Objective: To investigate the clinical features of regional portal hypertension (RPH) in children whose primary presentation was isolated gastric fundal varices. Methods: This case series study reviewed clinical data from 8 children diagnosed with RPH complicated with isolated gastric fundal varices at Beijing Children's Hospital, Capital Medical University from June 2017 to January 2026. Data on demographics, clinical manifestations, laboratory findings, diagnosis, and treatment were extracted and analyzed for etiology, clinical characteristics, and outcomes. Results: Among 8 children there were 6 males and 2 females, with a diagnostic age of 2.2-11.5 years. The underlying etiologies were pancreatic in 6 children (5 with severe pancreatitis complicated by pancreatic pseudocyst, and 1 with pancreatic involvement secondary to acute leukemia), all involving pancreatic body and tail. One child was of splenic origin (disseminated tuberculosis with splenic involvement), and 1 child was retroperitoneal (secondary to retroperitoneal neuroblastoma). Presenting features included abdominal pain (7 children), vomiting (6 children), and gastrointestinal bleeding (2 children, 1 of whom with isolated hematemesis, another 1 with hematemesis and melena). Splenomegaly occurred in 7 children. Ascites observed in 5 children, was attributed to the primary disease rather than to RPH. All 8 children had normal platelets, 3 children presented with leukopenia and anemia, which were attributed to the primary disease and secondary lesions. All 8 children had normal liver function, and coagulation function, but all demonstrated splenic vein abnormalities. All 8 children received treatment targeting at the underlying etiology. Somatostatin was administered to 5 children, and hemostatic agents to the 2 children with gastrointestinal bleeding. No child underwent endoscopic intervention. The follow-up ranged from 2 months to 8 years; 1 child with tuberculosis was lost to follow-up. The remaining 7 children showed symptomatic relief and improvement or complete resolution of gastric fundal varices. Conclusions: Regional portal hypertension often manifests as isolated gastric fundus varices and splenomegaly, and may be accompanied by gastrointestinal bleeding. Pancreatic, splenic, and retroperitoneal etiologies should be vigilanted. Individualized treatment strategies focused on the primary disease are crucial for improving pediatric RPH outcomes.

The spleen is usually viewed as a hematologic, vascular and immune organ, but rarely within lipid-associated disease. This review proposes splenic steatopathy as a provisional clinical and experimental framework rather than an established diagnosis or a splenic equivalent of fatty liver disease. Evidence is organized into pre-splenic drivers, intra-splenic pathology and post-splenic consequences. Upstream drivers include metabolic dysfunction-associated steatotic liver disease, obesity, severe hypertriglyceridemia, lysosomal storage disorders, drug-induced phospholipidosis, altered lymphatic lipid handling and portal-hemodynamic stress. Spleen-level findings include altered volume, attenuation, stiffness or metabolic activity; lipid-laden histiocytes; lysosomal or phospholipid storage; and immune-cell remodeling. Potential downstream relevance includes cytopenias, immune dysfunction, infection vulnerability, diagnostic redirection, cancer-associated immune biology and perioperative risk. Human evidence currently consists mainly of observational imaging studies and rare tissue reports; animal studies provide mechanistic support, while several proposed clinical links remain hypotheses. No validated diagnostic criteria, imaging thresholds or biomarkers currently exist. The framework may help distinguish unexplained splenomegaly from common mimics and, if validated, support long-term risk stratification for infection and malignancy in patients with metabolic liver disease. Future studies should prioritize spleen-specific imaging methods, tissue-imaging correlation, lipidomics, immune phenotyping and prospective outcome validation.

The development of hepatocellular carcinoma (HCC) in a chronically diseased liver, often referred to as hepatocarcinogenesis, is a complex process characterized by the progressive accumulation and interaction of genetic alterations, leading to abnormal growth, malignant transformation of liver parenchymal cells, followed by vascular invasion and metastasis.

Patent ductus venosus (PDV) is a rare intrahepatic congenital portosystemic shunt that can result in hepatic encephalopathy and hepatopulmonary syndrome due to diversion of portal blood away from hepatic metabolism. Although typically diagnosed in infancy, PDV may rarely remain undetected until adolescence. Management of late presenters is challenging, as abrupt closure of large shunts may precipitate portal hypertension, and coil embolization carries a higher risk of systemic migration. We report a 16-year-old boy presenting with unexplained hypoxemia (SpO2 84% on room air), digital clubbing, and hyperammonemia. Bubble contrast echocardiography demonstrated an extracardiac right-to-left shunt, while abdominal imaging confirmed a large PDV with associated diffuse pulmonary arteriovenous malformations. Temporary balloon occlusion testing demonstrated hemodynamic suitability for closure. Transcatheter occlusion using a Konar-Multifunctional Occluder resulted in significant improvement in arterial oxygen saturation and normalization of serum ammonia levels, sustained during one-year follow-up. This case highlights the importance of meticulous hemodynamic assessment and demonstrates the safety and efficacy of the Konar-MFO device in late-presenting PDV.

Shear wave elastography is a non-invasive ultrasound technique that quantifies tissue stiffness with high reproducibility. While its role in assessing portal hypertension is well established, the spleen has recently gained attention as a potential biomarker in non-portal conditions. As a vascular and immune organ, the spleen undergoes structural and functional changes in various systemic diseases, making splenic shear wave elastography a promising diagnostic and prognostic tool. This review summarizes current evidence regarding splenic shear wave elastography in healthy individuals and in diseases beyond portal hypertension. In hematologic disorders, particularly myeloproliferative neoplasms, splenic stiffness correlates with bone marrow fibrosis, extramedullary hematopoiesis, and thrombotic complications. In cardiovascular conditions, splenic congestion reflects systemic venous pressure and has been studied in acute and chronic heart failure, as well as in congenital circulatory alterations such as Fontan physiology. Research in inherited storage diseases, transfusion-dependent anemias, and autoinflammatory syndromes suggests a role for shear wave elastography in detecting parenchymal infiltration and monitoring disease progression. Infectious diseases including brucellosis, schistosomiasis, and HIV-related vasculopathy, further highlight its sensitivity to microvascular and inflammatory changes. Pediatric studies have expanded normative reference ranges and explored applications in systemic infections and splenomegaly. Despite encouraging findings, methodological heterogeneity, variability among ultrasound platforms, and physiological confounders limit comparability across studies. Current evidence remains predominantly based on small, single-center cohorts, and disease-specific cutoff values have yet to be established. In conclusion, splenic shear wave elastography shows potential for broad clinical applicability, but standardization of protocols, multicenter validation, and integration into multiparametric ultrasound frameworks are needed before implementation in routine practice.

Endo-hepatology is a rapidly evolving field encompassing endoscopic diagnosis and treatment of hepatobiliary diseases, primarily using endoscopic ultrasound (EUS). Since its conceptualization in 2012, the field has expanded dramatically alongside advances in interventional EUS. A landmark 2024 review broadened the concept to include endoscopic management of hepatobiliary diseases using interventional EUS/endoscopic retrograde cholangiopancreatography and advanced imaging technologies. In portal hypertension (PH), diagnostic applications include EUS-based variceal hemodynamic assessment, EUS-guided shear wave elastography, EUS-guided porto-systemic pressure gradient measurement (EUS-PPGM), EUS-guided liver biopsy (EUS-LB), and portal vein sampling. Therapeutic applications include EUS-guided vascular embolization for gastric, esophageal, and ectopic varices; portosystemic shunt obliteration for refractory hepatic encephalopathy; radiofrequency ablation of hepatocellular carcinoma; and emerging procedures such as intrahepatic portosystemic shunt creation and portal vein stenting. EUS-PPGM enables direct, minimally invasive portal pressure measurement and correlates strongly with the hepatic venous pressure gradient (HVPG). It may offer particular advantages in pre-sinusoidal PH, such as porto-sinusoidal vascular disorder, and in conditions with mixed sinusoidal and presinusoidal components, including metabolic dysfunction-associated steatotic liver disease, where HVPG may underestimate portal pressure. The "one-stop" concept integrates portal pressure measurement, LB, liver stiffness assessment, and variceal evaluation and treatment within a single endoscopic session. EUS-guided coil plus cyanoacrylate injection has demonstrated favorable efficacy and safety for gastric varices, while EUS-LB provides diagnostic accuracy comparable to percutaneous biopsy with less post-procedure pain. By integrating diagnostic and therapeutic procedures, endo-hepatology bridges endoscopy and hepatology and may provide comprehensive, minimally invasive, and personalized management of PH and chronic liver disease.

Hepatic vein catheterization (HVC) in man was first performed 80 years ago in the wake of right-sided heart catheterization. Several new methods, recognitions and concepts followed, especially the indirect Fick-method for determination of splanchnic blood flow. Five years passed before pressure measurements were performed and routine HVCs were done in liver patients. Later came splanchnic kinetics of test substances, dyes, metabolites, hormones and bioactive material and counter current wedged and balloon occlusion pressure. HVC has been a prerequisite for our understanding of the pathogenesis of portal hypertension and variceal bleeding leading to serial measurements, stratification, quantification and treatment of portal hypertension, and transvenous liver biopsy and portosystemic stent insertion (TIPS) followed. Measurements and pathophysiology of formation and amelioration of ascites advanced with HVC, likewise pathophysiology of known and new diseases in other organs, secondary to liver disease (kidney, heart, lung, systemic circulation). The hepatic venous pressure gradient (HVPG, i.e., wedged-to-free hepatic venous pressure) is today considered the gold standard for portal pressure measurement in intrasinusoidal and postsinusoidal portal hypertension. In spite of substantial developments in route, catheter type, pressure measurements and -registration, fluoroscopy, transvascular technique, etc. the basic concepts are the same today. New advanced technology, materials, imaging techniques and IT-power have together with the inborn dynamics of the catheterization given a progressive development, and there are potentials of further refinement and applications in science, clinical physiology, gastroenterology and hepatology.