原発性肝外門脈閉塞症

PURPOSE: Extrahepatic portal vein obstruction is a leading cause of upper gastrointestinal bleeding in India, with limited options once first- and second-line treatments fail. We describe a novel, fully endovascular technique for transjugular mesocaval shunt creation using transabdominal ultrasound guidance and report our single-center outcomes in patients with refractory variceal hemorrhage secondary to extrahepatic portal vein obstruction. MATERIALS AND METHODS: Five consecutive male patients (age range 17-54 years; mean 31.4 years) with confirmed extrahepatic portal vein obstruction in whom surgical shunting or who were unsuitable for portal vein recanalization with transjugular intrahepatic portosystemic shunt underwent transjugular mesocaval shunt creation between 2024 and 2025. Anticoagulation was initiated with intravenous heparin and transitioned to direct oral anticoagulants. RESULTS: Technical success was achieved in all five patients (100%). Mean procedure time was 71 minutes (range 55-100 minutes), mean fluoroscopy time was 48 minutes (range 38.8-62.3 minutes), and mean postprocedural portosystemic pressure gradient was 5.6 mmHg (range 5-6 mmHg), confirming effective shunt decompression in all cases. Single-pass inferior vena cava-to-superior mesenteric vein puncture was achieved in four of five patients (80%). Four patients had previously undergone splenectomy with distal splenorenal shunt, and one had a prior failed surgical mesocaval shunt. One patient underwent combined portal vein recanalization with transjugular intrahepatic portosystemic shunt and mesocaval shunt creation as a bridge to transplantation, underwent liver transplantation after one month, and died of unrelated sepsis. In the remaining four patients, all shunts remained patent on Doppler follow-up (range 7-14 months), with no recurrent variceal bleeding and no procedure-related complications. CONCLUSIONS: Transjugular endovascular mesocaval shunt creation using transabdominal ultrasound guidance is feasible, safe, and reproducible in patients with extrahepatic portal vein obstruction who have undergone failed surgical shunting or are unsuitable for portal vein recanalization with transjugular intrahepatic portosystemic shunt. This approach eliminates the need for percutaneous abdominal wall access, computed tomography guidance, or intravascular ultrasound, making it accessible in resource-constrained settings.

OBJECTIVES: The hepatology committee of the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) aims to provide education on key aspects of paediatric hepatology. Here we provide recent developments in the understanding, diagnosis and clinical consequences of portal hypertension (PHT) in children and young people, an area in which evidence remains scarce and fragmented. METHODS: In 2024, an EPSGHAN 3-day monothematic conference on paediatric PHT, held in Dublin (Ireland), brought together international experts in PHT. The first sessions of the conference were dedicated to PHT classification, pathophysiology, epidemiology, diagnostic dilemmas and major complications. Lectures have been integrated to provide an up-to-date overview of clinical practice. RESULTS: Opening sessions addressed the definition and classification of paediatric PHT, highlighting key differences between cirrhotic and non-cirrhotic causes. Advances in diagnostic imaging, the role of non-invasive tests and biopsy limitations are reviewed. Subsequent discussions focused on the mechanisms and management of major complications, including renal impairment, encephalopathy, cardiopulmonary dysfunction and endocrine consequences. Nutritional challenges and the interaction between PHT and gut-liver axis were emphasised as possible determinants of outcomes. CONCLUSIONS: This report summarises the key learning points of the first part of the meeting. These discussions set the foundation for defining research priorities and improving early recognition and multidisciplinary care strategies.

Blue Rubber Bleb Nevus Syndrome (BRBNS) is a rare venous malformation disorder causing recurrent gastrointestinal bleeding and severe anemia. We report three children illustrating endoscopic, surgical, and medical management. All presented with melena and transfusion-requiring anemia. Case 1 developed gastric perforation after endoscopic band ligation and underwent emergency primary repair with bowel-preserving excision of accessible lesions, followed by recovery. Case 2 had more than 100 gastrointestinal lesions and underwent lesion-directed wedge resections, achieving transfusion independence and hemoglobin normalization at 1 year. Case 3 had idiopathic extrahepatic portal vein obstruction, portal hypertension, and diffuse mesenteric disease that was not safely resectable; sirolimus was initiated, but intermittent bleeding and transfusion dependence persisted. Management should be individualized according to lesion burden, anatomical extent, procedural risk, and feasibility of bowel-preserving treatment. Surgery remains important for complications and resectable disease, whereas systemic therapy may be required for diffuse unresectable involvement.

BACKGROUND: Since its introduction in 1992, the Rex shunt (also called Meso-Rex bypass, MRB) has substantially advanced the management of pediatric extrahepatic portal vein obstruction (EHPVO) by restoring physiological hepatopetal portal flow. However, a comprehensive synthesis of the accumulating evidence and evolving practices is notably lacking, especially given that the latest Baveno VI consensus was published nine years ago in 2016. OBJECTIVE: This review aims to synthesize three decades of advances in Rex shunt evolution, from a novel technique to a widely adopted first-line surgical option. METHODS: We conducted a narrative review, synthesizing evidence from key studies, large case series, and expert consensus statements. RESULTS: Key topics addressed include the comparison between Rex shunt and portosystemic shunts, evolving surgical indications, technical evolution of the procedure, critical aspects of perioperative management, identification of prognostic factors, and management of vascular complications. The review highlights the superior potential of the Rex shunt in resolving the sequelae of EHPVO and outlines the importance of a holistic management approach. CONCLUSION: The Rex shunt represents a major advance in the management of pediatric EHPVO. Optimal outcomes depend on careful patient selection, meticulous surgical technique, and comprehensive perioperative care, supported by an experienced multidisciplinary team.

OBJECTIVES: To analyze the factors for predicting 1-year outcomes and optimize surgical planning for Meso-Rex bypass (MRB) in children with extrahepatic portal vein obstruction (EHPVO). MATERIALS AND METHODS: From October 2014 to July 2024, children with EHPVO after MRB were retrospectively analyzed. The number of MRB, the type of bypass vessel, and the position of the lower anastomosis were collected. Predictive variables include the diameter of the connection between the left and right portal veins, the Rex vein (RV) diameter, the number of RV branches, the diameter of the thickest RV branch, and the bypass-to-superior mesenteric vein (SMV) angle, which were measured by Contrast-enhanced CT (CECT) one week after MRB. The upper and lower anastomotic stenosis were diagnosed by Ultrasound and CECT one year after MRB. RESULTS: A total of 153 children (median age 72 months, 87 males) were included in this study. Univariate factors include the number of MRBs, the type of bypass vessel, and CECT variables (except the diameter of the thickest RV branch). Numbers of MRB and RV branches were retained in the final model for upper anastomotic stenosis. The combination of MRB > 1 and RV branches ≤ 4 predicts it post-MRB with 75% sensitivity, 86.4% specificity, and an area under the receiver operator characteristic curve (AUC) of 0.832. The bypass-to-SMV angle ≤ 110° predicts lower anastomotic stenosis after MRB with 90% sensitivity, 73.39% specificity, and AUC 0.847. CONCLUSIONS: Associated factors are useful for early postoperative risk stratification. Moreover, the type of bypass vessel and the bypass-to-SMV angle could be used to inform future surgical technique refinement. KEY POINTS: QuestionWhich factors predict 1-year outcomes and optimize surgical planning for MRB in children with EHPVO? FindingsAssociated factors include bypass-to-SMV angle, number of MRB, RV branches and diameter, left-right portal vein connection diameter, and type of bypass vessel. Clinical RelevanceAssociated factors are useful for early postoperative risk stratification. Moreover, the internal jugular vein as the bridge vessel and the bypass-to-SMV angle > 110° could be used to optimize the surgical plan for a better outcome.