https://orcid.org/0000-0001-7896-6751

Funding

None.

Conflict of Interest

The authors have no financial conflicts of interest.

selective beta-blockers. Standard acute management incorporates octreotide and restrictive transfusion strategies, with endoscopy performed within 24 hours. In refractory cases, the Meso-Rex stunt or transjugular intrahepatic portosystemic shunt may be considered depending on anatomy and available expertise.

Conclusion: These findings provide an evidence-based framework for the management of pediatric variceal bleeding. Individualized care is emphasized while highlighting the pressing need for high-quality pediatric studies to strengthen future recommendations.

Keywords: Child; Endoscopy; Varicose veins; Hemorrhage; Hemostasis

INTRODUCTION

The number of pediatric gastroenterologists performing endoscopic procedures has increased. Findings from the 2025 Asia Pacific Society of Pediatric Gastroenterology, Hepatology and Nutrition (APSPGHAN) survey highlighted that variceal hemostatic interventions, including esophageal varices ligation (EVL), esophageal varices sclerotherapy (EVS), and gastric varices (GVs) glue injection, ranked among the five most frequently performed procedures across 162 centers in 13 countries in the Asia-Pacific region [1]. Taken together, these interventions represent the most common pediatric therapeutic procedures, followed by foreign body removal and polypectomy. Regarding training, 57 medical centers agreed that mastering variceal hemostasis is one of the four highest priority skills for trainees, as achieving independent competence in this life-saving intervention is essential [2]. In response, the APSPGHAN Endoscopy Scientific Subcommittee, together with regional authorities, began developing a position statement focused on endoscopic hemostasis for variceal bleeding. This initiative aimed to provide evidence-based guidance to help pediatric gastroenterologists perform these procedures safely and efficiently.

MATERIALS AND METHODS

Following its implementation, the APSPGHAN Endoscopy Scientific Subcommittee identified key clinical questions regarding endoscopic hemostasis for variceal bleeding. The discussion expanded beyond procedural aspects to include the broader management of portal hypertension. A comprehensive literature review was conducted, focusing on studies involving pediatric populations published within the last 10 years, using the key words of "endoscopy," "esophageal varices," "bleeding," "pediatric," "treatment," "prophylaxis," "non-invasive test," and "shunt." Based on this, the first draft of 19 position statements was developed. Consensus was sought through electronic voting using an online form, and the percentage of agreement ("strongly agree" and "agree") across four response categories was reported for each statement. The initial review and voting were arranged electronically by 13 appointed members of the APSPGHAN Endoscopy Scientific Subcommittee. Virtual meetings were then organized to refine the statements, particularly those that reached a consensus of <80%. During this round, four additional regional experts (UP, KMK, KA, and WSL) were invited to assist in revising the statements. However, three controversial statements required re-voting: (1) the age for screening endoscopy in children with biliary atresia (BA), (2) the recommended dosage of octreotide infusion, and (3) the role of EVS for primary prophylaxis. These inputs (n=17) contributed to the development of the final version of the questionnaire. Levels of certainty of evidence (CoE) were evaluated primarily

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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with a focus on pediatric data; otherwise, they were extrapolated from adult studies before formulating each positional conclusion [3,4]. A summary flowchart guideline is also provided. The final draft was approved by all the subcommittee members and regional experts prior to submission for publication.

RESULTS

The consensus process yielded 19 position statements addressing the spectrum of endoscopic management of pediatric variceal bleeding, including screening and surveillance, prophylaxis, acute hemostasis, and surgical intervention (Tables 1-3). A consensus confirmed that the hepatic venous pressure gradient (HVPG) has not been validated in children. Although noninvasive tools (NITs) such as elastography and platelet (PLT)-based indices may assist in risk stratification, endoscopic examination remains the most viable option in children. EVL was endorsed as the preferred modality for both primary and secondary prophylaxis in children ≥10 kg, demonstrating superior efficacy and safety compared with EVS or non-selective beta-blockers (NSBBs). However, EVS is considered for smaller children for whom EVL is not feasible as a secondary prophylactic procedure.

|Clinical question|Position statement| |---|---| |Is HVPG a useful tool for diagnosis and monitoring therapeutic response in pediatric portal hypertension?|HVPG has not been validated for diagnosis or for assessing therapeutic response in children with portal hypertension.| |In children with cirrhosis or chronic liver disease who have never experienced variceal bleeding, can non-invasive tools, including platelet-based indices or elastography-derived liver stiffness, safely and reliably replace endoscopy for screening esophageal varices?|Non-invasive tools, including platelet count-based formulas and elastography-derived liver stiffness measures, may serve as preliminary "rule-out" strategies to defer the first screening endoscopy in select pediatric patients with cirrhosis or chronic liver disease who are clinically stable and have not bled from varices. However, these modalities lack sufficient validation and accuracy to replace endoscopic evaluation as the definitive screening method.| |What is the optimal timing and interval for endoscopic surveillance in children with portal hypertension from both cirrhosis and EHPVO who have not yet bled from varices?|There is insufficient pediatric-specific evidence to guide endoscopic surveillance strategies in patients with cirrhosis. Recent adult guidelines support tailored EGD using non-invasive tools, but liver stiffness and platelet count have not been validated to reliably exclude high-risk varices in children. Thus, initial EGD at diagnosis is recommended in children with cirrhotic portal hypertension, with individualized follow-up intervals, typically every 1-3 yr, pending validation of pediatric-specific non-invasive models. In children with biliary atresia, an initial surveillance EGD may be considered. However, the available evidence remains insufficient to define the optimal age for screening endoscopy. Early endoscopic evaluation at 9-12 mo of age has been proposed, particularly in those with an unsuccessful Kasai operation or in the presence of signs of portal hypertension. Subsequent surveillance is generally undertaken annually, although fixed re-screening intervals have not been clearly established. The majority of children with EHPVO present with esophageal varices at diagnosis, often accompanied by variceal bleeding. Accordingly, it is reasonable to recommend an initial EGD at the time of diagnosis. If the initial endoscopy reveals no varices or only small varices, a follow-up EGD may be scheduled within 1-2 yr. However, if larger varices are identified, an earlier follow-up, typically within 6-12 mo, should be considered if prophylactic endotherapy is not initiated, to guide appropriate surveillance and intervention.| |HVGP: hepatic venous pressure gradient, EHPVO: extrahepatic portal vein obstruction, EGD: esophagogastroduodenoscopy.||

HVPG: hepatic venous pressure gradient, EHPVO: extrahepatic portal vein obstruction, EGD: esophagogastroduodenoscopy.

|Clinical Question|Position statement| |---|---| |How are clinically significant esophageal varices defined in children with portal hypertension?|High-risk or clinically significant varices in children include grade II esophageal varices with red wark markings or other red-color signs, grade III esophageal varices, and any gastric varices.| |What is the optimal modality for primary prophylaxis of children presenting with clinically significant esophageal varices?|EVL is the preferred primary prophylaxis for high-risk varices in children ≥10 kg, offering better outcomes than NSBBs or sclerotherapy. Propranolol has limited efficacy and tolerability in children, while carvedilol lacks sufficient pediatric data. In children weighing <10 kg or in those who cannot tolerate endoscopic procedures, NSBBs or EVS may be considered as alternative strategies. Supporting evidence is limited, and these approaches should be applied cautiously on a case-by-case basis, with careful assessment of potential risks and benefits.|

EVL: endoscopic variceal ligation, NSBBs: non-selective beta-blockers, EVS: endoscopic variceal sclerotherapy.

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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|Clinical question|Position statement| |---|---| |What is the optimal vasoactive agent, dosing regimen, and duration for managing acute variceal hemorrhage in children?|Intravenous octreotide is the preferred vasoactive agent for acute variceal bleeding in children. A bolus of approximately 1µg/kg (max 50 µg), followed by continuous infusion of 1-2 µg/kg/h (max 50 µg/h), is commonly used and continued for 2-5 d post-endoscopy.| |Should prophylactic antibiotics be routinely prescribed in children with acute variceal bleeding, and what is their impact on clinical outcomes such as infection, rebleeding, and mortality?|Broad-spectrum intravenous antibiotics according to local sensitivity profiles (such as third-generation cephalosporins) should be initiated early in children with acute variceal bleeding, alongside vasoactive agents and urgent endoscopy, for 5-7 days in cirrhotic portal hypertension. This recommendation is based on expert consensus and extrapolation from adult guidelines, as pediatric-specific evidence for efficacy in reducing mortality or rebleeding is limited.| |What is the optimal hemoglobin threshold and transfusion strategy in children with acute variceal hemorrhage?|A hemoglobin threshold of <7 g/dL is used to guide PRC transfusion. A restrictive transfusion strategy, aiming to maintain hemoglobin between 7-9 g/dL, is strongly recommended, as it is associated with reduced risk of rebleeding and mortality, based on extrapolation from adult data.| |Does transfusion of blood components (such as FFP, platelets, cryoprecipitate) improve clinical outcomes (hemostasis, rebleeding, and mortality) during an episode of acute variceal bleeding?|Routine correction of INR using FFP does not reduce rebleeding risk or improve hemostasis in AVB and may be harmful. Elevated INR does not reflect actual bleeding risk in cirrhosis.| |What is the optimal timing for endoscopy in pediatric patients presenting with acute variceal bleeding?|EGD should be performed within 24 h following initial resuscitation in children with suspected variceal bleeding.| |In children with portal hypertension and esophageal variceal bleeding, should EVL be used instead of EVS for initial bleeding control and secondary prophylaxis?|EVL is preferred over EVS for both acute variceal bleeding and secondary prophylaxis in children. EVL provides equivalent immediate hemostasis with fewer sessions, faster variceal eradication, and lower rates of rebleeding and complications. EVS should be reserved for cases where technical limitations prevent band placement, such as in infants with very narrow esophageal lumens (<10 kg) or children with active bleeding.| |What are the recommended treatment options for acute bleeding from gastric varices in children?|In children with acute gastric variceal bleeding, N-butyl-2-cyanoacrylate is the preferred treatment, particularly for GOV extending beyond the cardia and for IGV. BRTO may be considered as salvage therapy if endoscopy fails.| |Should proton-pump inhibitors or suralfate be routinely used to prevent early rebleeding after endoscopic treatment for varices in children?|There is insufficient evidence to support the routine use of proton-pump inhibitors or suralfate to prevent early rebleeding after endoscopic variceal ligation, sclerotherapy, or glue injection in children. No pediatric studies have specifically evaluated their efficacy for this indication.| |What is the optimal step-wise approach for managing early rebleeding following endoscopic therapy for varices in children?|Recurrent early rebleeding (≤5 d) after EVL, EVS, or glue injection should be considered treatment failure. Management includes immediate repeat endoscopic intervention, continuation of vasoactive agents, and broad-spectrum antibiotics. If bleeding persists, balloon tamponade or a self-expandable metal stent may be used, particularly in adolescents, as a bridge to early portal decompression with TIPS, surgical portosystemic shunt, or liver transplantation.| |What is the recommended frequency for endoscopic treatment and surveillance in children undergoing secondary prophylaxis for esophageal varices?|In children requiring secondary prophylaxis, endoscopic sessions should be repeated every 2-4 wk until complete variceal eradication is achieved. Although no pediatric data define the optimal surveillance interval after eradication, adopting a 6-12 mo interval for follow-up endoscopy, based on adult practice, may be reasonable until pediatric-specific guidance becomes available.| |Should non-selective beta-blockers be used alone or alongside EVL/EVS for secondary prophylaxis of variceal bleeding in children?|There is insufficient evidence to recommend routine use of NSBB, alone or in combination with EVL, for secondary prophylaxis in children. Piediatric studies are limited and show variable efficacy with frequent intolerance. Although adult data suggest adding NSBB to EVL may reduce rebleeding, this benefit has not been confirmed in children.| |What are the indications and considerations for selecting surgical or interventional shunts in children with refractory variceal bleeding?|There is insufficient evidence to determine the optimal type, timing, or comparative efficacy of bypass surgeries in children with variceal bleeding. Surgical shunts, including Meso-Rex, TIPs, and non-selective shunts, may be considered in children with refractory variceal bleeding. Meso-Rex shunt is preferred in EHPVO, when anatomy allows, for primary and secondary prophylaxis. Surgical decisions should be individualized based on etiology, vascular anatomy, transplant status, and institutional expertise.|

FFP: fresh frozen plasma, EVL: endoscopic variceal ligation, EVS: endoscopic variceal sclerotherapy, PRC: packed red cell, INR: international normalized ratio, IGV: isolated gastric varices, BRTO: balloon-occluded retrograde transvenous obliteration, TIPS: transjugular intrahepatic porto-systemic shunt, NSBBs: non-selective beta-blockers.

For acute variceal bleeding, the use of octreotide and a restrictive transfusion strategy, followed by endoscopy within 24 hours, was recommended, with EVL prioritized over EVS. Cyanoacrylate injection was advised for GVs. Early bleeding within 5 days was defined as treatment failure requiring endoscopy or escalation to salvage interventions.

Secondary prophylaxis should include serial endotherapy every 2-4 weeks until complete variceal obliteration, with 6-12 monthly surveillance thereafter. In refractory cases, surgical approaches such as the Meso-Rex shunt or transjugular intrahepatic portosystemic shunt (TIPS) may be considered, requiring individualized decision-making based on disease etiology, vascular anatomy, and available expertise. A detailed discussion of the evidence,

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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![graph TD\n A1[Children with portal hypertension HVPG not validated] --> A2[Cirrhotic]\n A1 --> A3[Non-Cirrhotic: EHPVO]\n A2 --> A4[NITS may deter surveillance EGD]\n A3 --> A12[Initial surveillance EGD at diagnosis]\n A4 --> A5[NITS>defined cutoff values Biliary atresia with failed Kasai operation/signs of PHT]\n A5 --> A6[Screening EGD]\n A6 --> A7[Presence of EV grade II or grade II with red color signs]\n A6 -->|Otherwise| A8[Surveillance EGD every 6-24 mo]\n A7 --> A9[Primary prophylaxis • EVL(≥10 kg preferred) • NSBBs or EVS may be considered if <10 kg or EVL not feasible]\n A7 --> A10[Index bleeding]\n A8 --> A11[Endoscopic intervention for secondary prophylaxis(<24 h)]\n A9 --> A10\n A10 --> A13[Integral general management • Basic life support: ABC • Octreotide • Broad-spectrum antibiotics 5-7 d • Restrictive transfusion(Hb<7) • Avoid FFP to correct INR]\n A10 --> A14[Early bleeding(≤5 d) Repeat EGD±stent/balloon]\n A11 --> A17[Cyanoacrylate injection for GV APC/Hemospray for PHG]\n A12 --> A6\n](1/parseimages/figure2fce23eb6cb8483f847fcb75b9de9ed1_0_3.png) rationale, and controversies related to these recommendations is presented in the following sections. From a practical perspective, Fig. 1 illustrates a simplified flowchart based on the written statements.

DISCUSSION

Position statement: endoscopic screening and surveillance for esophageal varices (EV) in children with portal hypertension

Statement #1 HVPG has not been validated for diagnosis or for assessing therapeutic response in children with portal hypertension [77% Strongly agree and Agree, CoE: low].

In the Baveno consensus, the HVPG was used to classify the stages of portal hypertension in adults. An HVPG ≥10 mmHg indicates compensated advanced chronic liver disease

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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(cACLD), while the presence of EV and ascites denotes clinically significant portal hypertension (CSPH) [5]. HVPG is also considered the gold standard for validating potential NITs aimed at risk stratification and guiding tailored management strategies. However, in pediatric patients, HVPG measurement is not routinely recommended, primarily because of technical challenges and limited availability. In addition, most children with portal hypertension have non-cirrhotic etiologies, such as extrahepatic portal vein obstruction (EHPVO), for which HVPG does not adequately reflect disease severity. In selected cases where a transjugular liver biopsy is performed to investigate the etiology of portal hypertension, concurrent HVPG assessment may provide additional hemodynamic information.

Statement #2 Non-invasive tools, including platelet count-based formulas and elastography-derived liver stiffness measures, may serve as preliminary "rule-out" strategies to defer the first screening endoscopy in select pediatric patients with cirrhosis or chronic liver disease who are clinically stable and have not bled from varices. However, these modalities lack sufficient validation and accuracy to replace endoscopic evaluation as the definitive screening method [92.4% Strongly agree and Agree, CoE: moderate].

Statement #3 There is insufficient pediatric-specific evidence to guide endoscopic surveillance strategies in patients with cirrhosis. Recent adult guidelines support tailored EGD using non-invasive tools, but liver stiffness and platelet count have not been validated to reliably exclude high risk varices in children. Thus, initial EGD at diagnosis is recommended in children with cirrhotic portal hypertension, with individualized follow-up intervals, typically every 1–3 years, pending validation of pediatric-specific non-invasive models [84.6% Strongly agree and Agree, CoE: low].

The Baveno VII consensus proposed liver stiffness measurement (LSM) and PLT count as NIT screening tools for diagnosing cACLD, CSPH, and decompensated cirrhosis in adults [5]. This framework has prompted investigations into its pediatric application. Composite parameters such as spleen size, aspartate aminotransferase (AST), albumin, and LSM have been evaluated. The validated formulas include the clinical prediction rule (CPR), PLT-to-spleen size ratio, AST-to-PLT ratio index (APRI), fibrosis-4 index (FIB-4), risk score, variceal prediction rule (VPR), King's variceal prediction score (K-VaPS), and LSM plus PLT count [6-12]. The CPR, derived from PLT count, spleen size z-score, and albumin level, was originally applied to predict EV in children with portal hypertension, achieving a positive predictive value (PPV) of 86% at a cutoff of <116 in Gana JC's study [6]. The APRI, a simpler and widely used marker, has also been investigated, with cutoff thresholds ranging from >0.45 to 1.6 [7,11,12]. In 2017, Witters et al. [7] proposed the K-VaPS, which incorporates equivalent adult spleen size (EASS) and albumin to predict clinically significant varices (CSV) in pediatric chronic liver diseases. Compared to CPR, VPR, APRI, and PLT-to-EASS ratio, K-VaPS (<76) achieved the highest PPV (81.6%), with a sensitivity and specificity of 71.8% and 72.7%, respectively. Transient elastography (TE) has advanced LSM as an NIT for high-risk EV, particularly in BA. More stringent criteria may be required in children with BA. Kang et al. [10] evaluated 190 patients with post-Kasai BA using TE and EGD by applying three criteria: Baveno VI (LSM <20 kPa, PLT >150,000/mm$^{3}$), extended Baveno VI (LSM <25 kPa, PLT >110,000/mm$^{3}$), and stricter criteria (LSM <10 kPa, PLT >150,000/mm$^{3}$). The stricter approach reduced the risk of missed HRV from 13.24 to 5.88% compared with adult-based criteria. Furthermore, compared with biochemical indices such as APRI and FIB-4, the Baveno VI/VII criteria allowed clinicians to confidently avoid endoscopy in patients with BA with varices needing treatment (VNT), with a missed VNT rate of 0% [13]. Yokoyama et al. [14] found that combining LSM <25 kPa, spleen stiffness measurement ≤30 kPa, and

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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APRI <1.04 outperformed both Baveno VI and VII, further reducing the risk of missed VNT.

NITs described above have been studied mainly in patients with cirrhotic portal hypertension. However, their use cannot be extended to all pediatric patients, particularly those with extrahepatic portal vein thrombosis (EHVPO). For example, Fagundes et al. [15] reported that the prevalence of EV was 100% in children and adolescents with portal vein thrombosis (PVT) compared to 52% in patients with cirrhosis. This highlights that NIT-based strategies to avoid endoscopy should be restricted to cirrhotic portal hypertension. Notably, research on pediatric NITs is increasing, and accumulating evidence may allow reevaluation of current guidelines, particularly regarding whether endoscopic surveillance can be safely reduced. This question is highly relevant because EGD, although effective, carries risks both from the procedure itself and from anesthesia in children.

Statement #4 In children with biliary atresia, an initial surveillance EGD may be considered. However, the available evidence remains insufficient to define the optimal age for screening endoscopy. Early endoscopic evaluation at 9–12 months of age has been proposed, particularly in those with an unsuccessful Kasai operation or in the presence of signs of portal hypertension. Subsequent surveillance is generally undertaken annually, although fixed re-screening intervals have not been clearly established [76.9% Strongly agree and Agree, CoE: low].

Although NITs are increasingly being explored in pediatric populations, no formal recommendations currently exist for their use. Thus, endoscopy remains the gold standard for the diagnosis and management of EV in pediatric portal hypertension. In BA, the leading cause of cirrhotic portal hypertension in children, the value of routine endoscopic surveillance remains uncertain because primary prophylaxis with EVL may not be feasible in small infants. Similarly, the benefits of NSBBs have not been clearly demonstrated in pediatric patients. In a prospective study, Duché et al. [16] performed endoscopy in 139 children with BA at a median age of 13 months and identified EV in 88 cases. Among these, 30 had red wale markings, indicating VNT. Within a median of 4 months, 28 patients developed gastrointestinal (GI) bleeding [16]. In another study, Poddar et al. [17] retrospectively analyzed 110 patients with BA who underwent EGD at a median age of 12 months (interquartile range 9–30). EVs were found in 75.4% of cases, with 32% classified as HRV. Predictors of VNT included failed Kasia procedure, spleen size >3.5 cm, PLT count <150,000/mm$^{3}$, and an APRI score >2. The authors recommended surveillance EGD between 9 and 12 months of age, or earlier in symptomatic children [17]. Tamgal et al. [18] reported that 50% of patients with BA experienced EV bleeding within 5 years after a modified Kasia operation, most occurring by 36 months. A total bilirubin >9 mg/dL at 12 months post-Kasai significantly increased bleeding risk, supporting EGD surveillance at approximately 1.5 years of age [18]. In contrast, Ackermann et al. [19] retrospectively examined 113 patients with BA at a median age of 8 years and identified HRV in only 26.6% of patients. Similarly, a Hong Kong study by Chung et al. [20] reported that the youngest BA survivor with a native liver and EV was 3 years old. Differences in the prevalence and age at VNT detection likely reflect variations in population characteristics, surgical expertise, postoperative care, and rates of ascending cholangitis. Due to this variability, the optimal age for screening endoscopy remains undefined. Ackermann et al. [19] found that 33.8% (22/65) of children without HRV at baseline developed VNT within an average of 14 months and were most eligible for primary prophylaxis. This parallels Duché's finding that 20.1% of patients developed variceal bleeding within 4 months of their first endoscopy [16]. Although evidence remains limited, standardized surveillance intervals cannot be established.

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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However, a 12-month follow-up interval may be practical, particularly in children with failed Kasai surgery and persistent signs of portal hypertension such as jaundice, splenomegaly, or thrombocytopenia.

Statement #5 The majority of children with EHPVO present with esophageal varices at diagnosis, often accompanied by variceal bleeding. Accordingly, it is reasonable to recommend an initial EGD at the time of diagnosis. If the initial endoscopy reveals no varices or only small varices, a follow-up EGD may be scheduled within 1–2 years. However, if larger varices are identified, an earlier follow-up, typically within 6–12 months, should be considered if prophylactic endotherapy is not initiated, to guide appropriate surveillance and intervention [92.4% Strongly agree and Agree, CoE: moderate].

In children, EHPVO may occur in association with thrombophilic disorders, such as protein C/S deficiency, prematurity with sepsis, necrotizing enterocolitis, or previous umbilical vein catheterization. However, in most cases, the etiology remains idiopathic [21]. Clinically, this condition most often presents with upper GI bleeding and splenomegaly, and variceal bleeding is documented as the initial manifestation in 36–85% of patients. Importantly, unlike cirrhotic portal hypertension, EVs are identified during the first surveillance endoscopy in 87–96% of non-cirrhotic EHPVO cases [21-23]. This high prevalence supports the recommendation for initial EGD after diagnosis. However, the optimal interval for subsequent follow-up remains uncertain and is typically guided by variceal severity, with suggested intervals ranging from 6 to 24 months.

Position statement: primary prophylaxis of esophageal variceal bleeding in children with portal hypertension

Statement #6 High-risk or clinically significant varices in children include grade II esophageal varices with red wale markings or other red-color signs, grade III esophageal varices, and any gastric varices [100% Strongly agree and Agree, CoE: moderate].

In 1980, the Japanese Research Society for Portal Hypertension (JRSPH) introduced a classification system to standardize endoscopic assessment of EV. The original system included four key criteria: fundamental color, red color, variceal form, and location [24]. It gained wide acceptance and has been employed, with minor revisions. In 1995, Idezuki [25] updated the classification by incorporating two additional categories: bleeding signs and mucosal findings.

According to the JRSPH system, EVs are classified into three grades: grade I aligns with F1 (straight small-calibered, ≤5 mm varices); grade II with F2 (moderately enlarged, beady varices occupying <1/3 of the lumen); and grade III with F3 (markedly enlarged, nodular or tumor-shaped varices involving >1/3 of the lumen and usually >5 mm). CSVs are commonly designated as grade III or II with red color signs.

GVs are also considered significant risk factors for GI bleedings. Evidence from a large retrospective cohort of 1,300 children with BA showed that GI bleeding occurred in 96% of those with HRV compared to 11% of those without [26].

Statement #7 EVL is the preferred primary prophylaxis for high-risk varices in children ≥10 kg, offering better outcomes than NSBBs or sclerotherapy. Propranolol has limited efficacy and tolerability in children, while carvedilol lacks sufficient pediatric data. In children weighing <10 kg or in those who cannot tolerate endoscopic procedures, NSBBs or EVS may be considered as alternative strategies. Supporting evidence is

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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limited, and these approaches should be applied cautiously on a case-by-case basis, with careful assessment of potential risks and benefits [100% Strongly agree and Agree, CoE: moderate].

Primary prophylaxis of HRV aims to reduce morbidity and mortality associated with GI bleeding, while balancing potential risks. A prospective cohort study of children with cirrhosis and those with PVT found similar eradication outcomes after an average of two EVL sessions, with 10% recurrence over 12–28 months [27]. Propranolol has been used for primary prophylaxis, mainly based on adult data. A retrospective study reported that 81.5% of 62 children with cirrhosis treated with propranolol were free of index bleeding during 2 years of follow-up [28]. However, a 2021 meta-analysis found limited evidence to confirm or exclude the benefit of beta-blockers for primary prophylaxis compared to placebo, as no well-conducted randomized controlled trials (RCTs) existed [29].

A retrospective pediatric cohort demonstrated that EVL was superior to propranolol, with a higher risk of index variceal hemorrhage in the propranolol group (hazard ratio [HR], 2.6; 95% confidence interval [CI], 1.53–3.67) [30]. A subsequent RCT showed that EVL for grade II–III EV reduced first variceal bleeding by half (7 vs. 14%) compared with propranolol [31]. Adult meta-analyses similarly show NSBBs are associated with higher rates of first EV bleeding (odds ratio [OR], 2.06; 95% CI, 1.55–2.73), although mortality was comparable [32]. In addition to reducing the risk of index EV bleeding, primary EVL prophylaxis also appears to improve overall shunt-free survival in children with portal hypertension [33]. Compared with secondary prophylaxis, primary prophylaxis requires fewer sessions to achieve variceal eradication and intensive care unit (ICU) admissions [12,33-35]. The mean difference in the number of endoscopic sessions was 1.73 [35]. Combination therapy with EVL and NSBBs did not improve clinical outcomes, including index bleeding, hepatorenal syndrome, and spontaneous bacterial peritonitis, and was associated with higher mortality than EVL alone, especially in patients with ascites [36]. Nevertheless, one RCT reported better outcomes with EVL plus carvedilol than either therapy alone, showing lower rates of index bleeding within 12 months and reduced mortality, without a significant increase in adverse events [37]. To date, no well-designed pediatric RCTs have clarified the benefits of such combination therapies. Given that EVS carries potential risks, including ulcers, strictures, and perforations, it is not recommended for primary prophylaxis, particularly in adults [38]. With EVL being the only remaining endoscopic therapeutic option, a major limitation is the small esophageal lumen in children, particularly infants, which restricts the application of currently available devices. A survey of 28 pediatric centers in Europe reported a preference for EVL in infants older than 8 months or weighing >8–10 kg, whereas EVS was still reserved for selected smaller children [39]. Based on the current evidence and practice, we recommend EVL as the initial therapeutic option for primary EV prophylaxis in children weighing >10 kg with CSV. NSBBs may be considered as an alternative in patients weighing <10 kg or in those unable or unwilling to undergo the procedure. Regarding EVS, its use in small children has been reported; however, evidence remains limited, and it should only be considered on an individualized basis when careful evaluation suggests potential benefits outweigh the risks. Despite its efficacy, EVL may carry future GI bleeding risks, including the potential to accelerate the development of portal hypertensive gastropathy (PHG) and GVs, attributable to altered hemodynamics following EV obliteration. Emerging evidence from a limited pediatric study suggests that endoscopic variceal clipping, when applied as an adjunct to gastroesophageal varices (GOV) in the fundus, may prevent the rapid progression and subsequent rupture of GVs [40]. Further well-designed pediatric trials are warranted to confirm its role in clinical practice.

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

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Position statement: general management of acute variceal bleeding in children

Statement #8 Intravenous octreotide is the preferred vasoactive agent for acute variceal bleeding in children. A bolus of approximately 1 µg/kg (max 50 µg), followed by continuous infusion of 1–2 µg/kg/h (max 50 µg/h), is commonly used and continued for 2–5 days post-endoscopy [92.3% Strongly agree and Agree, CoE: moderate].

Statement #9 Broad-spectrum intravenous antibiotics according to local sensitivity profiles (such as third-generation cephalosporins) should be initiated early in children with acute variceal bleeding, alongside vasoactive agents and urgent endoscopy, for 5-7 days in cirrhotic portal hypertension. This recommendation is based on expert consensus and extrapolation from adult guidelines, as pediatric-specific evidence for efficacy in reducing mortality or bleeding is limited [100% Strongly agree and Agree, CoE: low].

Acute variceal bleeding is a serious and life-threatening event in pediatric practice. The presence of chronic liver disease with signs of portal hypertension facilitates prompt diagnosis and timely management. In critical situations, stabilization of the airway, bleeding, and circulation must take precedence before identifying the underlying cause. In suspected variceal bleeding, vasoactive agents, most notably octreotide, a somatostatin analog, are recommended to reduce splanchnic blood flow and control ongoing bleeding. Octreotide offers better efficacy and safety than vasopressin [41]. Hemostasis is achieved in approximately 71% of cases [42], and bleeding risk is reduced by 42% compared to vasopressin or terlipressin (OR, 0.58; 95% CI, 0.42–0.81) [43]. The recommended regimen is an initial intravenous bolus of 1 µg/kg (max 50 µg) followed by continuous infusion at 1–2 µg/kg/h for 2–5 days [44]. In refractory cases, infusion may be escalated to 5 µg/kg/h (max 50 µg/h). Urine output and blood glucose should be monitored to detect potential adverse events, including renal insufficiency and hyperglycemia.

The efficacy of third-generation cephalosporins and quinolones was evaluated in a large prospective cohort study that enrolled 1,656 adults with cirrhosis and acute variceal bleeding. Despite prophylaxis, 19.3% of patients developed infections, typically within 3 days of antibiotic initiation, of which 43.6% were respiratory tract infections. Patients with grade III–IV hepatic encephalopathy, Child-Pugh class C, endotracheal or nasogastric tube intubation, or esophageal balloon tamponade had a significantly higher risk of infection. More than half of the isolated organisms demonstrated resistance to third-generation cephalosporins [45]. Meanwhile, a recent retrospective cohort study reported no significant differences in outcomes, including 6-week mortality, 4-week bleeding, and 4-week spontaneous bacterial peritonitis, between patients who received prophylactic antibiotics and those who did not [46]. However, most international guidelines continue to recommend prophylactic antibiotic use in patients with cirrhosis presenting with acute variceal bleeding [5]. A retrospective pediatric cohort study reported an overall mortality rate of 20.1% following variceal bleeding, with bacteremia and peritonitis identified as independent predictors of mortality (OR of 2.29 and 2.18, respectively) [47]. Together, these findings emphasize the strong link between infections and poor outcomes in patients with cirrhosis presenting with acute variceal hemorrhage. Although most evidence comes from adult studies, broad-spectrum intravenous antibiotic prophylaxis for 5–7 days is recommended in children with cirrhotic portal hypertension and acute variceal bleeding, who may be particularly vulnerable. Its role in non-cirrhotic portal hypertension remains unclear.

Statement #10 A hemoglobin threshold of <7 g/dL is used to guide PRC transfusion. A restrictive transfusion strategy, aiming to maintain hemoglobin between 7–9 g/dL, is strongly recommended, as it is

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associated with reduced risk of rebleeding and mortality, based on extrapolation from adult data [100% Strongly agree and Agree, CoE: high].

Statement #11 Routine correction of INR using FFP does not reduce rebleeding risk or improve hemostasis in AVB and may be harmful. Elevated INR does not reflect actual bleeding risk in cirrhosis [92.4% Strongly agree and Agree, CoE: moderate].

A pivotal RCT in adults showed that a restrictive transfusion approach, using a hemoglobin threshold of <7 g/dL, significantly lowered 6-week mortality compared with a liberal strategy maintaining hemoglobin >9 g/dL (HR, 0.55; 95% CI, 0.33-0.92). The observed benefit likely reflects a reduction in rebleeding risk (from 16 to 10%), as excessive transfusion may elevate intravarice pressure [48]. In line with this reasoning, prolonged INR and thrombocytopenia were not significantly associated with acute variceal bleeding, and correction of INR with FFP not only failed to improve hemostasis but was also linked to higher rates of unsuccessful bleeding control [49-51]. Supporting this, Mohanty et al. [52] found that FFP transfusion was associated with markedly higher mortality (OR, 7.09; 95% CI, 2.62-19.21), whereas transfusion of PRCs or PLT did not show this effect. Collectively, this evidence suggests that FFP transfusion is not advantageous and may cause harm. Therefore, routine INR correction using FFP is not recommended in children with acute variceal bleeding. However, in complex cases with additional bleeding sources, particularly those complicated by sepsis or disseminated intravascular coagulation, correction of coagulopathy and thrombocytopenia may be considered individually. According to an observational study by Giannini et al. [50], adult patients with cirrhosis with thrombocytopenia and low fibrinogen levels had a higher risk of post-EVL bleeding than those with fibrinogen levels >179 mg/dL. This finding supports the potential benefit of cryoprecipitate transfusions in selected populations.

Position statement: endoscopic management of acute variceal bleeding in children

Statement #12 EGD should be performed within 24 hours following initial resuscitation in children with suspected variceal bleeding [100% Strongly agree and Agree, CoE: low].

Retrospective adult studies have shown that endoscopy performed within 12 hours offers no significant advantage over procedures performed at 12–24 hours with respect to early rebleeding (<5 days post-intervention), in-hospital mortality, or ICU admission [53,54]. These findings were further corroborated by a meta-analysis demonstrating no significant differences in hospital mortality or early rebleeding between early and delayed endoscopy groups [55]. Thus, priority should be given to timely hemodynamic stabilization rather than performing urgent endoscopy. However, the meta-analysis noted a modest reduction in overall mortality among patients who underwent early endoscopy (OR, 0.56; 95% CI, 0.33–0.95). In line with the findings of Zhang et al. [54], patients undergoing urgent endoscopy had a longer mean hospital stay, although it remains uncertain whether this was due to higher initial disease severity or insufficient resuscitation before the procedure. Collectively, these findings suggest that timely and appropriate cardiovascular stabilization should be prioritized over rushing to perform endoscopy within 12 hours. Pediatric data support a similar approach: in a retrospective study of 73 children, endoscopy performed within 12–24 hours after stabilization was associated with favorable outcomes and no increase in adverse events [56].

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Statement #13 EVL is preferred over EVS for both acute variceal bleeding and secondary prophylaxis in children. EVL provides equivalent immediate hemostasis with fewer sessions, faster variceal eradication, and lower rates of rebleeding and complications. EVS should be reserved for cases where technical limitations prevent band placement, such as in infants with very narrow esophageal lumens (<10 kg) or children with active bleeding [100% Strongly agree and Agree, CoE: moderate].

EVL was first introduced at the Baveno II consensus in 1995 and subsequently gained broad acceptance in adult clinical practice at the Baveno IV consensus in 2005. Since then, studies investigating its application in pediatric populations have been published. Although retrospective pediatric studies with portal hypertension suggest that EVL or EVL+EVS has advantages over EVS alone for AVB, these findings must be interpreted cautiously due to inherent limitations of retrospective designs [57-60]. Nevertheless, the trend was supported by an early pediatric RCT, providing more reliable, although still limited, evidence. Consistent with adult RCT findings [61], this pediatric RCT demonstrated that EVL was superior to EVS, requiring fewer sessions for variceal eradication (3.9±1.1 vs. 6.1±1.7) and resulting in fewer rebleeding events or major complications (4% vs. 25%) [62]. A meta-analysis including 1,236 adult patients further showed that EVL was associated with a lower rebleeding rate (relative ratio [RR], 0.68; 95% CI, 0.57-0.81) and higher eradication efficacy (RR, 1.06; 95% CI, 1.01-1.12) than EVS [63]. To date, no meta-analysis has evaluated EVL versus EVS for acute management and secondary prophylaxis of AVB in children, likely reflecting the scarcity of high-quality pediatric RCTs. As noted in Statement #7, esophageal size remains critical for EVL application; in small children requiring endoscopic secondary prophylaxis, EVS may be used as a bridging strategy until a liver transplant can be performed.

Statement #14 In children with acute gastric variceal bleeding, N-butyl-2-cyanoacrylate is the preferred treatment, particularly for GOV extending beyond the cardia and for IGV. BRTO may be considered as salvage therapy if endoscopy fails [92.1% Strongly agree and Agree, CoE: low].

Diagnosis of GVs is challenging, as they can easily be mistaken for normal gastric folds. Endoscopic ultrasound has been shown to improve diagnostic sensitivity, as reported in a pediatric case series by McKiernan et al. [64]. GVs are classified into GOV type 1 (GOV1), type 2 (GOV2), and IGVs, each requiring different therapeutic approaches. According to the Baveno VII consensus, GOV1 may be treated with either EVL (when located within the cardiac region) or N-butyl-2-cyanoacrylate injections, whereas GOV2 and IGV should be treated with N-butyl-2-cyanoacrylate injections, often in combination with BRTO. In selected high-risk patients with cirrhotic portal hypertension, preemptive TIPs within 72 hours are recommended, specifically for those with Child-Pugh class C <14, Child-Pugh class B >7 with acute bleeding at initial endoscopy, or HVPG >20 mmHg at the time of hemorrhage [5]. Two Asian centers reported cyanoacrylate glue injection as a safe and effective treatment for pediatric GVs. Poddar et al. [65] administered cyanoacrylate glue injections in 28 children with non-cirrhotic portal hypertension (25 EHPVO) with IGVs. Hemostasis was achieved in 100% of cases, and obliteration in 57%, with a median volume of 2 (1-5) mL [65]. The average injected volume varied across institutions. Oh et al. [66] recommended small, repeated injection aliquots to minimize procedure-related complications. However, the risk of rebleeding remained substantial at 52.4%, with approximately half occurring within the first year after intervention [66]. Extrapolating from adult studies, N-butyl-2-cyanoacrylate injection is recommended as first-line therapy for GOV extending beyond the cardia and IGV in children. This technique appears safe in children aged <2 years or those weighing <10 kg. In a series by Rivet et al. [67], immediate hemostasis was achieved in all

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eight infants, requiring a mean of 1.4 sessions, with injection volumes ranging from 0.5-2 mL per session. Endoscopic ultrasound-guided coil placement is a potential therapeutic option in selected cases, demonstrating safety and efficacy, reduced radiation exposure, and a 12-month bleeding-free interval in 75% of pediatric cases [68].

Evidence on the management of PHG bleeding in children is limited. One prospective pediatric study available suggested that endoscopic band ligation is superior to argon plasma coagulation (APC), being associated with shorter procedures, fewer sessions, reduced transfusion requirements, and a lower 6-month recurrence rate. However, the study was confounded by inhomogeneously enrolled participants, including patients with gastric antral vascular ectasia, which includes portal hypertension-related and non-variceal cases [69]. In the absence of robust pediatric evidence, APC or hemospray may be employed, extrapolating from adult practice.

Statement #15 There is insufficient evidence to support the routine use of proton-pump inhibitors or sucralfate to prevent early bleeding after endoscopic variceal ligation, sclerotherapy, or glue injection in children. No pediatric studies have specifically evaluated their efficacy for this indication [92.3% Strongly agree and Agree, CoE: low].

Theoretically, proton pump inhibitors (PPIs) may accelerate ulcer healing after endoscopic treatment of esophageal and GVs and may help prevent PHG bleeding, particularly in patients with cirrhosis. Supporting this, a cross-sectional observational study demonstrated that PPI administration after EVL was associated with a reduced incidence of rebleeding (from 16 to 7%) [70]. Similarly, Kang et al. [71] conducted a retrospective review of 505 adults with cirrhosis and showed that the absence of PPI therapy was an independent factor associated with increased post-EVL bleeding (OR, 8.217; p=0.002). Furthermore, an RCT demonstrated that 2-year rabeprazole therapy significantly reduced the risk of treatment failure (HR, 0.098; 95% CI, 0.012–0.79). However, the trial was prematurely terminated after an interim analysis, limiting the strength of conclusions [72]. A systematic review by Lo et al. [73], including 20 articles (five RCTs), supported short-term (10-day) PPI benefits but found no evidence for long-term outcomes. More recently, a 2025 real-world analysis of 6,196 patients (764 receiving post-EVL PPI therapy) reported that PPI did not reduce the risk of esophageal bleeding at 4 or 8 weeks [74]. Thus, there is insufficient evidence to support routine prescription of PPIs or sucralfate to prevent early rebleeding after endoscopic variceal therapy, particularly in children.

Statement #16 Recurrent early rebleeding (≤5 days) after EVL, EVS, or glue injection in children should be considered treatment failure. Management should include immediate repeat endoscopic intervention, continuation of vasoactive agents, and broad-spectrum antibiotics. If bleeding persists, balloon tamponade or a self-expandable metal stent may be used, particularly in adolescents as a bridge to early portal decompression with TIPS, surgical portosystemic shunt, or liver transplantation [100% Strongly agree and Agree, CoE: low].

Early rebleeding, regarded as endoscopic treatment failure, is typically defined as hemorrhage from post-interventional ulcers eroding underlying varices within 5 days after the procedure. A retrospective pediatric study reported an 8% incidence of early rebleeding, most occurring after EVL below the esophagogastric junction [75]. This incidence aligns with results from a large multicenter retrospective adult study, which reported a rebleeding rate of 7.5% after EVL and a 6-week mortality rate of 16.5% [76]. Independent predictors of

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early rebleeding include higher total bilirubin, prolonged prothrombin time, elevated Child-Pugh and Model for End-Stage Liver Disease scores, a greater number of ligation rings, larger variceal diameter (F3, ≥1.4 cm), lower albumin, and the presence of hepatocellular carcinoma. In an RCT, Garcia-Pagan et al. [77] reported that adults with cirrhosis with AVB who underwent early TIPS had significantly better 2-year rebleeding-free survival and overall survival than those managed with pharmacologic therapy and EVL. A meta-analysis of five studies (1,754 patients) further demonstrated that early TIPS placement (within 72 hours of admission) markedly reduced early rebleeding (OR, 0.11; 95% CI, 0.05–0.23) and 6-week mortality (OR, 0.2; 95% CI, 0.13–0.46) [78]. Based on this evidence, Baveno VII recommends preemptive TIPS in selected high-risk patients, guided by Child-Pugh classification and HVPG measurement. In cases of treatment failure, rescue therapeutic options such as self-expandable metallic stents, balloon tamponade, salvage TIPS, or embolization may be employed as bridging strategies for liver transplantation. Given the limitations of these strategies in pediatric patients related to device size, availability, and technical feasibility, this position statement advises immediate repeat endoscopic intervention, with adjunctive therapies, such as endoscopic clipping or embolization of feeding vessels, considered where appropriate. General management with octreotide infusion and broad-spectrum antibiotics should continue. Children with persistent failure should undergo surgical consultation for portosystemic shunt placement or liver transplantation as the next-line rescue option.

Position statement: secondary prophylaxis of EV bleeding

Statement #17 In children requiring secondary prophylaxis, endoscopic sessions should be repeated every 2-4 weeks until complete variceal eradication is achieved. Although no pediatric data define the optimal surveillance interval after eradication, adopting a 6-12-months interval for follow-up endoscopy, based on adult practice, may be reasonable until pediatric-specific guidance becomes available [92.3% Strongly agree and Agree, CoE: low].

Endoscopic intervention for secondary prophylaxis to achieve complete variceal eradication typically requires more sessions than primary prophylaxis, averaging approximately four sessions (range 1–8). The optimal interval for eradication has not been standardized, although Price et al. [79] suggested scheduling procedures every 2–4 weeks until eradication is confirmed endoscopically. Recurrence remains a concern: approximately half of children with portal hypertension developed new varices at alternative sites after primary (41.7%) and secondary (45.9%) prophylaxis. Index bleeding and rebleeding rates of 14.3% and 34.1%, respectively, were observed in children with EHPVO undergoing endoscopic intervention [80]. These findings emphasize the need for long-term surveillance. Drawing from adult protocols, follow-up endoscopy every 6–12 months is reasonable, with shorter intervals individualized for children with more severe portal hypertension until pediatric-specific guidelines become available.

Statement #18 There is insufficient evidence to recommend routine use of NSBB, alone or in combination with EVL, for secondary prophylaxis in children. Pediatric studies are limited and show variable efficacy with frequent intolerance. Although adult data suggest adding NSBB to EVL may reduce rebleeding, this benefit has not been confirmed in children [92.4% Strongly agree and Agree, CoE: low].

The role of NSBBs as adjunctive therapy for secondary prophylaxis remains uncertain. In a retrospective study of 43 children with portal hypertension, adding propranolol to endoscopic therapy did not significantly improve outcomes compared to EVL/EVS alone,

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with similar rates of recurrent varices and rebleeding [81]. A 2021 Cochrane systematic review reached a similar conclusion for primary prophylaxis, noting the absence of RCTs and insufficient evidence to draw firm conclusions [29]. In adults, a retrospective study suggested that carvedilol may offer advantages over propranolol, improving hemodynamics, variceal outcomes, liver-related mortality, and ascites [82]. Despite these encouraging findings, carvedilol has been minimally studied in children.

Position statement: surgical approaches and liver transplantation

Statement #19 There is insufficient evidence to determine the optimal type, timing, or comparative efficacy of bypass surgeries in children with variceal bleeding. Surgical shunts, including Meso-Rex, TIPs, and non-selective shunts, may be considered in children with refractory variceal bleeding. Meso-Rex shunt is preferred in EHPVO, when anatomy allows, for primary and secondary prophylaxis. Surgical decisions should be individualized based on etiology, vascular anatomy, transplant status, and institutional expertise [100% Strongly agree and Agree, CoE: very low].

In refractory cases, surgical options may be explored, although standardized stepwise guidelines for children remain lacking because of limited availability, technical expertise, and reliance on case reports and small case series. Splenic artery embolization can be used to control EV bleeding in patients with portal hypertension and severe thrombocytopenia [83]. The Meso-Rex shunt has become the most accepted treatment option for refractory variceal hemorrhage and severe hypersplenism, particularly in children with EHPVO [84-87]. Compared to traditional portosystemic shunts, it carries a higher risk of thrombosis (14.1% vs. 5.8%) and the need for reoperation (11.8% vs. 4.1%) [87]. Therefore, careful planning of post-procedural anticoagulation with a multidisciplinary team is essential. Long-term outcomes are encouraging; in one institutional series, shunt patency rates were 71% at 1 year and 66% at 5 years [85]. In certain Asian centers, the Meso-Rex shunt has been established as the treatment of choice for both primary and secondary prophylaxis of EHPVO [88,89]. Despite requiring advanced surgical expertise, this procedure has demonstrated high success rates with a low risk of recurrence. By restoring physiological portal venous flow, the shunt is considered a definitive and potentially curative therapeutic option. A technical modification utilizing the coronary vein has been described, obviating the need for cervical or groin dissection for autologous vein harvesting [88]. BRTO has also been successfully applied, including in a 15-month-old infant with BA presenting with fundal GVs [90]. A retrospective study in children (mean age 12.6 years) showed that TIPS achieved a high technical and hemodynamic success rate (post-TIPS gradient ≤12 mmHg) of 93.4% and 94%, respectively. Furthermore, the clinical success rate for AVB was 94.1%, with a patency rate of 91.3% at 2 years, leading to a transplant-free survival rate of 88.8% [91]. These results suggest that TIPS is technically feasible and should be considered as a bridging therapy to liver transplantation, especially in children and adolescents with cirrhosis and refractory variceal bleeding.

Compared to the Pediatric Gastrointestinal Endoscopy guidelines published by the European Society for Pediatric Gastroenterology, Hepatology and Nutrition in 2017, our statements provide a more comprehensive focus on the management of variceal bleeding. Both societies recommend EVL over EVS [92]. As pediatric evidence remains limited, definitive recommendations for the management of esophageal variceal bleeding in children have not yet been well established. Future collaborative research should be encouraged, particularly within the Asia-Pacific region. Directions for future studies should focus on developing and validating NITs to guide the optimal timing of endoscopic screening, especially in children

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with cirrhosis, and defining the definitive role of primary prophylactic interventions in preventing catastrophic outcomes from severe acute bleeding episodes.

CONCLUSION

The APSPGHAN position statement provides a comprehensive framework for guiding pediatric gastroenterologists in the management of variceal bleeding. EVL has emerged as the preferred modality for both prophylaxis and treatment, whereas beta-blockers and sclerotherapy have more limited roles and are reserved for specific circumstances. Octreotide, antibiotics, and restrictive transfusion strategies are integral components of acute management. Surgical approaches, such as the Meso-Rex shunt and TIPS, are considered for refractory cases. Overall, the statement emphasizes individualized care based on the best available evidence, while highlighting the urgent need for high-quality pediatric research to further optimize outcomes.

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Pediatric Gastroenterology, Hepatology & Nutrition

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52. Mohanty A, Kapuria D, Canakis A, Lin H, Amat MJ, Paniz GR, et al. Fresh frozen plasma transfusion in acute variceal haemorrhage: Results from a multicentre cohort study. Liver Int 2021;41:1901-8. PUBMED | CROSSREF 53. Peng M, Bai Z, Zou D, Xu S, Wang C, Başaranoğlu M, et al. Timing of endoscopy in patients with cirrhosis and acute variceal bleeding: a single-center retrospective study. BMC Gastroenterol 2023;23:219. PUBMED | CROSSREF 54. Zhang W, Huang Y, Xiang H, Zhang L, Yuan L, Wang X, et al. Timing of endoscopy for acute variceal bleeding in patients with cirrhosis (CHESS1905): A nationwide cohort study. Hepatol Commun 2023;7:e0152. PUBMED | CROSSREF 55. Bai Z, Wang R, Cheng G, Ma D, Ibrahim M, Chawla S, et al. Outcomes of early versus delayed endoscopy in cirrhotic patients with acute variceal bleeding: a systematic review with meta-analysis. Eur J Gastroenterol Hepatol 2021;33(1S Suppl 1):e868-76. PUBMED | CROSSREF 56. Bello FPS, Cardoso S, Tannuri AC, Preto-Zamperlini M, Schvartsman C, Farhat SCL. Acute upper gastrointestinal bleeding due to portal hypertension in children: What is the best timing of endoscopy? Dig Liver Dis 2022;54:63-8. PUBMED | CROSSREF 57. Poddar U, Thapa BR, Singh K. Band ligation plus sclerotherapy versus sclerotherapy alone in children with extrahepatic portal venous obstruction. J Clin Gastroenterol 2005;39:626-9. PUBMED | CROSSREF 58. Poddar U, Bhatnagar S, Yachha SK. Endoscopic band ligation followed by sclerotherapy: Is it superior to sclerotherapy in children with extrahepatic portal venous obstruction? J Gastroenterol Hepatol 2011;26:255-9. PUBMED | CROSSREF 59. Pimenta JR, Ferreira AR, Fagundes ED, Bittencourt PF, Moura AM, Carvalho SD. Evaluation of endoscopic secondary prophylaxis in children and adolescents with esophageal varices. Arq Gastroenterol 2017;54:21-6. PUBMED | CROSSREF 60. Li MM, Sun F, Huai MX, Qu CY, Shen F, Zhang Y, et al. Endoscopic variceal ligation combined with sclerotherapy for management of gastroesophageal varicaal bleeding in pediatric patients: a single-center retrospective study. Front Pediatr 2024;12:1325419. PUBMED | CROSSREF 61. Saeed ZA, Stiegmann GV, Ramirez FC, et al. Endoscopic variceal ligation is superior to combined ligation and sclerotherapy for esophageal varices: a multicenter prospective randomized trial. Hepatology 1997;25:71-4. PUBMED | CROSSREF 62. Zargar SA, Javid G, Khan BA, Reveille RM, Goff JS, Hepps KS, et al. Endoscopic ligation compared with sclerotherapy for bleeding esophageal varices in children with extrahepatic portal venous obstruction. Hepatology 2002;36:666-72. PUBMED | CROSSREF 63. Dai C, Liu WX, Jiang M, Sun MJ. Endoscopic variceal ligation compared with endoscopic injection sclerotherapy for treatment of esophageal variceal hemorrhage: a meta-analysis. World J Gastroenterol 2015;21:2534-41. PUBMED | CROSSREF 64. McKiernan PJ, Sharif K, Gupte GL. The role of endoscopic ultrasound for evaluating portal hypertension in children being assessed for intestinal transplantation. Transplantation 2008;86:1470-3. PUBMED | CROSSREF 65. Poddar U, Borkar V, Yachha SK, Srivastava A. Endoscopic management of bleeding gastric varices with N-butyl, 2-cyanoacrylate glue injection in children with non-cirrhotic portal hypertension. Endosc Int Open 2016;4:E1063-7. PUBMED | CROSSREF 66. Oh SH, Kim SJ, Rhee KW, Kim KM. Endoscopic cyanoacrylate injection for the treatment of gastric varices in children. World J Gastroenterol 2015;21:2719-24. PUBMED | CROSSREF 67. Rivet C, Robles-Medranda C, Dumortier J, Le Gall C, Ponchon T, Lachaux A. Endoscopic treatment of gastroesophageal varices in young infants with cyanoacrylate glue: a pilot study. Gastrointest Endosc 2009;69:1034-8. PUBMED | CROSSREF 68. Barakat MT, Foley MA, Gugig R. Initial experience with endoscopic ultrasound-guided coil placement for pediatric gastric variceal hemostasis. J Pediatr Gastroenterol Nutr 2021;72:532-7. PUBMED | CROSSREF 69. Ghobrial C, Rabea M, Mohsen N, Eskander A. Gastric antral vascular ectasia in portal hypertensive children: Endoscopic band ligation versus argon plasma coagulation. J Pediatr Surg 2019;54:1691-5. PUBMED | CROSSREF 70. Khawaja I, Babar M, Awan SA, Shaikh AJ, Abbasi AA. Effectiveness of proton pump inhibitor therapy in the prevention of bleeding after prophylactic endoscopic variceal band ligation. Cureus 2023;15:e33932. PUBMED | CROSSREF 71. Kang SH, Yim HJ, Kim SY, Suh SJ, Hyun JJ, Jung SW, et al. Proton pump inhibitor therapy is associated with reduction of early bleeding risk after prophylactic endoscopic variceal band ligation: a retrospective cohort study. Medicine (Baltimore) 2016;95:e2903. PUBMED | CROSSREF

APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

Pediatric Gastroenterology, Hepatology & Nutrition

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72. Hidaka H, Nakazawa T, Wang G, Kokubu S, Minamino T, Takada J, et al. Long-term administration of PPI reduces treatment failures after esophageal variceal band ligation: a randomized, controlled trial. J Gastroenterol 2012;47:118-26. PUBMED | CROSSREF 73. Lo EA, Wilby KJ, Ensom MH. Use of proton pump inhibitors in the management of gastroesophageal varices: a systematic review. Ann Pharmacother 2015;49:207-19. PUBMED | CROSSREF 74. Amin N, Chela H, Mubarak MF, Ayoub M, Daglilar E. Proton pump inhibitor use following esophageal variceal ligation and its impact on clinical outcomes: real-world data from the TriNetX global collaborative network. Diagnostics (Basel) 2025;15:1653. PUBMED | CROSSREF 75. Yokoyama S, Ishizu Y, Ishigami M, Honda T, Kuzuya T, Ito T, et al. Factors associated with bleeding after endoscopic variceal ligation in children. Pediatr Int 2021;63:1223-9. PUBMED | CROSSREF 76. Cao C, Jin J, Cai R, Chu Y, Wu K, Wang Z, et al. Correlation between diameter of esophageal varices and early rebleeding following endoscopic variceal ligation: a multicenter retrospective study based on artificial intelligence-based endoscopic virtual rule. Front Med (Lausanne) 2024;11:1406108. PUBMED | CROSSREF 77. Garcia-Pagan JC, Caca K, Bureau C, Laleman W, Appenrodt B, Luca A, et al.; Early TIPS (Transjugular Intrahepatic Portosystemic Shunt) Cooperative Study Group. Early use of TIPS in patients with cirrhosis and variceal bleeding. N Engl J Med 2010;362:2370-9. PUBMED | CROSSREF 78. Li S, Zhang C, Lin LL, Wang Q, Zuo HX, Zhan AL, et al. Early-TIPS versus current standard therapy for acute variceal bleeding in cirrhosis patients: a systemic review with meta-analysis. Front Pharmacol 2020;11:603. PUBMED | CROSSREF 79. Price MR, Sartorelli KH, Karrer FM, Narkewicz MR, Sokol RJ, Lilly JR. Management of esophageal varices in children by endoscopic variceal ligation. J Pediatr Surg 1996;31:1056-9. PUBMED | CROSSREF 80. Oliveira APP, Ferreira AR, Fagundes EDT, Queiroz TCN, Carvalho SD, Neto JAF, et al. Endoscopic prophylaxis and factors associated with bleeding in children with extrahepatic portal vein obstruction. J Pediatr (Rio J) 2020;96:755-62. PUBMED | CROSSREF 81. dos Santos JMR, Ferreira AR, Fagundes EDT, Ferreira APS, Ferreira LS, Magalhães MCR, et al. Endoscopic and pharmacological secondary prophylaxis in children and adolescents with esophageal varices. J Pediatr Gastroenterol Nutr 2013;56:93-8. PUBMED | CROSSREF 82. Jachs M, Hartl L, Simbrunner B, Bauer D, Paternostro R, Balcar L, et al. Carvedilol achieves higher hemodynamic response and lower rebleeding rates than propranolol in secondary prophylaxis. Clin Gastroenterol Hepatol 2023;21:2318-26.e7. PUBMED | CROSSREF 83. Dinçer BT, Urganci N, Bayrak AH, Durmaz O, Özden I. The role of partial splenic artery embolization in the management of refractory esophageal variceal bleeding due to portal vein thrombosis. BMC Pediatr 2025;25:49. PUBMED | CROSSREF 84. Superina R, Shneider B, Emre S, Sarin S, de Ville de Goyet J. Surgical guidelines for the management of extra-hepatic portal vein obstruction. Pediatr Transplant 2006;10:908-13. PUBMED | CROSSREF 85. Cortez AR, Kassam AF, Jenkins TM, Nathan CJ, Nathan JD, Alonso MH, et al. The role of surgical shunts in the treatment of pediatric portal hypertension. Surgery 2019;166:90713. PUBMED | CROSSREF 86. Yamoto M, Chusilp S, Alganabi M, Sayed BA, Pierro A. Meso-Rex bypass versus portosystemic shunt for the management of extrahepatic portal vein obstruction in children: systematic review and meta-analysis. Pediatr Surg Int 2021;37:1699-710. PUBMED | CROSSREF 87. Zielsdorf S, Narayanan L, Kantymyr S, Barbetta A, Kwon Y, Etesami K, et al. Surgical shunts for extrahepatic portal vein obstruction in pediatric patients: a systematic review. HPB (Oxford) 2021;23:656-65. PUBMED | CROSSREF 88. Cho YP, Ha TY, Ko GY, Kim KM, Lee SG. Use of meso-Rex shunt with transposition of the coronary vein for the management of extrahepatic portal vein obstruction. Ann Surg Treat Res 2014;86:105-8. PUBMED | CROSSREF 89. Ha TY, Kim KM, Ko GY, Oh SH, Kwon YW, Cho YP, et al. Variant meso-Rex bypass with transposition of abdominal autogenous vein for the management of idiopathic extrahepatic portal vein obstruction: a retrospective observational study. BMC Surg 2015;15:116. PUBMED | CROSSREF 90. Komada T, Suzuki K, Oshima K, Matsushima M, Nagasaka K, Naganawa S. Balloon-occluded retrograde transvenous obliteration for fundal gastric variceal bleeding in a small child. J Vasc Interv Radiol 2019;30:1624-5. PUBMED | CROSSREF 91. Bertino F, Hawkins CM, Shivaram G, Gill AE, Lungren MP, Reposar A, et al. Technical feasibility and clinical effectiveness of transjugular intrahepatic portosystemic shunt creation in pediatric and adolescent patients. J Vasc Interv Radiol 2019;30:178-86.e5. PUBMED | CROSSREF

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APSPGHAN Statement on Endoscopic Variceal Bleeding Hemostasis

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