Up to 90% of people with CF (pwCF) will have some form of hepatobiliary involvement. This manuscript aims to explore the different endovascular, endoscopic, radiological and surgical procedures available to diagnose and manage the most severe form of CF hepatobiliary involvement (CFHBI) known as advanced cystic fibrosis liver disease (aCFLD), seen in 10% of pwCF. These procedures and interventions include liver biopsy, hepatic venous pressure gradient measurement, gastrostomy tube placement to optimize nutrition, paracentesis, endoscopic variceal control of bleeding and portosystemic shunting before liver transplantation. By utilizing advanced diagnostic or surgical techniques, healthcare professionals of pwCF can more effectively manage patients with CFHBI and aCFLD and potentially improve patient outcomes.

Interventions for portal hypertension are continuously evolving and expanding beyond the realm of medical management. When complications such as varices and ascites persist despite conservative interventions, procedures including transjugular intrahepatic portosystemic shunt creation, transvenous obliteration, portal vein recanalization, splenic artery embolization, surgical shunt creation, and devascularization are all potential interventions detailed in this article. Selection of the optimal procedure to address the underlying cause, treat symptoms, and, in some cases, bridge to liver transplantation depends on the specific etiology of portal hypertension and the patient\'s comorbidities.

Neurological symptoms that occur after treatment of portosystemic shunts, in cats, known as post-attenuation neurological signs (PANS) can be quite severe. This study seeks to analyze a better understanding of the neurological outcomes that result from reducing portosystemic shunts in felines and provide insights that could guide future clinical approaches and treatment strategies for congenital portosystemic shunts (CPSS).The research utilized the MOOSE Checklist as a guide. PubMed/MEDLINE, Web of Science ScienceDirect, Embase, Scopus, ProQuest, and Google Scholar were used. The study investigated diversity using variance, Cochran Q tests with Applied fixed effects, and random effects models. A meta-regression model identified contributors. Eggers test funnel plot and Beggs test for asymmetry addressed publication bias. 12 high-quality studies were discovered from 664 research papers. This research covered years, shunt morphology, and surgery. PANS occurred 38.9 % of the time in cats, while PAS occurred 20.2 %. The overall PANS mortality rate was 17 %, while PAS was 37.2 %. The complete ligation technique was most common in subgroup analysis. PANS occurrence ranged from 26.8 % to 56.5 % in cats with congenital extrahepatic portosystemic shunts The cause of PANS in cats is still unknown, and there is only limited evidence to justify the use of preventive antiepileptic medications such as levetiracetam. The treatment primarily aims to control neurologic symptoms, and the long-term outlook varies, with the potential for the reappearance of symptoms.

OBJECTIVE: The aim: To analyze retrospectively our experience of Rex shunt in children with symptomatic portal hypertension, its effect on hypersplenism regression and varices eradication, assess shunt survival and investigate risk factors, that could lead to shunt dysfunction and thrombosis.
METHODS: Materials and methods: 24 children (16 males, 8 females) ,with portal hypertension included into the study. All surgeries were performed within single center in a period from January 2010 to March 2022. Follow up period was 6.75±1.19 years.
RESULTS: Results: Age at diagnosis was 5.39±0.64 years. 5 (20.8%) had umbilical catheter in anamnesis. 16 (66.7%) manifested bleeding episodes as the first sign of portal hypertension. 9 (37.5%) of children manifested severe hypersplenism. Age at Rex shunting was 7.5±0.7 years. In 7 (31.8%) cases Rex shunt thrombosis occurred. 1 successful thrombectomy and 6 splenorenal shunting were performed. Kaplan-Meyer analysis showed Rex shunt survival 0.670 (95%CI 0.420-0.831). Logistic regression model indicated thrombocytes count (p=0.0423) and cytopenia (p=0.0272) as factors that could influence shunt thrombosis. Follow-up group included 18 patients. Spleen volume regression became significant by 1 p/o year p<0,05, thrombocytes significant increasement reached in 1 p/o months (p<0.01), varices involution was achieved by 1 p/o year (p<0,001).
CONCLUSIONS: Conclusions: Rex shunt effectiveness in study group was 70.9%., shunt survival assessed 0.670 (95%CI 0.420-0.831). Rex shunt was effective in bleeding prophylaxis in all patients of follow up group. Preoperative thrombocytes count (p=0.0423) and cytopenia (p=0.0272) were detected as factors that could influence shunt thrombosis, that is to be considered in RS preoperative period and require following studies.

Introduction: Portal hypertension is a syndrome characterized by increased pressure in the portal vein system and can be caused by impaired blood flow in the portal vein, hepatic veins, or inferior vena cava. The main complications of this condition are bleeding from varicose veins of the esophagus (in our study in 100% of patients), splenomegaly with hypersplenism (in our study in 98% of patients), ascites (in our study in 1 patient). The main goal of treating portal hypertension is to prevent bleeding from esophageal varices. However, today the goal of surgical treatment of portal hypertension in children is not only to prevent the development of bleeding but also the possible restoration of intrahepatic blood flow. Materials and Methods: A retrospective analysis of the results of treatment of portal hypertension in 75 children (41 boys, 34 girls) operated in our Center for the period from 2019 to 2022 was carried out. The mean age of the patients was 7 ± 1 years. Sixty-nine patients had an extrahepatic form of portal hypertension, and 6 patients had an intrahepatic form (liver fibrosis). In 14 patients (18.6%), the operation was repeated (a vascular shunt was previously applied in another hospital; 4 children were operated on repeatedly). Results: A good result was obtained in all children, and the risk of bleeding from varicose veins of the esophagus was eliminated. Vascular bypass surgery was performed in all cases: mesoportal bypass in 17 (22.7%) patients, splenorenal bypass in 37 (49.3%) patients, mesocaval bypass in 21 (28%) patients. In 10 (13%) cases, repeated bypass surgery was required due to dysfunction or thrombosis of the previously performed bypass. In 14 (18.6%) patients with mesoportal shunts, blood flow in the liver was completely restored. Conclusions: The main method of surgical treatment of portal hypertension today is portosystemic bypass surgery, which effectively prevents bleeding from varicose veins of the esophagus. Mesoportal shunting is a definitive treatment for extrahepatic portal hypertension that restores portal perfusion of the liver.

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Occlusion of spontaneous portosystemic shunts (SPSSs) in patients with cirrhosis may be required in recurrent or refractory hepatic encephalopathy. We describe a novel method for occlusion of SPSS using endoscopic ultrasound (EUS).
EUS-guided transgastric shunt obliteration was performed by injecting glue and coils directly into SPSS.
EUS-guided transgastric shunt obliteration was performed for 7 patients in 9 sessions. Complete cessation of Doppler flow was achieved in 6/7 cases. Adequate clinical response was observed in 6/7 patients. No procedure-related severe adverse events were seen.
This novel technique is a potentially effective and efficient method for shunt obliteration.

Abernethy syndrome (AS or extrahepatic portosystemic shunt) is an uncommon congenital malformation consisting of agenesis or hypoplasia of the portal vein (PV) in such a way that splanchnic venous blood drains directly into the systemic circulation through aberrant communications, resulting in a portosystemic shunt that bypasses the liver AS is an underdiagnosed condition with unknown incidence and complication rate given that symptoms are usually absent. AS identification is increasingly common because of improved imaging techniques, hence prognostic implications and clinical management need be understood. This editorial reviews the natural history of AS and its diagnostic-therapeutic implications, illustrating the process with a series of cases from our institution.

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